neurology

Question 1

Migraine: diagnostic criteria

Answer 1

A At least 5 attacks fulfilling criteria B-D

B Headache attacks lasting 4-72 hours* (untreated or unsuccessfully treated)

C Headache has at least two of the following characteristics:

1. unilateral location*
2. pulsating quality
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs)

D During headache at least one of the following:

1. nausea and/or vomiting*
2. photophobia and phonophobia

E Not attributed to another disorder

Question 2

Epilepsy: treatment

Answer 2

Most neurologists now start antiepileptics following a second epileptic seizure.

NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present: the patient has a neurological deficit brain imaging shows a structural abnormality the EEG shows unequivocal epileptic activity the patient or their family or carers consider the risk of having a further seizure unacceptable

Seizure Type 1st line 2nd line

Tonic-Clonic: Valproate, Lamotrigine

Absences: Valproate/Ethosuximide, Lamotrigine

Tonic, atonic or myoclonic: Valproate, Levetiracetam

Focal ± 2O gen: Lamotrigine, CBZ

Most neurologists now start antiepileptics following a second epileptic seizure. NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present:

the patient has a neurological deficit
brain imaging shows a structural abnormality
the EEG shows unequivocal epileptic activity
the patient or their family or carers consider the risk of having a further seizure unacceptable

Sodium valproate is considered the first line treatment for patients with generalised seizures with carbamazepine used for partial seizures

Generalised tonic-clonic seizures

sodium valproate
second line: lamotrigine, carbamazepine

Absence seizures* (Petit mal)

sodium valproate or ethosuximide
sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy

Myoclonic seizures

sodium valproate
second line: clonazepam, lamotrigine

Partial seizures

carbamazepine
second line: lamotrigine**, sodium valproate

*carbamazepine may actually exacerbate absence seizure
**the 2007 SANAD study indicated that lamotrigine may be a more suitable first-line drug for partial seizures although this has yet to work its way through to guidelines

Question 3

Essential tremor - features and management

Answer 3

Essential tremor (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs Features postural tremor: worse if arms outstretched improved by alcohol and rest most common cause of titubation (head tremor) Management propranolol is first-line primidone is sometimes used

Question 4

Parkinson’s disease: features

Answer 4

Parkinson’s disease is a progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra.. This results in a classic triad of features: bradykinesia, tremor and rigidity.

The symptoms of Parkinson’s disease are characteristically asymmetrical.

Bradykinesia - poverty of movement also seen, sometimes referred to as hypokinesia short, shuffling steps with reduced arm swinging difficulty in initiating movement

Tremor - most marked at rest, 3-5 Hz worse when stressed or tired typically ‘pill-rolling’, i.e. in the thumb and index finger

Rigidity - lead pipe + cogwheel: due to superimposed tremor

Other characteristic features mask-like facies flexed posture micrographia drooling of saliva psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur

impaired olfaction

REM sleep behaviour disorder

Drug-induced parkinsonism has slightly different features to Parkinson’s disease: motor symptoms are generally rapid onset and bilateral rigidity and rest tremor are uncommon

Question 5

Migraine: management

Answer 5

It should be noted that as a general rule 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis.

Acute treatment first-line:

an oral triptan and an NSAID, or an oral triptan and paracetamol

if not effective or not tolerated you can add a non-oral preparation of metoclopramide (young people can get acute dystonic reactions to this) or prochlorperazine

Prophylaxis

prophylaxis should be given if patients are experiencing 2 or more attacks per month. Modern treatment is effective in about 60% of patients.

NICE advise either topiramate or propranolol

topiramate is teratogenic and impairs hormonal contraceptives.

if these measures fail NICE recommend acuptuncture or gabapentin

• riboflavin (400 mg once a day) may be effective in reducing migraine frequency and intensity for some people’

for women with predictable menstrual migraine treatment NICE recommend either frovatriptan (2.5 mg twice a day) or zolmitriptan (2.5 mg twice or three times a day) as a type of ‘mini-prophylaxis’

Adverse effects such as weight gain & drowsiness are common

partial triggers - CHOCOLATE - chocolate, hangovers, orgasms, cheese, oral contraceptives, lie-ins, alcohol, tumult, exercise.

Question 6

Stroke: assessment

Answer 6

Whilst the diagnosis of stroke may sometimes be obvious in many cases the presenting symptoms may be vague and accurate assessment difficult. The FAST screening tool (Face/Arms/Speech/Time) is widely known by the general public following a publicity campaign. It has a positive predictive value of 78%.

A variant of FAST called the ROSIER score is useful for medical professionals. It is validated tool recommended by the Royal College of Physicians.

ROSIER score Exclude hypoglycaemia first, then assess the following: Assessment Scoring

Loss of consciousness or syncope - 1 point

Seizure activity - 1 point

New, acute onset of: • asymmetric facial weakness + 1 point

• asymmetric arm weakness + 1 point
• asymmetric leg weakness + 1 point
• speech disturbance + 1 point
• visual field defect + 1 point

A stroke is likely if > 0

Question 7

Carpal tunnel syndrome - features and treatment

Answer 7

Carpal tunnel syndrome is caused by compression of median nerve in the carpal tunnel.

History pain/pins and needles in thumb, index, middle finger unusually the symptoms may ‘ascend’ proximally patient shakes his hand to obtain relief, classically at night

Examination weakness of thumb abduction (abductor pollicis brevis) wasting of thenar eminence (NOT hypothenar)

Tinel’s sign: tapping causes paraesthesia

Phalen’s sign: flexion of wrist causes symptoms

Causes

idiopathic

pregnancy

oedema e.g. heart failure

lunate fracture

rheumatoid arthritis

Electrophysiology motor + sensory: prolongation of the action potential

Treatment corticosteroid injection wrist splints at night surgical decompression (flexor retinaculum division)

Question 8

Subarachnoid haemorrhage - causes, investigations and management

Answer 8

Causes 85% are due to rupture of berry aneurysms (conditions associated with berry aneurysms include adult polycystic kidney disease, Ehlers-Danlos syndrome and coarctation of the aorta)

AV malformations, trauma, tumours

symptoms - sudden (seconds), devastating typically occipital headache. “kicked in the head”. vomit, collapse, siezure, coma. neck stiffness

dd - 25% of those with thunderclap headache have SAH. 50% idiopathic. remainder = meningitis, migrane, cerebral bleeds, cortical vein thrombosis.

Investigations

CT: negative in 5%

LP: done after 12 hrs (allowing time for xanthochromia to develop)

Complications:

rebleeding (in 30%)

obstructive hydrocephalus (due to blood in ventricles)

vasospasm leading to cerebral ischaemia

Management:

• neurosurgical opinion: no clear evidence over early surgical intervention against delayed intervention
• maintain cerebral perfusion via hydration.
• nimodipine (e.g. 60mg / 4 hrly, if BP allows or 1mg/hr IVI) has been shown to reduce the severity of neurological deficits but doesn’t reduce rebleeding* *the way nimodipine works in subarachnoid haemorrhage is not fully understood. It has been previously postulated that it reduces cerebral vasospasm (hence maintaining cerebral perfusion) but this has not been demonstrated in studies
• endovascular coiling. or surgical clipping. need CT angiography.

SAH are graded 1-5. grading based on signs and predicts mortality. 1 = no signs = 0% mortality. 5 = prolonged coma = 100% mortality 3 = drowsy = 37% mortality.

Question 9

Guillain-Barre syndrome: features

Answer 9

Guillain-Barre syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). The characteristic features of Guillain-Barre syndrome is progressive weakness of all four limbs. The weakness is classically ascending i.e. the lower extremities are affected first, however it tends to affect proximal muscles earlier than the distal ones. Sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs. Some patients experience back pain in the initial stages of the illness Other features areflexia cranial nerve involvement e.g. diplopia autonomic involvement: e.g. urinary retention Less common findings papilloedema: thought to be secondary to reduced CSF resorption

Question 10

main characteristics of Migraine

Answer 10

Recurrent, severe headache which is usually unilateral and throbbing in nature May be be associated with aura, nausea and photosensitivity Aggravated by, or causes avoidance of, routine activities of daily living. Patients often describe ‘going to bed’. In women may be associated with menstruation

Question 11

main characteristics of Tension headache

Answer 11

Recurrent, non-disabling, bilateral headache, often described as a ‘tight-band’ Not aggravated by routine activities of daily living

Question 12

main characteristics of Cluster headache*

Answer 12

Pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours with clusters typically lasting 4-12 weeks Intense pain around one eye (recurrent attacks ‘always’ affect same side) Patient is restless during an attack Accompanied by redness, lacrimation, lid swelling More common in men and smokers

treat with 100% oxygen and zolmitriptan nasal spray 5mg. or sumatriptan 6mg SC.

Question 13

main characteristics of Temporal arteritis

Answer 13

Typically patient > 60 years old Usually rapid onset (e.g. < 1 month) of unilateral headache Jaw claudication (65%) Tender, palpable temporal artery Raised ESR

Typically patient > 60 years old
Usually rapid onset (e.g. < 1 month) of unilateral headache
Jaw claudication (65%)
Tender, palpable temporal artery
Raised ESR

Question 14

main characteristics of Medication overuse headache

Answer 14

Present for 15 days or more per month Developed or worsened whilst taking regular symptomatic medication Patients using opioids and triptans are at most risk May be psychiatric co-morbidity

Question 15

Motor neuron disease: types

Answer 15

Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including:

amyotrophic lateral sclerosis,

primary lateral sclerosis,

progressive muscular atrophy

and progressive bulbar palsy.

In some patients however, there is a combination of clinical patterns

Amyotrophic lateral sclerosis (50% of patients) typically LMN signs in arms and UMN signs in legs in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase

Primary lateral sclerosis UMN signs only

Progressive muscular atrophy LMN signs only affects distal muscles before proximal carries best prognosis

Progressive bulbar palsy palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei carries worst prognosis

Question 16

Stroke: management

Answer 16

CT imaging

if thromboembolic: aspirin 300mg, alteplase within 4.5 hrs, control AF and cholesterol

aspirin 300mg orally or rectally should be given as soon as possible if a haemorrhagic stroke has been excluded

If in AF you should anticoagulate but only after excluding haemorrhagic stroke and only then after 14 days post presentation.

if the cholesterol is > 3.5 mmol/l patients should be commenced on a statin.

Thrombolysis should only be given if it is administered within 4.5 hours of onset of stroke symptoms and haemorrhage has been definitively excluded (i.e. Imaging has been performed) Alteplase is currently recommended by NICE.

Question 17

Secondary prevention of stroke

Answer 17

clopidogrel

if CI then: combination use of aspirin plus modified release (MR) dipyridamole

no limit on duration of treatment

carotid artery endarterectomy: recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled should only be considered if carotid stenosis > 70% according ECST** criteria or > 50% according to NASCET*** criteria

Question 18

what is Normal pressure hydrocephalus and management

Answer 18

Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis A classical triad of features is seen urinary incontinence dementia and bradyphrenia gait abnormality (may be similar to Parkinson’s disease) Imaging hydrocephalus with an enlarged fourth ventricle Management ventriculoperitoneal shunting

Question 19

features and management of cluster headaches

Answer 19

Cluster headaches* are more common in men (5:1) and smokers

Features pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours

clusters typically last 4-12 weeks

intense pain around one eye (recurrent attacks ‘always’ affect same side) patient is restless during an attack accompanied by redness, lacrimation, lid swelling nasal stuffiness miosis and ptosis in a minority

Management acute: 100% oxygen, subcutaneous or a nasal triptan

prophylaxis: verapamil, prednisolone

NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches with respect to neuroimaging *some neurologists use the term trigeminal autonomic cephalgia to group a number of conditions including cluster headache, paroxysmal hemicrania and short-lived unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). It is recommended such patients are referred for specialist assessment as specific treatment may be required, for example it is known paroxysmal hemicrania responds very well to indomethacin

Question 20

spinal cord blood supply

Answer 20

The spinal cord is supplied with blood by three arteries that run along its length starting in the brain, and many arteries that approach it through the sides of the spinal column. The three longitudinal arteries are called the anterior spinal artery, and the right and left posterior spinal arteries.[2] These travel in the subarachnoid space and send branches into the spinal cord. They form anastamoses (connections) via the anterior and posterior segmental medullary arteries, which enter the spinal cord at various points along its length.[2] The actual blood flow caudally through these arteries, derived from the posterior cerebral circulation, is inadequate to maintain the spinal cord beyond the cervical segments. The major contribution to the arterial blood supply of the spinal cord below the cervical region comes from the radially arranged posterior and anterior radicular arteries, which run into the spinal cord alongside the dorsal and ventral nerve roots, but with one exception do not connect directly with any of the three longitudinal arteries.[2] These intercostal and lumbar radicular arteries arise from the aorta, provide major anastomoses and supplement the blood flow to the spinal cord. In humans the largest of the anterior radicular arteries is known as the artery of Adamkiewicz, or anterior radicularis magna (ARM) artery, which usually arises between L1 and L2, but can arise anywhere from T9 to L5.[3] Impaired blood flow through these critical radicular arteries, especially during surgical procedures that involve abrupt disruption of blood flow through the aorta for example during aortic aneursym repair, can result in spinal cord infarction and paraplegia.

Question 21

termination of the spinal cord

Answer 21

-terminates at about L1/2 In the upper part of the vertebral column, spinal nerves exit directly from the spinal cord, whereas in the lower part of the vertebral column nerves pass further down the column before exiting. The terminal portion of the spinal cord is called the conus medullaris. The pia mater continues as an extension called the filum terminale, which anchors the spinal cord to the coccyx. The cauda equina (“horse’s tail”) is the name for the collection of nerves in the vertebral column that continue to travel through the vertebral column below the conus medullaris. The cauda equina forms as a result of the fact that the spinal cord stops growing in length at about age four, even though the vertebral column continues to lengthen until adulthood. This results in the fact that sacral spinal nerves actually originate in the upper lumbar region.

Question 22

what is the internal capsule

Answer 22

The internal capsule is a white matter structure situated in the inferomedial part of each cerebral hemisphere of brain. It carries information past the basal ganglia, separating the caudate nucleus and the thalamus from the putamen and the globus pallidus. The internal capsule contains both ascending and descending axons. The internal capsule contains fibres going to and coming from the cerebral cortex. The corticospinal tract constitutes a large part of the internal capsule, carrying motor information from the primary motor cortex to the lower motor neurons in the spinal cord. Above the basal ganglia the corticospinal tract is a part of the corona radiata, below the basal ganglia the tract is called crus cerebri (a part of the cerebral peduncle) and below the pons it is referred to as the corticospinal tract.

Question 23

pathway of descending motor fibres

Answer 23

cortex, internal capsule, cerebral peduncles in the midbrain, basis pontis tracts in the pons, the medullary pyramids, the lateral corticospinal tracts.

Question 24

how can you block NMJ activity

Answer 24

1. Block presynaptic choline uptake: hemicholinium 2. Block ACh vesicle fusion: botulinum, LEMS 3. Block nicotinic ACh receptors

Question 25

dopamine pathways in the brain

Answer 25

There are eight dopaminergic pathways. The four major ones are: Mesocorticolimbic projection = mesolimbic pathway and mesocortical pathway The mesolimbic pathway transmits dopamine from the ventral tegmental area (VTA) to the limbic system via the nucleus accumbens. The VTA is located in the midbrain, and the nucleus accumbens is in the ventral striatum. The "meso" prefix in the word "mesolimbic" refers to the midbrain, or "middle brain", since "meso" means "middle" in Greek. This pathway is part of the mesocorticolimbic projection. The mesocortical pathway transmits dopamine from the VTA to the frontal cortex. The "meso" prefix in "mesocortical" refers to the VTA, which is located in the midbrain, and "cortical" refers to the cortex. This pathway is part of the mesocorticolimbic projection. nigrostriatal pathway - The nigrostriatal pathway transmits dopamine from the substantia nigra pars compacta (SNc) to the dorsal striatum (specifically, the caudate nucleus and putamen). This pathway is associated with motor control. tuberoinfundibular pathway - The tuberoinfundibular pathway transmits dopamine from the hypothalamus (arcuate nucleus aka "infundibular nucleus") to the pituitary gland. This pathway influences the secretion of certain hormones, including prolactin. "Infundibular" in the word "tuberoinfundibular" refers to the cup or infundibulum, out of which the pituitary gland develops.

Question 26

origin of all CN nuclei

Answer 26

Midbrain: 3, 4, (5)
 Pons:5,6,7,8
 Medulla: (5), 9, 10, 11, 12 
NB. All nuclei except 4, innervate ipsilateral side. Fibres from trochlear nucleus decussate in medulla and supply contralateral SOB muscle. 


Question 27

vestibulo-ocular reflex pathway

Answer 27

Vestibulo-occular reflex (VOR) axons from the vestibular neurones project via the MLF to the abducens and occulomotor nuclei if head turns lef the eyes turn right absent dolls eye sign = brianstem death

Question 28

path of optical neurones from retina to cortex

Answer 28

retina optic nerve optic chiasm - crossing optic tract lateral geniculate body optic radiation with lower fibres in the temporal lobe and upper fibres in the anterior parietal lobe occipital cortex

Question 29

pupillary light reflex pathway

Answer 29

bilaterally innervates the edinger-westphal nuclei in the midbrain. these innervate the ciliary ganglion which innervates the sphincter pupillae

Question 30

types of peripheral neuropathy

Answer 30

Usually distal weakness In GBS weakness is proximal (root involvement) Single nerve = mononeuropathy: trauma or entrapment Several nerves = mononeuritis multiplex: vasculitis or DM

Question 31

UMN Lesions

Answer 31

UMN Lesions Motor cells in pre-central gyrus to anterior horn cells in the cord Pyramidal weakness: extensors in UL, flexors in LL No wasting Spasticity: increase tone ± clonus Hyperreflexia Up-going plantars

Question 32

LMN Lesions

Answer 32

LMN Lesions Anterior horn cells to peripheral nerves Wasting Fasciculation Flaccidity: decrease tone Hyporeflexia Down-going plantars

Question 33

sensory pathways

Answer 33

Patterns of Sensory Deficits Pain and temp travel in small fibres in peripheral nerves and in anterolateral spinothalamic tracts Touch, joint position and vibrations travel in large fibres peripherally and in dorsal columns centrally

Question 34

what causes Brown-Sequard syndrome

Answer 34

Hemi-cord lesion causes Brown-Sequard syndrome Ipsilateral loss of proprioception / vibration and UMN weakness with contralateral loss of pain

Question 35

Cerebellar Syndrome

Answer 35

Effects: DANISH Dysdiadochokinesia Dysmetria: past-pointing Ataxia: limb / trunkal Nystagmus: horizontal = ipsilateral hemisphere Intention tremor Speech: slurred, staccato, scanning dysarthria Hypotonia

Question 36

common causes of cerebellar syndrome

Answer 36

Common Causes: PASTRIES Paraneoplastic: e.g. from bronchial Ca Alcohol: thiamine and B12 deficiency Sclerosis Tumor: e.g. CPA lesion Rare: MSA, Friedrich’s, Ataxia Telangiectasia Iatrogenic: phenytoin Endo: hypothyroidism Stroke: vertebrobasilar

Question 37

importance of the anterior cerebral artery

Answer 37

Anterior Cerebral Artery Supplies frontal and medial part of cerebrum Contralateral motor / sensory loss in the legs \> arms Face is spared Abulia (pathological laziness)

Question 38

importance of the Middle Cerebral Artery

Answer 38

Middle Cerebral Artery Supplies lateral / external part of hemisphere Contralateral motor / sensory loss in face and arms \> legs. Contralateral homonymous hemianopia due to involvement of optic radiation Cognitive changes: Dominant (L): aphasia Non-dominant (R): neglect, apraxia

Question 39

importance of the Posterior Cerebral Artery

Answer 39

Supplies occipital lobe Contralateral homonymous hemianopia ̄c macula sparing. .

Question 40

importance of the Vertebrobasilar Circulation

Answer 40

Vertebrobasilar Circulation Supplies cerebellum, brainstem and occipital lobes Combination of symptoms Visual: hemianopia, cortical blindness Cerebellar: DANISH CN lesions Hemi- / quadriplegia Uni- / bi-lateral sensory symptoms

Question 41

Lateral Medullary Syn. / Wallenberg’s Syn.

Answer 41

Lateral Medullary Syn. / Wallenberg’s Syn. Occlusion of one vertebral A. or PICA Features: DANVAH Dysphagia Ataxia (ipsilateral) Nystagmus (ipsilateral) Vertigo Anaesthesia: -Ipsilat facial numbness + absent corneal reflex - Contralateral pain loss Horner’s syndrome (ipsilateral)

Question 42

Millard-Gubler Syndrome: crossed hemiplegia

Answer 42

Millard-Gubler Syndrome: crossed hemiplegia Pontine lesions (e.g. infarct) Effects: 6th and 7th CN palsy + contralateral hemiplegia

Question 43

what is Locked-in Syndrome

Answer 43

Locked-in Syndrome Pt. is aware and cognitively intact but completely paralysed except for the eye muscles. Causes Ventral pons infarction: basilar artery Central potine myelinolysis: rapid correction of hyponatraemia

Question 44

Cerebellopontine Angle Syndrome

Answer 44

Cerebellopontine Angle Syndrome Causes: acoustic neuroma, meningioma, cerebellar astrocytoma, metastasis (e.g. breast) Effects: ipsilat CN 5, 6, 7, 8 palsies + cerebellar signs Absent corneal reflex LMN facial palsy LR palsy Sensorineural deafness, vertigo, tinnitus DANISH

Question 45

Subclavian Steal Syndrome

Answer 45

Subclavian Steal Syndrome Subclavian A. stenosis proximal to origin of vertebral A. may lead to blood being stolen from this vertebral artery by retrograde flow. Syncope / presyncope or focal neurology on using the arm. BP difference of \>20mmHg between arms

Question 46

Anterior Spinal Artery / Beck’s Syndrome

Answer 46

Anterior Spinal Artery / Beck’s Syndrome Infarction of spinal cord in distribution of anterior spinal artery: ventral 2/3 or cord. Causes: Aortic aneurysm dissection or repair Effects Para- / quadri-paresis Impaired pain and temperature sensation Preserved touch and proprioception

Question 47

Muscle Weakness Differential

Answer 47

1. Cerebrum / Brainstem Vascular: infarct, haemorrhage Inflammation: MS SOL Infection: encephalitis, abscess 2. Cord Vascular: anterior spinal artery infarction Inflammation: MS Injury 3. Anterior Horn MND, polio 4. Roots / Plexus Spondylosis Cauda equina syndrome Carcinoma 5. Motor Nerves Mononeuropathy: e.g. compression Polyneuropathy: e.g. GBS, CMT 6 NMJ GB, LEMS, botulism 7. Muscle Toxins: steroids Poly- / Dermato-myositis Inherited: DMD, BMD, FSH

Question 48

Gait Disturbance Differential

Answer 48

Motor causes: - Basal Ganglia: festinating / shuffling PD Parkinsonism: MSA, PSP, Lewy body dementia, CBD - UMN Bilateral: spastic, scissoring Cord: compression, trauma, hereditary spastic paraparesis, syringomyelia, transverse myelitis Bihemispheric: CP, MS - UMN Unilateral: spastic circumducting Hemisphere lesion: CVS, MS, SOL Hemicord: MS, tumour - LMN Bilateral: bilat foot drop Polyneuropathy: CMT, GBS Corda equina - LMN Unilateral: foot drop leads to high stepping gait Ant horn: polio Radicular: L5 root lesion Sciatic / common peroneal nerve: trauma, DM - Mixed UMN and LMN: MAST MND Ataxia: Friedrich’s SACD Taboparesis Sensory causes: - Vestibular (Romberg’s +ve) Meniere’s Viral labyrinthitis Brainstem lesion - Cerebellar: ataxic EtOH Infarct - Proprioceptive Loss (Romberg’s +ve) Dorsal columns: B12 deficiency Peripheral neuropathy: DM, EtOH, uraemia - Visual Loss - Other Myopathy, MG/LEMS Medical: postural hypotension, Stokes-Adams, arthritis

Question 49

Hand Wasting Differential

Answer 49

1. Cord Anterior Horn: MND, polio Syringomyelia 2. Roots (C8 T1) Compression: spondylosis, neurofibroma 3. Brachial Plexus Compression Cervical rib Tumour: Pancoast’s, breast Avulsion: Klumpke’s palsy 4. Neuropathy Generalised: CMT Mononeuritis multiplex: DM Compressive mononeuropathy Median: thenar wasting Ulnar: hypothenar and interossei wasting 5. Muscle Disuse: RA Compartment syndrome: Volkman’s ischaemic contracture Distal myopathy: myotonic dystophy Cachexia

Question 50

Blackouts Causes

Answer 50

Cardiac: Stokes-Adams Attacks Brady: heart block, sick sinus, long-QT Tachy: SVT, VT Structural Weak heart: LVF, tamponade Block: AS, HOCM, PE Reflexes 1. Vagal overactivity Vasovagal syncope Situational: cough, effort, micturition Carotid sinus syncope 2. Sympathetic underactivity = Post. Hypotension STANDUP Salt deficiency: hypovolaemia, Addison’s Toxins Cardiac: ACEi, diuretics, nitrates, alpha-Block Neuro: TCAs, benzos, antipsychotics, LDOPA Autonomic Neuropathy: DM, Parkinson’s, GBS Dialysis Unwell: chronic bed-rest Pooling, venous: varicose veins, prolonged standing Arterial Vertebrobasilar insufficiency: migraine, TIA, CVA, subclavian steal Shock Hypertension: phaeochromocytoma Systemic Metabolic: drop glucose Resp: hypoxia, hypercapnoea (e.g. anxiety) Blood: anaemia, hyperviscosity Head Epilepsy Drop attacks

Question 51

Blackouts - Examination and ix

Answer 51

Examination Postural hypotension: difference of \>20/10 after standing for 3min vs. lying down Cardiovascular Neurological Ix ECG ± 24hr ECG U+E, FBC, Glucose Tilt table EEG, sleep EEG Echo, CT, MRI brain

Question 52

Presentation and Investigation - Cardiogenic Syncope

Answer 52

Cardiogenic Syncope Trigger: exertion, drug, unknown Before: palpitations, chest pain, dyspnoea During: pale, slow/absent pulse, clonic jerks may occur After: rapid recovery Ix: ECG, 24hr ECG, Echo

Question 53

Presentation and Investigation - Reflex: Vasovagal syncope

Answer 53

Reflex: Vasovagal Trigger: prolonged standing, heat, fatigue, stress Before: Gradual onset: secs to mins Nausea, pallor, sweating, tunnel vision, tinnitus Cannot occur lying down During Pale, grey, clammy, brady Clonic jerks and incontinence can occur, but no tongue biting After: rapid recovery ix: Tilt-table testing

Question 54

Presentation and Investigation - Reflex: Postural Hypotension syncope

Answer 54

Reflex: Postural Hypotension Trigger: Standing up Before, During and After as for vagal above Ix: Tilt-table testing

Question 55

Presentation and Investigation - Arterial syncope

Answer 55

Arterial Trigger: Arm elevation (subclavian steal), migraine Before, During and After as for vasovagal ± brainstem Sx (diplopia, nausea, dysarthria) Ix: MRA, duplex vertebrobasilar circulation

Question 56

Presentation and Investigation - systemic causes of syncope

Answer 56

Systemic Hypoglycaemia: tremor, hunger, sweating, lightheadedness then LOC

Question 57

Presentation and Investigation - causes of syncope - Head: Epileptic

Answer 57

Head: Epileptic Trigger: flashing lights, fatigue, fasting Before: e.g. aura in complex partial seizures – feeling strange, epigastric rising, deja/jamias vu, smells, lights, automatisms During: Tongue biting, incontinence, stiffness then jerking, eyes open, cyanosis, decrease SpO2 After: headache, confusion, sleeps, Todd’s palsy Ix: EEG, increase se prolactin at 10-20min

Question 58

vertigo vs dizziness

Answer 58

Vertigo The illusion of movement: usually rotatory Of patient or surroundings Worse on movement Not Vertigo Faintness Light-headedness Lost awareness Dizziness c¯ Impaired consciousness = blackout w/o impaired consciousness Vertigo: vestibular Imbalance: vestibular, cerebellar, extrapyramidal

Question 59

Causes of Vertigo:

Answer 59

Causes of Vertigo: IMBALANCE Infection / Injury Labyrinthitis: post-viral severe vertigo, n/v Ramsay Hunt Trauma: to petrous temporal bone Meniere’s Recurrent vertigo (~20min) ± n/v Fluctuating SNHL Tinnitus Aural fullness Benign Positional Vertigo Sudden vertigo provoked by head rotation Aminoglycosides / frusemide Lymph, Peri-, fistula Path: Connection between inner and middle ears Causes: Congenital, trauma PC: vertigo, SNHL o/e: Tullio’s phen – nystagmus evoked by loud sound Arterial Migraine TIA / stroke Nerve Acoustic neuroma / vestibular schwannoma Central lesions Demyelination, tumour, infarct (e.g. LMS) Epilepsy Complex partial

Question 60

causes of conductive hearing loss

Answer 60

Conductive: WIDENING Wax or foreign body Infection: otitis media, OME Drum perforation Extra: ossicle discontinuity – otosclerosis, trauma Neoplasia: carcinoma INjury: e.g. barotrauma Granulomatous: Wegener’s, Sarcoid

Question 61

causes of sensorineural hearing loss

Answer 61

Sensorineural: DIVINITY - Developmental Genetic: Alport’s, Waardenburgs Congenital: TORCH Perinatal: anoxia - Degenerative Presbyacusis - Infection VZV, measles, mumps, influenza Meningitis - Vascular Ischaemia: Internal Auditory Artery (AICA) causes sudden hearing loss and vertigo Stroke - Inflammation Vasculitis Sarcoidosis - Neoplasia CPA tumours: acoustic neuroma (commonest cause of unilateral SNHL) - Injury Noise Head trauma - Toxins Gentamicin Frusemide Aspirin - lYmph Endolymphatic Hydrops = Meniere’s Perilymphatic fistula: ruptured round window

Question 62

what is a tremor

Answer 62

Tremor Regular, rhythmic oscillation NB. Asterixis = rhythmic myoclonus

Question 63

types of different tremor

Answer 63

Resting Action / Postural Intention Dystonic Benign Essential Tremor

Question 64

types/causes of a resting tremor

Answer 64

Resting Features 4-6Hz, pill-rolling Abolished on voluntary movement increased c¯ distraction (e.g. counting backwards) Causes: Parkinsonism Rx: Da agonists, antimuscarinic (e.g. procyclidine)

Question 65

types/causes of an action/postural tremor

Answer 65

Action / Postural Features 6-12Hz Absent at rest Worse c¯ outstretched hands or movement Equally bad at all stages of movement Causes: BEATS Benign essential tremor Endocrine: thyroxicosis, drop glucose, phaeo Alcohol withdrawal (or caffeine, opioids…) Toxins: beta-agonists, theophylline, valproate, PHE Sympathetic: physiological tremor may be enhanced: e.g. in anxiety

Question 66

types/causes of an intention tremor

Answer 66

Intention Features \>6Hz, irregular, large amplitude Worse at end of movement E.g. past-pointing Causes: cerebellar damage

Question 67

types/causes of a dystonic tremor

Answer 67

Dystonic Features: variable Causes: mostly idiopathic, as for dystonia

Question 68

characteristics of Benign Essential Tremor

Answer 68

Benign Essential Tremor Autosomal dominant Occur c¯ action and worse c¯ anxiety, emotion, caffeine Arms, neck, voice Doesn’t occur during sleep Better c¯ EtOH

Question 69

what is myoclonus

Answer 69

Sudden, involuntary jerks

Question 70

causes of myoclonus

Answer 70

Causes Metabolic = Asterixis (L, R, increase CO2) Neurodegenerative diseases (LSDs) CJD Myoclonic epilepsies (e.g. infantile spasms) Benign Essential Myoclonus Auto dom Childhood onset frequent generalised myoclonus w/o progression. May respond to valproate

Question 71

what is Benign Essential Myoclonus

Answer 71

Benign Essential Myoclonus Auto dom Childhood onset frequent generalised myoclonus w/o progression. May respond to valproate

Question 72

what is dystonia

Answer 72

Dystonia Prolonged muscle contracture causes unusual joint posture or repetitive movements

Question 73

what is Idiopathic generalised dystonia

Answer 73

Idiopathic generalised dystonia Often autosomal dominant Childhood onset: starts in one leg and spreads on that side of the body over 5-10yrs

Question 74

what is Idiopathic focal dystonia

Answer 74

Idiopathic focal dystonia Commonest form of dystonia Confined to one part of the body Worsened by stress Types: Spasmodic torticollis Blepharospasm Oromandibular Writer’s / Muscician’s cramp

Question 75

causes of Acute Dystonia

Answer 75

Acute Dystonia Torticollis, trismus and/or occulogyric crisis Typically a drug reaction: Neuroleptics Metoclopramide L-DOPA Rx: procyclidine (antimuscarinic)

Question 76

what is and what causes chorea

Answer 76

Chorea Non-rhythmic, purposeless, jerky, flitting movements E.g. facial grimacing, flexing / extending the fingers Causes Huntington’s Sydenham’s Wilson’s L-DOPA Chorea describes involuntary, rapid, jerky movements which often move from one part of the body to another. Slower, sinuous movement of the limbs is termed athetosis. Chorea is caused by damage to the basal ganglia, especially the caudate nucleus. Causes of chorea Huntington's disease, Wilson's disease, ataxic telangiectasia SLE, anti-phospholipid syndrome rheumatic fever: Sydenham's chorea drugs: oral contraceptive pill, L-dopa, antipsychotics neuroacanthocytosis pregnancy: chorea gravidarum thyrotoxicosis polycythaemia rubra vera carbon monoxide poisoning cerebrovascular disease

Question 77

what is and what causes athetosis

Answer 77

Athetosis Slow, sinuous, writhing movements Causes Cerebral palsy Kernitcterus

Question 78

what is and what causes Hemiballismus

Answer 78

Hemiballismus Large amplitude, flinging hemichorea Contralateral to a vascular lesion in the subthalamic nucleus: often elderly diabetics Recovers spontaneously over months

Question 79

classification causes and treatment of tardive syndromes

Answer 79

Tardive Syndromes Delayed onset following chronic exposure to Da agonists (e.g. antipsychotics, antiemetics, L-DOPA) Classification Dyskinesia: orobuccolingual, truncal or choreifirm movements Dystonia: sustained, stereotyped muscle spasms of twitching or turning Akathisia: unpleasant sense of inner restlessness ± repetitive movements (e.g. pacing) Rx Change (e.g. to atypical) or slowly withdraw drug Dyskinesia: Da antagonist (tetrabenazine) Akathisia: beta-Blockers

Question 80

define dementia

Answer 80

Definition Chronically impaired cognition that affects multiple domains: memory, attention, language No impairment of consciousness Acquired and progressive (cf. LD) non-cognitive symptoms such as agitation aggression and apathy can complicate things. causes: - AD - vascular dementia - lewy body dementia - fronto-temporal (pick's) dementia - others eg ETOH, head trauma.

Question 81

alzheimer's disease def, genetics, pathology

Answer 81

Alzheimer's disease is a progressive degenerative disease of the brain accounting for the majority of dementia seen in the UK Alzheimer’s Disease Epi: 50% Path: neurofibrillary tangles and beta amyloid plaques PC: progressive, global cognitive decline RFs: ApoE4 allele, presenillin 1/2 mutations, Down’s Ix: MRI – medial temporal lobe atrophy Rx: cholinesterase inhibitors (donepezil, rivastigmine) if MMSE is 10-20 Alzheimer's disease is a progressive degenerative disease of the brain accounting for the majority of dementia seen in the UK Genetics most cases are sporadic 5% of cases are inherited as an autosomal dominant trait mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14) and presenilin 2 (chromosome 1) genes are thought to cause the inherited form apoprotein E allele E4 - encodes a cholesterol transport protein

Question 82

vascular dementia overview

Answer 82

Vascular Dementia Epi: 20% Path: multiple infarcts PC: sudden onset, stepwise deterioration, patchy deficits, vascular RFs. cumulative effects. Ix: MRI – extensive infarcts or small vessel disease Rx: manage predisposing factors

Question 83

lewy body dementia overview

Answer 83

Lewy Body Dementia Epi: 20% Path: Lewy Bodies in occipito-parital cortex. The characteristic pathological feature is alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas. The relationship between Parkinson's disease and Lewy body dementia is complicated, particularly as dementia is often seen in Parkinson's disease. Also, up to 40% of patients with Alzheimer's have Lewy bodies PC: Fluctuating cognitive dysfunction, visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen), parkinsonism Diagnosis: usually clinical single-photon emission computed tomography (SPECT) is increasingly used. It is currently commercially known as a DaTscan. Dopaminergic iodine-123-radiolabelled 2-carbomethoxy-3-(4-iodophenyl)-N-(3-fluoropropyl) nortropane (123-I FP-CIT) is used as the radioisotope. The sensitivity of SPECT in diagnosing Lewy body dementia is around 90% with a specificity of 100% Rx: cholinesterase inhibitors. Neuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible parkinsonism. Questions may give a history of a patient who has deteriorated following the introduction of an antipsychotic agent avoid antipsychotics if agitated as this can cause deterioration.

Question 84

Frontotemporal Dementia (Pick’s) overview

Answer 84

Frontotemporal Dementia (Pick’s) Epi: \<5% Path: Pick Bodies PC: disinhibition, personality change, early memory preservation, progressive aphasia Ix: MRI – frontal or temporal atrophy

Question 85

treatable causes of dementia

Answer 85

Infection Viral: HIV, HSV, PML Helminth: cysticercosis, toxo Vascular Chronic subdural haematoma Inflammation SLE Sarcoid Neoplasia Nutritional Thiamine deficiency B12 and folate deficiency Pellagra (B3 / niacin deficiency) Hypothyroid Hypoadrenalism Hypercalcaemia Hydrocephalus (normal pressure)

Question 86

what is delirium

Answer 86

Globally impaired cognition and impaired consciousness - consider any acute fluctuating baffling behaviour change as possible delirium. Features Disorientation to person, time and place Reversal of sleep-wake cycle (hyperactive at night) Labile mood Illusions, delusions and hallucinations Cognitive impairment: mem, language, concentration…

Question 87

causes of delirium

Answer 87

Causes: DELIRIUMS Drugs: opioids, sedatives, L-DOPA Eyes, ears and other sensory deficits Low O2 states: MI, stroke, PE Infection Retention: stool or urine Ictal Under- hydration / -nutrition Metabolic: DM, post-op, sodium, uraemia Subdural haemorrhage or other intracranial pathology

Question 88

management of delirium

Answer 88

Ix Bloods: FBC, U+E, LFTs, glucose, ABG Urine dip Septic screen ECG, LP Mx ID and Rx underlying cause Surround c¯ familiar people Nurse in moderately lit, quiet room Find glasses, hearing aids… give repeated reassurance. Avoid sedatives if possible, but if disruptive: Haloperidol 0.5-2mg PO/IM Chlorpromazine 50-100mg PO/IM (avoid in elderly) wait 20min to judge effect

Question 89

causes of acute onset headache

Answer 89

Acute: VICIOUS Vascular Haemorrhage: SAH, intracranial, intracerebral Infarction: esp. posterior circulation Venous: Sinus / cortical thrombosis Infection/Inflammation Meningitis Encephalitis Abscess Compression Obstructive hydrocephalus: tumour Pituitary enlargement: apoplexy ICP Spontaneous intracranial hypotension Acute dural CSF leak causing low pressure headache. Worse on standing initially. Ophthalmic Acute glaucoma - typically elderly. Unknown Situational: cough, exertion, coitus Systemic HTN: Phaeo, PET Infection: sinusitis, tonsillitis, atypical pneumonia Toxins: CO

Question 90

causes of chronic headache

Answer 90

Chronic: MCD TINGS Migraine Cluster headaches Drugs Analgesics Caffeine Vasodilators: Ca2+ antagonists, nitrates Tension headaches ICP up/down UP: tumour, aneurysm, AVM, benign intracranial HTN DOWN: spontaneous intracranial hypotension Neuralgia (trigeminal) Giant cell arteritis Systemic HTN Organ failure: e.g. uraemia

Question 91

headache investigations

Answer 91

Bloods Urine Micro Blood cultures Serology: enterovirus (common cause of viral meningitis), HSV, HIV, syphilis, crypto CSF Radiology Non-contrast CT SAH: blood in sulci, cisterns (white). 90% sensitivity in first 24h MRI MRA: aneurysm MRV: sinus thrombosis Special: CSF Opening pressure (norm = 5-20cm H2 O): UP: SAH, meningitis DOWN: spontaneous intracranial hypotension Xanthochromia: yellow appearance of CSF due to bilirubin. Detect by spectrophotometry.

Question 92

bacterial csf vs tb and viral

Answer 92

bacterial vs TB vs viral appearance - Turbid - Fibrin web - Clear cells - PMN - Lympho / mononuc - Lympho / mononuc count - 100-1000 : 10-1000 : 50-1000 glucose: less than half plasma, less than half, greater than half plasma protein - raised over 1.5, raised 1-5, mildly raised but less than 1

Question 93

define Parkinson’s disease

Answer 93

Parkinson's disease (PD) is a common clinical condition affecting approximately 1% of the population older than 65 years.1 ,2 Many lay people and non-specialists inevitably associate PD with tremor. However, tremor is a presenting feature in only half of all patients with PD, although 90%–100% of patients with PD have tremor at some stage in their disease course.3–6 There are other common causes of tremor—as well as conditions that cause slowness or a change in gait and posture—that can present as PD mimics.7 Our concept of PD has changed from a pure movement disorder to a clinicopathological entity with protean features, including changes in mood, sleep, behaviour, cognition and autonomic function. PD can cause symptoms that lead to referrals to psychiatry, general medicine, care of the elderly, orthopaedic and rheumatology clinics, before arriving at the correct primary underlying diagnosis. A definite diagnosis of PD can only be made at postmortem by identifying degeneration of the substantia nigra pars compacta and the presence of Lewy bodies—insoluble cytoplasmic inclusions containing aggregated alpha-synuclein. Although early diagnosis is occasionally difficult, by the time of death at least 90% of postmortem-proven patients with PD have a correct clinical diagnosis.

Question 94

define motor neuron disease

Answer 94

A motor neuron disease (MND) is any of five neurological disorders that selectively affect motor neurons, the cells that control voluntary muscle activity including speaking, walking, swallowing, and general movement of the body. In the United States the term motor neuron disease is often used interchangeably with amyotrophic lateral sclerosis.[2] The other four motor neuron diseases are: primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy.[2] In the United Kingdom motor neurone disease is used to differentiate this disorder from the other motor neuron diseases though it is also known as amyotrophic lateral sclerosis. To avoid confusion, the annual scientific research conference dedicated to the study of MND is called the "International ALS/MND Symposium".[citation needed] While MND refers to a specific subset of similar diseases, there are numerous other diseases of motor neurons that as a group are referred to as motor neuron disorders.[1] Example include spinal muscular atrophy, spinobulbar muscular atrophy, Charcot–Marie–Tooth disease, Guillain–Barré syndrome, and many others.

Question 95

define dystonia

Answer 95

Dystonia is a disorder characterized by involuntary muscle contractions that cause slow repetitive movements or abnormal postures. The movements may be painful, and some individuals with dystonia may have a tremor or other neurologic features. There are several different forms of dystonia that may affect only one muscle, groups of muscles, or muscles throughout the body. Some forms of dystonia are genetic but the cause for the majority of cases is not known.

Question 96

define Progressive Supranuclear Palsy

Answer 96

Progressive supranuclear palsy (PSP) is a rare brain disorder that causes serious and progressive problems with control of gait and balance, along with complex eye movement and thinking problems. One of the classic signs of the disease is an inability to aim the eyes properly, which occurs because of lesions in the area of the brain that coordinates eye movements. Some individuals describe this effect as a blurring. Affected individuals often show alterations of mood and behavior, including depression and apathy as well as progressive mild dementia. The disorder's long name indicates that the disease begins slowly and continues to get worse (progressive), and causes weakness (palsy) by damaging certain parts of the brain above pea-sized structures called nuclei that control eye movements (supranuclear). PSP was first described as a distinct disorder in 1964, when three scientists published a paper that distinguished the condition from Parkinson's disease. It is sometimes referred to as Steele-Richardson-Olszewski syndrome, reflecting the combined names of the scientists who defined the disorder. Although PSP gets progressively worse, no one dies from PSP itself.

Question 97

define Multiple System Atrophy

Answer 97

Multiple system atrophy is a progressive neurodegenerative disorder characterized by a combination of symptoms that affect both the autonomic nervous system and movement. Shy-Drager syndrome is a type of multiple system atrophy Features parkinsonism autonomic disturbance (atonic bladder, postural hypotension) cerebellar signs Symptoms of autonomic failure that may be seen in MSA include fainting spells and problems with heart rate, erectile dysfunction, and bladder control. Some of these features are similar to those seen in Parkinson’s disease, and early in the disease course it often may be difficult to distinguish these disorders. MSA is a rare disease, affecting potentially 15,000 to 50,000 Americans, including men and women and all racial groups. Symptoms tend to appear in a person’s 50s and advance rapidly over the course of 5 to 10 years, with progressive loss of motor function and eventual confinement to bed. People with MSA often develop pneumonia in the later stages of the disease and may suddenly die from cardiac or respiratory issues. While some of the symptoms of MSA can be treated with medications, currently there are no drugs that are able to slow disease progression and there is no cure. MSA includes disorders that historically had been referred to as Shy-Drager syndrome, olivopontocerebellar atrophy, and striatonigral degeneration.

Question 98

define Corticobasal degeneration

Answer 98

Corticobasal degeneration is a progressive neurological disorder characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia. Corticobasal degeneration progresses gradually. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body (unilateral), but eventually affect both sides as the disease progresses. Symptoms are similar to those found in Parkinson disease, such as poor coordination, akinesia (an absence of movements), rigidity (a resistance to imposed movement), disequilibrium (impaired balance); and limb dystonia (abnormal muscle postures). Other symptoms such as cognitive and visual-spatial impairments, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (muscular jerks), and dysphagia (difficulty swallowing) may also occur. An individual with corticobasal degeneration eventually becomes unable to walk.

Question 99

UK Queen Square Brain Bank Criteria for the diagnosis of Parkinson's disease9

Answer 99

Step 1 Diagnosis of a parkinsonian syndrome Bradykinesia (slow to initiate voluntary movement with progressively reduced speed and amplitude of repetitive actions) and at least one of the following: Muscular rigidity Rest tremor (4–6 Hz) Postural instability unrelated to primary visual, cerebellar, vestibular or proprioceptive dysfunction Step 2 Exclusion criteria of Parkinson's disease History of: Repeated strokes with stepwise progression Repeated head injury Antipsychotics or dopamine-depleting drugs Definite encephalitis and/or oculogyric crisis on no drug treatment More than one affected relative (Note: this exclusion criteria is usually ignored) Sustained remission Negative response to large doses of levodopa (if malabsorption excluded) Strictly unilateral features after 3 years Other neurological features: supranuclear gaze palsy, cerebellar signs, early severe autonomic involvement, Babinski's sign, early severe dementia with disturbance of language, memory or praxis Exposure to known neurotoxin Presence of cerebral tumour or communicating hydrocephalus on neuroimaging Step 3 Supportive criteria for Parkinson's disease Three or more required for the diagnosis of definite Parkinson's disease Unilateral onset Rest tremor present Progressive disorder Persistent asymmetry affecting the side of onset most Excellent response to levodopa (70%–100%) Severe levodopa-induced chorea Levodopa response for more than 5 years Clinical course of over 10 years

Question 100

what does SUNCT syndrome stand for

Answer 100

short-lived unilateral neuralgiform headache with conjunctival injection and tearing

Question 101

dd for headache Acute single episode

Answer 101

meningitis encephalitis subarachnoid haemorrhage head injury sinusitis glaucoma (acute closed-angle) tropical illness e.g. Malaria also poss - temporal arteritis tension headache first migrane

Question 102

dd of Chronic headache

Answer 102

chronically raised ICP Paget's disease psychological medication overuse headache

Question 103

management of parkinson's disease

Answer 103

delay treatment until the onset of disabling symptoms then to introduce a dopamine receptor agonist. Dopamine receptor agonists - e.g. Bromocriptine, ropinirole, cabergoline, apomorphine ergot-derived dopamine receptor agonists (bromocriptine, cabergoline, pergolide\*) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. monitor PTs. warn about impulse control disorders and excessive daytime somnolence Levodopa - usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopamine reduced effectiveness with time (usually by 2 years) unwanted effects: dyskinesia (involuntary writhing movements), 'on-off' effect, dry mouth, anorexia, palpitations, postural hypotension, psychosis, drowsiness MAO-B (Monoamine Oxidase-B) inhibitors - e.g. Selegiline inhibits the breakdown of dopamine secreted by the dopaminergic neurons Amantadine - mechanism is not fully understood, probably increases dopamine release and inhibits its uptake at dopaminergic synapses COMT (Catechol-O-Methyl Transferase) inhibitors - e.g. Entacapone, tolcapone COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy used in conjunction with levodopa in patients with established PD Antimuscarinics- block cholinergic receptors now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease

Question 104

how does sodium valproate work, when is it used and what are some of its side effects

Answer 104

Sodium valproate is used in the management of epilepsy and is first line therapy for generalised seizures. It works by increasing GABA activity. Adverse effects gastrointestinal: nausea increased appetite and weight gain alopecia: regrowth may be curly ataxia tremor hepatitis pancreatitis thromobcytopaenia teratogenic hyponatraemia

Question 105

what is huntington's disease, what are its features and what are the genetics behind it

Answer 105

Huntington's disease is an inherited neurodegenerative condition. It is a progressive and incurable condition that typically results in death 20 years after the initial symptoms develop. Genetics autosomal dominant trinucleotide repeat disorder: repeat expansion of CAG results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia due to defect in huntingtin gene on chromosome 4 Features typical develop after 35 years of age chorea personality changes (e.g. irritability, apathy, depression) and intellectual impairment dystonia saccadic eye movements

Question 106

what are congruous and incongruous visual field defects

Answer 106

A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric. incongruous defects = optic tract lesion; congruous defects = optic radiation lesion or occipital cortex

Question 107

causes of Homonymous hemianopia

Answer 107

Homonymous hemianopia incongruous defects: lesion of optic tract congruous defects: lesion of optic radiation or occipital cortex macula sparing: lesion of occipital cortex

Question 108

what is hemianopia and what is homonymous hemianopia

Answer 108

Hemianopsia or hemianopia is visual field loss on the left or right side of the vertical midline. It can affect one eye but usually affects both eyes. Homonymous hemianopsia, or homonymous hemianopia, is hemianopic visual field loss on the same side of both eyes. Homonymous hemianopsia occurs because the right half of the brain has visual pathways for the left hemifield of both eyes, and the left half of the brain has visual pathways for the right hemifield of both eyes.

Question 109

causes of Homonymous quadrantanopias\*

Answer 109

Homonymous quadrantanopias\* superior: lesion of temporal lobe inferior: lesion of parietal lobe mnemonic = PITS (Parietal-Inferior, Temporal-Superior) \*this is very much the 'exam answer'. Actual studies suggest that the majority of quadrantanopias are caused by occipital lobe lesions. Please see the following link for more details: http://www.ncbi.nlm.nih.gov/pubmed/9109741

Question 110

causes of bitemporal hemianopia

Answer 110

Bitemporal hemianopia lesion of optic chiasm upper quadrant defect \> lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour lower quadrant defect \> upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma

Question 111

what does left homonymous hemianopia mean

Answer 111

left homonymous hemianopia means visual field defect to the left, i.e. Lesion of right optic tract

Question 112

Motor neuron disease: features

Answer 112

Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy There are a number of clues which point towards a diagnosis of motor neuron disease: fasciculation absence of sensory signs/symptoms\* lower motor neuron signs in arms and upper motor neuron signs in legs wasting of the small hand muscles/tibialis anterior is common Other features doesn't affect external ocular muscles no cerebellar signs abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature The diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy. Electromyography shows a reduced number of action potentials with an increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy \*vague sensory symptoms may occur early in the disease (e.g. limb pain) but 'never' sensory signs

Question 113

moa use and problems of benzodiazepines

Answer 113

Benzodiazepines enhance the effect of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA) by increasing the frequency of chloride channels. They therefore are used for a variety of purposes: sedation hypnotic anxiolytic anticonvulsant muscle relaxant Patients commonly develop a tolerance and dependence to benzodiazepines and care should therefore be exercised on prescribing these drugs. The Committee on Safety of Medicines advises that benzodiazepines are only prescribed for a short period of time (2-4 weeks). The BNF gives advice on how to withdraw a benzodiazepine. The dose should be withdrawn in steps of about 1/8 (range 1/10 to 1/4) of the daily dose every fortnight. A suggested protocol for patients experiencing difficulty is given: switch patients to the equivalent dose of diazepam reduce dose of diazepam every 2-3 weeks in steps of 2 or 2.5 mg time needed for withdrawal can vary from 4 weeks to a year or more

Question 114

features of benzodiazepine withdrawal

Answer 114

If patients withdraw too quickly from benzodiazepines they may experience benzodiazepine withdrawal syndrome, a condition very similar to alcohol withdrawal syndrome. This may occur up to 3 weeks after stopping a long-acting drug. Features include: insomnia irritability anxiety tremor loss of appetite tinnitus perspiration perceptual disturbances seizures

Question 115

management of alzheimer's

Answer 115

Management NICE now recommend the three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer's disease memantine (a NMDA receptor antagonist) is reserved for patients with moderate - severe Alzheimer's

Question 116

Nerve, Functions, Clinical, Pathway/foramen of CN1

Answer 116

I (Olfactory) Smell Cribriform plate

Question 117

Nerve, Functions, Clinical, Pathway/foramen of CN2

Answer 117

II (Optic) Sight Optic canal

Question 118

Nerve, Functions, Clinical, Pathway/foramen of CN3

Answer 118

III (Oculomotor) Eye movement (MR, IO, SR, IR) Pupil constriction Accomodation Eyelid opening Palsy results in ptosis 'down and out' eye dilated, fixed pupil Superior orbital fissure (SOF)

Question 119

Nerve, Functions, Clinical, Pathway/foramen of CN4

Answer 119

IV (Trochlear) Eye movement (SO) Palsy results in defective downward gaze → vertical diplopia Superior orbital fissure (SOF)

Question 120

Nerve, Functions, Clinical, Pathway/foramen of CN5

Answer 120

V (Trigeminal) Facial sensation Mastication Lesions may cause: trigeminal neuralgia loss of corneal reflex (afferent) loss of facial sensation paralysis of mastication muscles deviation of jaw to weak side V1: SOF, V2: Foramen rotundum, V3: Foramen ovale

Question 121

Nerve, Functions, Clinical, Pathway/foramen of CN6

Answer 121

VI (Abducens) Eye movement (LR) Palsy results in defective abduction → horizontal diplopia SOF

Question 122

Nerve, Functions, Clinical, Pathway/foramen of CN7

Answer 122

VII (Facial) Facial movement Taste (anterior 2/3rds of tongue) Lacrimation Salivation Lesions may result in: flaccid paralysis of upper + lower face loss of corneal reflex (efferent) loss of taste hyperacusis = Hyperacusis (also spelled hyperacousis) is a health condition characterized by an over-sensitivity to certain frequency and volume ranges of sound (a collapsed tolerance to usual environmental sound). A person with severe hyperacusis has difficulty tolerating everyday sounds, some of which may seem unpleasantly or painfully loud to that person but not to others. Internal auditory meatus

Question 123

Nerve, Functions, Clinical, Pathway/foramen of CN8

Answer 123

VIII (Vestibulocochlear) Hearing, balance Hearing loss Vertigo, nystagmus Acoustic neuromas are Schwann cell tumours of the cochlear nerve Internal auditory meatus

Question 124

Nerve, Functions, Clinical, Pathway/foramen of CN9

Answer 124

IX (Glossopharyngeal) Taste (posterior 1/3rd of tongue) Salivation Swallowing Mediates input from carotid body & sinus Lesions may result in; hypersensitive carotid sinus reflex loss of gag reflex (afferent) Jugular foramen

Question 125

Nerve, Functions, Clinical, Pathway/foramen of CN10

Answer 125

X (Vagus) Phonation Swallowing Innervates viscera Lesions may result in; uvula deviates away from site of lesion loss of gag reflex (efferent) Jugular foramen

Question 126

Nerve, Functions, Clinical, Pathway/foramen of CN11

Answer 126

XI (Accessory) Head and shoulder movement Lesions may result in; weakness turning head to contralateral side Jugular foramen

Question 127

Nerve, Functions, Clinical, Pathway/foramen of CN12

Answer 127

XII (Hypoglossal) Tongue movement Tongue deviates towards side of lesion Hypoglossal canal

Question 128

Some cranial nerves are motor, some sensory and some are both. The most useful mnemonic is given below.

Answer 128

Some Say Marry Money But My Brother Says Big Brains Matter Most S = Sensory, M = Motor, B = Both

Question 129

Reflex, Afferent limb, Efferent limb - corneal

Answer 129

Corneal Ophthalmic nerve (V1) Facial nerve (VII)

Question 130

Reflex, Afferent limb, Efferent limb - jaw jerk

Answer 130

Jaw jerk Mandibular nerve (V3) Mandibular nerve (V3)

Question 131

Reflex, Afferent limb, Efferent limb - gag

Answer 131

Gag Glossopharyngeal nerve (IX) Vagal nerve (X)

Question 132

Reflex, Afferent limb, Efferent limb - carotid sinus

Answer 132

Carotid sinus Glossopharyngeal nerve (IX) Vagal nerve (X)

Question 133

Reflex, Afferent limb, Efferent limb - pupillary light

Answer 133

Pupillary light Optic nerve (II) Oculomotor nerve (III)

Question 134

Reflex, Afferent limb, Efferent limb - lacrimation

Answer 134

Lacrimation Ophthalmic nerve (V1) Facial nerve (VII)

Question 135

what is aphasia

Answer 135

Aphasia from Greek a- ("without") + phásis (φάσις, "speech")) is the name given to a collection of language disorders caused by damage to the brain.[1][2] The word aphasia comes from the word ἀφασία aphasia, in Ancient Greek, which means[3] "speechlessness",[4] derived from ἄφατος aphatos, "speechless"[5] from ἀ- a-, "not, un" and φημί phemi, "I speak". A requirement for a diagnosis of aphasia is that, prior to the illness or injury, the person's language skills were normal (for developmental language disorders, see specific language impairment). The difficulties of people with aphasia can range from occasional trouble finding words to losing the ability to speak, read, or write, but does not affect intelligence.[1] This also affects visual language such as sign language.[2] The term "aphasia" implies a problem with one or more functions that are essential and specific to language function. It is not usually used when the language problem is a result of a more peripheral motor or sensory difficulty, such as paralysis affecting the speech muscles or a general hearing impairment.

Question 136

what is dysarthria

Answer 136

Dysarthria (from Ancient Greek δυσ- dys, "hard, difficult, bad" and ἄρθρωσις arthrosis, "articulation") is a motor speech disorder resulting from neurological injury of the motor component of the motor-speech system[1] and is characterized by poor articulation of phonemes (cf. aphasia: a disorder of the content of language).[2] In other words, it is a condition in which problems effectively occur with the muscles that help produce speech, often making it very difficult to pronounce words. It is unrelated to any problem with understanding cognitive language.[3] Any of the speech subsystems (respiration, phonation, resonance, prosody, and articulation) can be affected, leading to impairments in intelligibility, audibility, naturalness, and efficiency of vocal communication.[4]

Question 137

what are the different types of aphasia

Answer 137

Wernicke's (receptive) aphasia Broca's (expressive) aphasia Conduction aphasia Global aphasia

Question 138

what is Wernicke's (receptive) aphasia

Answer 138

Due to a lesion of the superior temporal gyrus This area 'forms' the speech before 'sending it' to Broca's area. Lesions result in sentences that make no sense, word substitution and neologisms but speech remains fluent Comprehension is impaired

Question 139

what is Broca's (expressive) aphasia

Answer 139

Due to a lesion of the inferior frontal gyrus Speech is non-fluent, laboured, and halting Comprehension is normal

Question 140

what is Conduction aphasia

Answer 140

Classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke's and Broca's area Speech is fluent but repetition is poor. Aware of the errors they are making Comprehension is normal

Question 141

what is Global aphasia

Answer 141

arcuate fasiculus inferior frontal gyrus superior temporal gyrus Large lesion affecting all 3 of the above areas resulting in severe expressive and receptive aphasia

Question 142

how is epilepsy classified

Answer 142

Basics two main categories are generalised and partial seizures partial seizures may progress to general seizures other types: myoclonic, atypical absence, atonic and tonic seizures are usually seen in childhood Generalised - no focal features, consciousness lost immediately grand mal (tonic-clonic) petit mal (absence seizures) myoclonic: brief, rapid muscle jerks partial seizures progressing to generalised seizures Partial - focal features depending on location simple (no disturbance of consciousness or awareness) complex (consciousness is disturbed) temporal lobe → aura, déjà vu, jamais vu; motor → Jacksonian

Question 143

what is myotonic dystrophy, what are the genetics and different types.

Answer 143

Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2. Genetics autosomal dominant a trinucleotide repeat disorder DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19 DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3 The key differences are listed in table below: DM1 DM2 - DMPK gene on chromosome 19 - Distal weakness more prominent DM2 - ZNF9 gene on chromosome 3 - Proximal weakness more prominent - Severe congenital form not seen

Question 144

physical features of myotonic dystrophy

Answer 144

General features myotonic facies (long, 'haggard' appearance) frontal balding bilateral ptosis cataracts dysarthria Other features myotonia (tonic spasm of muscle) weakness of arms and legs (distal initially) mild mental impairment diabetes mellitus testicular atrophy cardiac involvement: heart block, cardiomyopathy dysphagia

Question 145

what are the triptans and when are they used and what are the problems

Answer 145

Triptans are specific 5-HT1 agonists used in the acute treatment of migraine. They are generally used first-line in combination therapy with an NSAID or paracetamol. Prescribing points should be taken as soon as possible after the onset of headache, rather than at onset of aura oral, orodispersible, nasal spray and subcutaneous injections are available Adverse effects 'triptan sensations' - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure Contraindications patients with a history of, or significant risk factors for, ischaemic heart disease or cerebrovascular disease

Question 146

what is idiopathic intracranial hypertension, what are the features and risk factors.

Answer 146

Idiopathic intracranial hypertension (also known as pseudotumour cerebri and formerly benign intracranial hypertension) is a condition classically seen in young, overweight females. Features headache blurred vision papilloedema (usually present) enlarged blind spot sixth nerve palsy may be present Risk factors obesity female sex pregnancy drugs\*: oral contraceptive pill, steroids, tetracycline, vitamin A

Question 147

what is the management of idiopathic intracranial hypertension

Answer 147

Management weight loss diuretics e.g. acetazolamide topiramate is also used, and has the added benefit of causing weight loss in most patients repeated lumbar puncture surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure

Question 148

what is Acute angle closure glaucoma and what are its features

Answer 148

Glaucoma is a group disorders characterised by optic neuropathy due, in the majority of patients, to raised intraocular pressure (IOP). It is now recognised that a minority of patients with raised IOP do not have glaucoma and vice versa In acute angle closure glaucoma (AACG) there is a rise in IOP secondary to an impairment of aqueous outflow. Factors predisposing to AACG include: hypermetropia (long-sightedness) pupillary dilatation lens growth associated with age Features severe pain: may be ocular or headache decreased visual acuity symptoms worse with mydriasis (e.g. watching TV in a dark room) hard, red eye haloes around lights semi-dilated non-reacting pupil corneal oedema results in dull or hazy cornea systemic upset may be seen, such as nausea and vomiting and even abdominal pain seek expert help immediately - start acetazolamide 500mg IV over several minutes.

Question 149

what is the management of Acute angle closure glaucoma

Answer 149

Management urgent referral to an ophthalmologist management options include reducing aqueous secretions with acetazolamide and inducing pupillary constriction with topical pilocarpine

Question 150

Stroke by anatomy - Contralateral hemiparesis and sensory loss, lower extremity \> upper

Answer 150

Anterior cerebral artery

Question 151

Stroke by anatomy - Contralateral hemiparesis and sensory loss, upper extremity \> lower Contralateral homonymous hemianopia Aphasia

Answer 151

Middle cerebral artery

Question 152

Stroke by anatomy - Contralateral homonymous hemianopia with macular sparing Visual agnosia

Answer 152

Posterior cerebral artery

Question 153

Stroke by anatomy - Ipsilateral CN III palsy Contralateral weakness

Answer 153

Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain)

Question 154

Stroke by anatomy - Ipsilateral: facial pain and temperature loss Contralateral: limb/torso pain and temperature loss Ataxia, nystagmus

Answer 154

Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)

Question 155

Stroke by anatomy - Symptoms are similar to Wallenberg's (see above), but: Ipsilateral: facial paralysis and deafness

Answer 155

Anterior inferior cerebellar artery (lateral pontine syndrome)

Question 156

Stroke by anatomy - Amaurosis fugax

Answer 156

Retinal/ophthalmic artery

Question 157

Stroke by anatomy - 'Locked-in' syndrome

Answer 157

Basilar artery

Question 158

Lacunar strokes

Answer 158

Lacunar stroke or lacunar infarct (LACI) is a type of stroke that results from occlusion of one of the penetrating arteries that provides blood to the brain's deep structures. present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia strong association with hypertension common sites include the basal ganglia, thalamus and internal capsule

Question 159

sympathetic NS structure

Answer 159

Sympathetic NS Cell bodies from T1-L2 GVE preganglionic fibres synapse @ either: - Paravertebral ganglia - Prevertebral ganglia - Chromaffin cells of adrenal medulla Preganglionic fibres are myelinated and release ACh @ nicotinic receptors Postganglionic fibres are unmyelinated and release NA @ adrenergic receptors - Except @ sweat glands where they release ACh for muscarinic receptors.

Question 160

parasympathetic NS structure

Answer 160

Parasympathetic NS Cranial: CN 3, 7, 9, 10 - Ciliary: ciliary muscle and sphincter pupillae - Pterygopalatine: mucus mems of nose and palate, lacrimal gland - Submandibular: submandibular and sublingual glands - Otic: parotid gland - Vagus supplies thoracic and abdo viscera Sacral: pelvic splanchnic nerves (S2-4) innervate pelvic viscera - Preganglionic fibres release ACh @ nicotinic receptors - Postganglionic fibres release ACh @ muscarinic receptors

Question 161

key features of specific headache causes - Venous Sinus Thrombosis

Answer 161

most commonly sagittal sinus thrombosis. or transverse sinus. - symptoms come on gradually over days or weeks. may extend to cortical veins and cause infarction within a venous territory. - headache, papilloedema, CNS signs. siezures. - cavernous sinus specifically can be due to spread from facial pustules. get eye problems. ix - exclude SAH, meningitis, encephalitis. - CT/MRI venography may show the absence of a sinus. MRI t2 weighted can visulaise thrombus directly. Mx - heparin improves outcome.

Question 162

key features of specific headache causes - Cortical Vein Thrombosis

Answer 162

- often causes venous infarcts with stroke like focal symptoms that develop over days. however, the associated headache may coe on suddenly eg thunderclap. siezures are common and focal, unlike arterial stroke. - signs - encephalopathy, focal siezure, headache, slowely evolving focal defecits. same Ix and Rx as venous sinus thrombosis.

Question 163

key features of specific headache causes - Tension Headache

Answer 163

Recurrent, non-disabling, bilateral headache, often described as a 'tight-band' Not aggravated by routine activities of daily living

Question 164

key features of specific headache causes - Migraine

Answer 164

Recurrent, severe headache which is usually unilateral and throbbing in nature May be be associated with aura, nausea and photosensitivity Aggravated by, or causes avoidance of, routine activities of daily living. Patients often describe 'going to bed'. In women may be associated with menstruation

Question 165

key features of specific headache causes - Cluster

Answer 165

Pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours with clusters typically lasting 4-12 weeks Intense pain around one eye (recurrent attacks 'always' affect same side) Patient is restless during an attack Accompanied by redness, lacrimation, lid swelling More common in men and smokers

Question 166

key features of specific headache causes - Hemicrania

Answer 166

Hemicrania continua (HC) is a persistent unilateral headache that responds to indomethacin. It is usually unremitting, but rare cases of remission have been documented.[1] Hemicrania continua is considered a primary headache disorder, meaning that it is not caused by another condition. All of the following characteristics: Unilateral pain without side-shift Daily and continuous, without pain-free periods Moderate intensity, but with exacerbations of severe pain

Question 167

key features of specific headache causes - Analgesia Overuse

Answer 167

Present for 15 days or more per month Developed or worsened whilst taking regular symptomatic medication Patients using opioids and triptans are at most risk May be psychiatric co-morbidity

Question 168

key features of specific headache causes - Giant Cell Arteritis

Answer 168

Typically patient \> 60 years old Usually rapid onset (e.g. \< 1 month) of unilateral headache Jaw claudication (65%) Tender, palpable temporal artery Raised ESR

Question 169

Migraine symptoms and diagnostic criteria

Answer 169

Headache Aura lasting 15-30min then unilat, throbbing headache Phono/photophobia n/v Allodynia Often premenstrual Prodrome (50%): precede migraine by hrs - days Yawning Food cravings Changes in sleep, appetite or mood Aura (20%): precedes migraine by mins and may persist Visual: distortion, lines, dots, zig-zags, scotoma, hemianopia Sensory: paraesthesia (fingers to face) Motor: dysarthria, ataxia, ophthalmoplegia, hemiparesis (hemiplegic migraine) Speech: dysphasia, paraphasia

Question 170

migrane treatment

Answer 170

Treatment Acute episode 1st: Paracetamol + metoclopramide / domperidone 2nd: NSAID (e.g. ketoprofen) + M/D 3rd: Rizatriptan CI: IHD, uncontrolled HTN, SSRIs 4th: ergotamine Prophylaxis Avoid triggers 1st: Propanolol, topiramate 2nd: Valproate, pizotifen (increases wt.), gabapentin

Question 171

SAH investigation and management

Answer 171

CT Detects \>90% of SAH w/i first 48hrs LP If CT-ve and no CIs \>12h after start of headache Xanthochromia due to breakdown of bilirubin Mx Frequent neuro obs: pupils, GCS, BP Maintain CPP: keep SBP \>160 Nimodipine for 3wks causes decreased cerebral vasospasm Endovascular coiling (preferable to surgical clipping

Question 172

stroke definition

Answer 172

Rapid onset, focal neurological deficit due to a vascular lesion lasting \>24h Infarction due ischaemia (80%) or intracerebral haemorrhage (20%).

Question 173

Oxford / Bamford Classification of stroke

Answer 173

Oxford / Bamford Classification Based on clinical localisation of infarct S=syndrome: prior to imaging I=infarct: after imaging when atheroembolic infarct confirmed The registrar wants his junior to use the Bamford classifi cation system. This woman has motor weakness and higher cortical dysfunction (dysphasia), therefore scoring 2/3 (she doesn’t score for hemianopia). Cerebellar or brainstem signs indicate a posterior circulation infarct, whilst A does not exist. The system is quick and easy to use, adds weight to out-of-hours radiology or neurology referrals (‘Hi, I have a 78-yearold who’s having what looks like an acute PACI on the ward . . . ’), and is informative with regard to prognosis. The defi nitions according to the Bamford classifi cation system are: ● Total anterior circulation infarct (TACI). All of the following: • Higher dysfunction (decreased level of consciousness, dysphasia, visuospatial) * Homonymous hemianopia * Motor/sensory defi cit (\>2/3 face/arm/leg). ● Partial anterior circulation infarct (PACI). Any two of the three features of TACI or: * Higher dysfunction alone or * Limited motor sensory defi cit. ● Posterior circulation infarct (POCI). Any of the following: * Cranial nerve palsy and CL motor/sensory defi cit * Bilateral motor/sensory defi cit * Conjugate eye movement problems * Cerebellar dysfunction * Isolated homonymous hemianopia ● Lacunar infarct (LACI). Any of the following (all aff ecting \>2/3 face/ arm/leg): * Pure sensory defi cit * Pure motor defi cit * Sensorimotor defi cit * Ataxic hemiparesis. The patient must not have new dysphasia, visuospatial problem, proprioceptive loss, any vertebrobasilar features.

Question 174

acute management of stroke

Answer 174

Resuscitate Ensure patent airway: consider NGT NBM until swallowing assessed by SALT Don’t overhydrate: risk of cerebral oedema Monitor Glucose: 4-11mM: sliding scale if DM BP: \<185/110 (for thrombolysis) Rx of HTN can decrease cerebral perfusion Neuro obs Imaging Urgent CT/MRI Diffusion-weighted MRI is most sensitive for acute infarct CT will exclude primary haemorrhage Medical Consider thrombolysis if 18-80yrs and \<4.5hrs since onset of symptoms Alteplase (recombinant tissue plasminogen activator - 0.9mg/kg over 1hr.) decreases death and dependency (OR 0.64) CT 24h post-thrombolysis to look for haemorrhage Aspirin 300mg PO/PR once haemorrhagic stroke excluded ± PPI Clopidogrel if aspirin sensitive Surgery Neurosurgical opinion if intracranial haemorrhage May coil bleeding aneurysms Decompressive hemicraniectomy for some forms of MCA infarction. Stroke Unit Specialist nursing and physio Early mobilisation DVT prophylaxis Secondary Prevention Rehabilitation

Question 175

primary prevention of stroke

Answer 175

Primary Prevention Control RFs: HTN, lipids, DM, smoking, cardiac disease Consider life-long anticoagulation in AF (use CHADS2) Carotid endarterectomy if symptomatic 70% stenosis Exercise

Question 176

secondary prevention of stroke

Answer 176

Secondary Prevention Risk factor control Start a statin after 48h Aspirin / clopi 300mg for 2wks after stroke then either Clopidogrel 75mg OD (preferred option) Aspirin 75mg OD + dipyridamole MR 200mg BD Warfarin instead of aspirin/clopidogrel if Cardioembolic stroke or chronic AF Start from 2wks post-stroke (INR 2-3) Don’t use aspirin and warfarin together. Carotid endarterectomy if good recovery + ipsilat stenosis greater than 70%

Question 177

stroke rehabilitation

Answer 177

Rehabilitation: MENDS MDT: physio, SALT, dietician, OT, specialist nurses, neurologist, family Eating Screen swallowing: refer to specialist, NG/PEG if unable to take oral nutrition Screen for malnutrition (MUST tool), Supplements if necessary Neurorehab: physio and speech therapy Botulinum can help spasticity DVT Prophylaxis Sores: must be avoided @ all costs

Question 178

what is a TIA

Answer 178

Sudden onset focal neurology lasting \<24h due to temporary occlusion of part of the cerebral circulation. ~15% of 1st strokes are preceded by TIAs.

Question 179

TIA management

Answer 179

1 - Aim to find cause and 2 - define vascular risk: 1 - Bloods: FBC, U&E, ESR glucose (hypo), lipids. - looking for causes of hyperviscosity CXR ECG - AF Echo - mural thrombus post MI, valve problems. Carotid doppler ± angiography. atherothromboembolism from the carotid is the chief cause. Consider brain imaging - vasculitis is a rare cause. Diffusion weighted MRI is best 2 - ABCD2 score This gives a total score ranging from 0 to 7. People who have had a suspected TIA who are at a higher risk of stroke (that is, with an ABCD2 score of 4 or above) should have: - aspirin (300 mg daily) started immediately - specialist assessment and investigation within 24 hours of onset of symptoms - risk factor secondary prevention If the ABCD2 risk score is 3 or below: - specialist assessment within 1 week of symptom onset, including decision on brain imaging People with crescendo TIAs (two or more episodes in a week) should be treated as being at high risk of stroke, even though they may have an ABCD2 score of 3 or below. With regards to carotid artery endarterectomy: recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled should only be considered if carotid stenosis \> 70% according ECST\* criteria or \> 50% according to NASCET\*\* criteria \*European Carotid Surgery Trialists' Collaborative Group \*\*North American Symptomatic Carotid Endarterectomy Trial Differential Vascular: CVA, migraine, GCA Epilepsy Hyperventilation Hypoglycaemia Mx: ACAS Time to intervention is crucial. Intervention w/i 72hrs results in 2% strokes @ 90d Intervention w/i 3wks results in 10% strokes @ 90d Avoid driving for 1mo 1. Antiplatelet Therapy / Anticoagulate Aspirin/clopi 300mg/d for 2wks then 75mg/d Add dipyridamole MR to aspirin Warfarin if cardiac emboli: AF, MI, MS After 2wks 2. Cardiac Risk Factor Control BP, lipids, DM, smoking Exercise Diet: decrease salt 3. Assess risk of subsequent stroke ABCD2 score 4. Specialist referral to TIA clinic ABCD2 greater than or equal to 4: w/i 24hrs ABCD2 \<4: w/i 1wk

Question 180

when is Carotid Endarterectomy done after TIA

Answer 180

Carotid Endarterectomy Beneficial if greater than 70% symptomatic stenosis 50-70% stenosis may benefit if operative risk is \<3% Surgery should be performed w/i 2wks. Endovascular stenting is an alternative, but safety and long-term benefits (in-stent restenosis is common) are still under Ix. Major complications are stroke and death.

Question 181

what is the ABCD2 Score

Answer 181

ABCD2 Score Predicts stroke risk following TIA Score at least 6 = 8% risk w/i 2d, 35% risk w/i 1wk Score at least 4 = pt. assessment by specialist w/i 24hrs All pts with suspected TIA should be seen by specialist w/i 7d. 1. Age at least 60 2. BP at least 140/90 3. Clinical features a. Unilateral weakness (2 points) b. Speech disturbance w/o weakness 4. Duration a. at least 1h (2 points) b. 10-59min 5. DM NB. 7 points max. People who have had a suspected TIA who are at a higher risk of stroke (that is, with an ABCD2 score of 4 or above) should have: aspirin (300 mg daily) started immediately specialist assessment and investigation within 24 hours of onset of symptoms measures for secondary prevention introduced as soon as the diagnosis is confirmed, including discussion of individual risk factors If the ABCD2 risk score is 3 or below: specialist assessment within 1 week of symptom onset, including decision on brain imaging if vascular territory or pathology is uncertain, refer for brain imaging People with crescendo TIAs (two or more episodes in a week) should be treated as being at high risk of stroke, even though they may have an ABCD2 score of 3 or below. Antithrombotic therapy clopidogrel is recommended first-line (as for patients who've had a stroke) aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel these recommendations follow the 2012 Royal College of Physicians National clinical guideline for stroke. Please see the link for more details (section 5.5) these guidelines may change following the CHANCE study (NEJM 2013;369:11). This study looked at giving high-risk TIA patients aspirin + clopidogrel for the first 90 days compared to aspirin alone. 11.7% of aspirin only patients had a stroke over 90 days compared to 8.2% of dual antiplatelet patients

Question 182

what is a Subdural Haemorrhage

Answer 182

Bleeding from bridging veins between cortex and sinuses Haematoma between dura and arachnoid Often due to minor trauma that occurred a long time previously – especially deceleration injuries Imaging: CT / MRI Crescentic haematoma over one hemisphere Clot goes from white goes to grey c¯ time Mid-line shift Bleeding into the outermost meningeal layer. Most commonly occur around the frontal and parietal lobes. Risk factors include old age, alcoholism and anticoagulation. Slower onset of symptoms than a epidural haematoma. CONSCIOUS level fluctuation. the trauma can be so long ago as to be forgotten.

Question 183

management of Subdural Haemorrhage

Answer 183

Mx 1st line: irrigation/evacuation via burr-hole craniostomy 2nd line: craniotomy Address causes of trauma

Question 184

what is a Extradural Haemorrhage

Answer 184

Extradural Haemorrhage Often due to # temporal or parietal bone causing laceration of middle meningeal artery and vein. Blood between bone and dura Suspect if after head injury GCS falls, is slow to improve or there is a lucid interval. Presentation Lucid Interval Deterioration of GCS after head injury that caused no LOC, or following initial improvement in GCS. Lucid interval may be hrs or days ICP Headache Vomiting Confusion then coma Fits Ipsilateral blown pupil (3rd nerve palsy) ± hemiparesis c¯ upgoing plantars and upgoing reflexes Brainstem Compression Deep irregular breathing (brainstem compression) Cushing response (increase BP, decrease HR) is late Death by cardiorespiratory arrest Imaging: CT Lens-shaped haematoma Skull # (greatly increase risk of extradural haemorrhage) Bleeding into the space between the dura mater and the skull. Often results from acceleration-deceleration trauma or a blow to the side of the head. The majority of epidural haematomas occur in the temporal region where skull fractures cause a rupture of the middle meningeal artery. Features features of raised intracranial pressure some patients may exhibit a lucid interval

Question 185

Extradural Haemorrhage management

Answer 185

Mx Neuroprotective ventilation (O2\>100, CO2 35-40) Consider mannitol (1g/kg IV via central line) Craniectomy for clot evacuation and vessel ligation

Question 186

features of meningitis

Answer 186

Meningitic Headache Neck stiffness Kernig’s: Straightening leg c¯ hip @ 90O Brudzinski’s: lifting head causes lifting of legs Photophobia n/v Neurological decreased GCS then coma Seizures (20%) Focal neuro (20%): e.g. CN palsies Septic Fever decreased BP, increased HR increased CRT Purpuric rash DIC

Question 187

acute management of meningitis

Answer 187

step 1 - ABC O2 15L – SpO2 94-98% IVI fluid resus c¯ crystalloid step 2 - if Mainly Septicaemic Don’t attempt LP Ceftriaxone 2g IVI Consider ITU if shocked or step 2 - if Mainly Meningitic If no shock or CIs do LP Dexamethasone 0.15mg/kg IV QDS Ceftriaxone 2g IVI post-LP step 3 - Continuing Management Ceftriaxone 2g BD IVI Meningococcus: 7d IV then review Pneumococcus: 14d IV then review Maintenance fluids UO 30ml/h SBP \>80mmHg If response is poor, consider intubation ± inotropic support Rifampicin prophylaxis for household contacts.

Question 188

CIs to LP: Try LP Unless ContraINdicated

Answer 188

CIs to LP: Try LP Unless ContraINdicated Thrombocytopenia Lateness (delay in antibiotic admin) Pressure (signs of raised ICP) Unstable (Cardio + resp systems) Coagulation disorder Infection at LP site Neurology (focal neurological signs)

Question 189

presentation of encephalitis

Answer 189

Presentation Infectious prodrome: fever, rash, LNs, cold sores, conjunctivitis, meningeal signs. Bizarre behaviour or personality change Confusion decreased GCS then coma Fever Headache Focal neuro Seizures Hx of travel or animal bite Causes Usually viral HSV1/2 CMV, EBV, VZV Arboviruses HIV

Question 190

investigation and management of encephalitis

Answer 190

Ix Bloods: cultures, viral PCR, malaria film Contrast CT: focal bilat temporal involvement suggests HSV LP: increased CSF protein, lymphocytes, PCR EEG: shows diffuse abnormalities, may confirm Dx Mx Aciclovir STAT: 10mg/kg/8h IVI over 1h for 14/7 Supportive measures in HDU/ITU Phenytoin for seizures Prognosis 70% mortality if untreated

Question 191

signs of a Cerebral Abscess

Answer 191

Signs Seizures Fever Localizing signs Signs of increased ICP Signs of infection elsewhere Ix CT/MRI: ring-enhancing lesion increased WCC, increased ESR Pre-disposing Factors Infection: ear, sinus, dental or periodontal Skull # Congenital heart disease Endocarditis Bronchiectasis Immunosuppression

Question 192

treatment of a cerebral abscess

Answer 192

Rx Neurosurgical referral Abx: e.g. ceftriaxone Treat raised ICP

Question 193

define epilepsy

Answer 193

Recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifest as seizures.

Question 194

types of epilepsy by features

Answer 194

Partial siezures: 1- simple partial 2 - complex partial 3 - partial with secondary generalization - can start as either simple or complex focal. primary generalised siezures 1 - absence 2 - tonic-clonic 3 - myoclonic 4 - atonic (akinetic) 5 - infantile spasms

Question 195

what is a Simple Partial siezure

Answer 195

Focal motor, sensory, autonomic or psychic symptoms. awareness unimpaired. no post ictal

Question 196

what is a Complex Partial siezure

Answer 196

Complex Partial: 5As Aura Autonomic: change in skin colour, temperature, palps Awareness lost: motor arrest, motionless stare Automatisms: lip-smacking, fumbling, chewing, swallowing Amnesia NB. Usually arise from temporal lobe

Question 197

what is a Absences (Petit mal) siezure

Answer 197

Absences (Petit mal) ABrupt onset and offset Short: \<10s Eyes: glazed, blank-stare Normal: intelligence, examination, brain-scan Clonus or automatisms may occur EEG: 3Hz spike and wave Stimulated by hyperventilation and photics suddenly stops talking mid sentence then carries on where left off.

Question 198

what is a Tonic-Clonic (Grand mal) siezure

Answer 198

Tonic-Clonic (Grand mal) LOC Tonic: limbs stiffen Clonic: rhythmic jerking of limbs ± cyanosis, incontinence, tongue biting (lateral) Post-ictal confusion and drowsiness

Question 199

what is a Myoclonic Seizure

Answer 199

Myoclonic Seizure Sudden jerk of limb, face or trunk.

Question 200

what is a Atonic (akinetic seizures)

Answer 200

Atonic (akinetic seizures) Sudden loss of muscle tone then fall No LOC

Question 201

Localising Features of siezures

Answer 201

Temporal Automatisms: lip smacking, chewing, fumbling Deja/jamias vu Delusional behaviour Abdominal: rising, n/v Emotional disturbance: terror, panic, anger, elation Tastes, smells Frontal Motor features: arrest, Jacksonian march, Todd’s palsy Parietal Sensory disturbance: tingling, numbness Occipital Visual phenomena: spots, lines, flashes

Question 202

general principles of epilepsy management

Answer 202

General Principles After any seizure advise against driving, swimming, et.c. until a Dx is established Don’t diagnose epilepsy from a single seizure Diagnosis should be made by a specialist After Dx, cannot drive until seizure-free for \>1yr 10yrs for HGV (no meds)

Question 203

epilepsy investigation

Answer 203

Investigations Basic bloods: FBC, U+Es, glucose Se raised Prl 10min after fit (relative to baseline) Se AED levels Urine toxicology ECG EEG Use to support Dx (cannot exclude or prove) Helps classification and prognosis Use c¯ hyperventilation and photic stimulation Neuroimaging: typically MRI Not routine for idiopathic generalised epilepsy Indications Developed epilepsy as an adult Any evidence of focal onset Seizures continue despite 1st-line Rx

Question 204

first and second line drug therapy for epilepsy

Answer 204

Seizure Type 1st line 2nd line Tonic-Clonic Valproate then Lamotrigine Absences Valproate and Ethosuximide, thenLamotrigine Tonic, atonic or myoclonic:Valproate then Levetiracetam Focal ± 2nd gen: Lamotrigine then CBZ

Question 205

what is status epilepticus

Answer 205

Definition Seizure lasting \>30min, or Repeated seizures w/o intervening consciousness

Question 206

acute management of status epilepticus

Answer 206

1 - ABC Oral / nasal airway, intubate Suction 100% O2 Capillary blood glucose 2 - IV Access + Bloods U+E, LFT, FBC, Glucose, Ca2+ AED levels Tox screen 3 - Reverse Potential Causes Thiamine 250mg IV if EtOH 100ml 20% glucose unless glucose known to be normal 4 - Slow IV Bolus Phase Lorazepam 2-4mg IV 2nd dose if no response w/i 2min 5 - IV Infusion Phase Phenytoin 18mg/kg IVI (then 100mg/6-8h) Or, diazepam 100mg in 500ml 5% dex IVI 6 - RSI Phase Never spend \>20min c¯ someone in status w/o getting an anaesthetist

Question 207

Head Injury Initial Mx

Answer 207

primary Survey A: ? intubation, immobilise C-spine B: 100% O2, RR C: IV access, BP, HR D: GCS, pupils Treat seizures Lorazepam 2-4mg IV Phenytoin18mg/kg IVI then 100mg/6-8h E: expose pt. and look for other obvious injuries secondary Survey Look for: Lacerations Obvious facial/skull deformity CSF leak from nose or ears Battle’s sign, Racoon eyes Blood behind TM C-spine tenderness ± deformity Head-to-toe examination for other injuries Log role Hx if possible How and when? GCS and other vitals immediately after injury Headache, fits, vomiting, amnesia, EtOH Ix Bloods: FBC, U+E, glucose, clotting, EtOH level, ABG ? CT head + c-spine

Question 208

after the primary and secondary survey and interventions and tests, how is a pt with head injury managed

Answer 208

Rx Neurosurgical opinion if signs of raised ICP, CT evidence of intracranial bleed significant skull # Admit if: Abnormalities on imaging Difficult to assess: EtOH, post-ictal Not returned to GCS 15 after imaging CNS signs: vomiting, severe headache Neuro-obs half-hrly until GCS 15 GCS Pupils HR, BP RR, SpO2 Temperature

Question 209

head injury - CT head guidelines

Answer 209

CT head guidelines: BANGS LOC Break: open, depressed or base of skull Amnesia \>30min retrograde Neuro deficit or seizure GCS: \<13 @ any time or \<15 2h after injury Sickness: vomited \> once LOC or any amnesia and any of: Dangerous mechanism: RTA, great height Age over 65 Coagulopathy (inc. warfarin)

Question 210

presenatation of space occupying lesions

Answer 210

Presentation raised ICP: Headache: worse on waking, lying down, bending forward, coughing, straining. Vomiting Papilloedema decreased GCS Seizures Exclude SOL in adult-onset seizures, especially c¯ localising aura or post-ictal weakness (Todd’s) Evolving Focal Neurology May be false-localising (esp. CN6 palsy) Subtle Personality Change

Question 211

causes of space occupying lesions

Answer 211

Causes Vascular: chronic subdural haematoma, AVM, aneurysm Infection: abscess, cyst (cysticercosis) Neoplasm: primary or secondary Granuloma: TB, sarcoid

Question 212

presentation cause management and prognosis of idiopathic intracranial hypertension

Answer 212

Presentation Typically obese females As if SOL raised ICP Headache Papilloedema Visual Blurred vision 6th CN palsy Enlarged blind spot Cause Usually idiopathic May be 2O to venous sinus thrombosis or drugs Mx Wt. loss Acetazolamide Loop diuretics Prednisolone Lumbar-peritoneal shunt may be necessary if drugs fail and visual loss deteriorates. Prognosis Usually self-limiting Permanent visual loss in 10%.

Question 213

types of cerebral oedema

Answer 213

Types of Cerebral Oedema 1. Vasogenic (increased cap permeability): trauma, tumour, ischaemia, infection 2. Cytotoxic: e.g. from hypoxia 3. Interstitial: e.g. obstructive hydrocephalus, decreased Na+

Question 214

acute management of raised ICP

Answer 214

ABC Treat seizures and correct hypotension Elevate bed to 40degrees Neuroprotective Ventilation PaO2: \>13KPa (100mmHg) PCO2: 4.5kPa Good sedation ± NM blockade Mannitol or Hypertonic Saline decrease ICP short-term, but may then rebound to rasied ICP later Mannitol 1g/kg (20% @ 5ml/kg)

Question 215

what is coning

Answer 215

Tonsillar herniation (Coning) increased pressure in posterior fossa causes displacement of cerebellar tonsils through foramen magnum then compression of brainstem and cardioresp centres in medulla CN6 palsy, upgoing plantars then irregular breathing then apnoea

Question 216

types of parkinsonisms

Answer 216

1. Parkinson’s disease 2. Parkinson’s-plus syndromes (basal ganglia degeneration + other system) a. Multiple Systems Atrophy / Shy-Drager Autonomic dysfunction: post hypotension, bladder dysfunction Cerebellar + pyramidal signs Rigidity \> Tremor - MSAc or MSAp? b. Progressive Supranuclear Palsy Postural instability then falls Speech disturbance (+ dementia) Palsy: vertical gaze - early postural instability. more symmetrical onset. c. Corticobasilar Degeneration: Aphasia, dysarthria, apraxia Akinetic rigidity in one limb Astereognosis (cortical sensory loss) Alien limb phenomenon d. Lewy Body Dementia: Fluctuating cognition Visual hallucinations

Question 217

parkinsonism syndromes symptoms

Answer 217

Tremor Worse at rest Exacerbated by distraction 4-6hz, pill-rolling Rigidity increased tone in all muscle groups: lead-pipe rigidity Rigidity + tremor causing cog-wheel rigidity Bradykinesia Slow initiation of movement c¯ reduction of amplitude on repetition Expressionless face Monotonous voice Micrografia Gait decreased arm swing Festinance (tottering/shufling gait) Freezing (esp. in doorways)

Question 218

pathophys of parkinsons disease

Answer 218

Pathophysiology Destruction of dopaminergic neurones in pars compacta of substantia nigra. beta-amyloid plaques Neurofibrillary tangles: hyperphosphorlated tau

Question 219

overview features of PD

Answer 219

Features: TRAPPS PD Asymmetric onset: side of onset remains worst Tremor: increased by stress, decreased by sleep Rigidity: lead-pipe, cog-wheel Akinesia: slow initiation, difficulty c¯ repetitive movement, micrographia, monotonous voice, mask-like face Postural instability: stooped gait c¯ festination Postural hypotension: + other autonomic dysfunction Sleep disorders: insomnia, EDS, OSA, RBD Psychosis: esp. visual hallucinations Depression / Dementia / Drug SEs

Question 220

L-DOPA SEs:

Answer 220

L-DOPA SEs: DOPAMINE Dyskinesia On-Off phenomena = Motor fluctuations Psychosis ABPdecreased Mouth dryness Insomnia N/V EDS (excessive daytime sleepiness

Question 221

motor fluctuations of PD pt on L-DOPA

Answer 221

Motor Fluctuations End-of-dose: deterioration as dose wears off c¯ progressively shorter benefit. On-Off effect: unpredictable fluctuations in motor performance unrelated to timing of dose.

Question 222

Ix and Mx of PD

Answer 222

Ix DaTSCAN Mx MDT: neurologist, PD nurse, physio, OT, social worker, GP and carers Assess disability e.g. UPDRS: Unified Parkinson’s Disease Rating Scale Physiotherapy: postural exercises Depression screening Medical Young onset ± biologically fit 1. Da agonists: ropinirole, pramipexole 2. MOA-B inhibitors: rasagiline, selegiline 3. L-DOPA: co-careldopa or co-beneldopa Biologically frail ± comorbidities 1. L-DOPA 2. MOA-B inhibitors Other therapies COMT inhibitor: tolcapone, entacapone Lessen end-of-dose effect Apomorphine: potent Da agonist SC rescue pen for sudden “off” freezing Amantidine: weak Da agonist Rx of drug-induced dyskinesias Atypical antipsychotics: e.g. quetiapine Disease-induced psychosis SSRIs: citalopram, sertraline Depression Surgical Interrupt basal ganglia Deep brain stimulation

Question 223

define MS

Answer 223

A chronic inflammatory condition of the CNS characterised by multiple plaques of demyelination disseminated in time and space. or Multiple sclerosis is chronic cell-mediated autoimmune disorder characterised by demyelination in the central nervous system. A variety of subtypes have been identified: Relapsing-remitting disease ``` most common form, accounts for around 80% of patients acute attacks (e.g. last 1-2 months) followed by periods of remission ``` Secondary progressive disease describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis gait and bladder disorders are generally seen Primary progressive disease accounts for 10% of patients progressive deterioration from onset more common in older people

Question 224

pathophy and classification of multiple sclerosis

Answer 224

Pathophysiology CD4 cell-mediated destruction of oligodendrocytes causes demyelination and eventual neuronal death. Initial viral inflam primes humoral Ab responses vs. MBP Plaques of demyelination are hallmark Classification: Relapsing-remitting: 80% Secondary progressive Primary progressive: 10% Progressive relapsing

Question 225

presentation and features of MS

Answer 225

Presentation: TEAM Tingling Eye: optic neuritis (decreased central vision + eye move pain) Ataxia + other cerebellar signs Motor: usually spastic paraparesis Clinical features Sensory: Dys/paraesthesia decreased vibration sense Trigeminal neuralgia Motor Spastic weakness Transverse myelitis Eye: Diplopia Visual phenomena Bilateral INO Optic neuritis causes atrophy Cerebellum: Trunk and limb ataxia Scanning dysarthria Falls GI: Swallowing disorders Constipation Sexual/GU: ED + anorgasmia Retention Incontinence Lhermitte’s Sign Neck flexion causes electric shocks in trunk/limbs

Question 226

Ix and differential for MS

Answer 226

Ix MRI: Gd-enhancing or T2 hyper-intense plaques Gd-enhancing = active inflammation Typically located in periventricular white matter LP: IgG oligoclonal bands (not present in serum) Abs:Anti-MBP NMO-IgG: highly specific for Devic’s syn. Evoked potentials: delayed auditory, visual and sensory Diagnosis: clinical Demonstration of lesions disseminated in time and space May use McDonald Criteria Differential Inflammatory conditions may mimic MS plaques: CNS sarcoidosis SLE Devic’s: Neuromyelitis optica (NMO) MS variant c¯ transverse myelitis and optic atrophy Distinguished by presence of NMO-IgG Abs

Question 227

management of MS

Answer 227

Treatment in multiple sclerosis is focused at reducing the frequency and duration of relapses. There is no cure. Mx MDT: neurologist, radiologist, physio, OT, specialist nurses, GP, family Acute Attack Methylpred 1g IV/PO /24h for 3d Doesn’t influence long-term outcome decreases duration and severity of attacks Preventing Relapse: DMARDs IFN-beta: decreases relapses by 30% in relapsing remitting MS Glatiramer: similar efficacy to IFN-beta. immunomodulating drug - acts as an 'immune decoy' Preventing Relapse: Biologicals Natalizumab: anti-VLA-4 Ab decreases Relapses by 2/3 in RRMS - a recombinant monoclonal antibody that antagonises Alpha-4 Beta-1-integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium across the blood-brain barrier Alemtuzumab (Campath): anti-CD52, 2nd line in RRMS fingolimod: sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes. An oral formulation is available Symptomatic treatments Fatigue: modafinil Depression: SSRI (citalopram) Pain: amitryptylline, gabapentin Spasticity: baclofen and gabapentin are first-line. physio, baclofen, dantrolene, botulinum Urgency / frequency: oxybutynin, tolterodine. Bladder dysfunction may take the form of urgency, incontinence, overflow etc guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients if significant residual volume → intermittent self-catheterisation if no significant residual volume → anticholinergics may improve urinary frequency ED: sildenafil Tremor: clonazepam

Question 228

signs and symptoms and causes of cord compression

Answer 228

Symptoms Deep, local spinal pain Stabbing, radicular pain in a dermatomal distribution and LMN weakness @ lesion level Progressive UMN weakness and sensory loss below lesion Bladder hesitancy, frequency then painless retention Faecal incontinence or constipation Signs Look for motor, reflex and sensory level Shooting, radicular pain @ level, anaesthesia below LMN signs @ level, UMN signs below level Tone and reflexes are usually reduced in acute cord compression Causes Trauma Infection: epidural abscess, TB 2O to malignancy: breast, thyroid, bronchus, kidney, prostate Disc prolapse Haematoma: warfarin Intrinsic cord tumour Myeloma

Question 229

Ix and Rx of cord dompression

Answer 229

Ix MRI is definitive modality CXR for primaries Rx This is a neurosurgical emergency Malignancy Dexamethasone IV Consider chemo, radio and decompressive laminectomy Abscess: abx and surgical decompression

Question 230

Cauda Equina and Conus Medullaris Lesions

Answer 230

Cauda Equina and Conus Medullaris Lesions Spinal cord tapers to its end at L1 Compared c¯ lesions higher up the cord, these lesions are flaccid and areflexic (cf, spastic and hyperreflexic) Conus Medullaris Lesions Mixed UMN/LMN weakness Early constipation and retention Back pain Sacral sensory disturbance ED Cauda Equina Lesions Saddle anaesthesia Back pain Radicular pain down legs Bilateral flaccid, areflexic lower limb weakness Incontinence / retention of faeces / urine Poor anal tone Mx As for cord compression These are neurosurgical emergencies and require urgent imaging and surgical decompression

Question 231

what is the hoffman reflex

Answer 231

Hoffman reflex: flick to middle finger pulp causes brief pincer flexion of thumb and index finger

Question 232

what happens in spinal stenosis

Answer 232

Spinal Stenosis Developmental predisposition ± facet joint osteoarthritis generalized narrowing of lumbar spinal canal. Presentation Spinal claudication Aching or heavy buttock and lower limb pain on walking Rapid onset May c/o paraesthesiae/numbness Pain eased by leaning forward (e.g. on bike) Pain on spine extension Negative straight leg raise Ix MRI Rx Corsets NSAIDs Epidural steroid injection Canal decompression surgery

Question 233

causes of a facial nerve palsy

Answer 233

Bell’s Palsy Ramsay Hunt Syndrome Other Causes of Facial Palsy May be suggested by: Bilat symptoms: Lyme, GBS, leukaemia, sarcoid, MG UMN signs: sparing of frontalis and orbicularis oculi Due to bilateral cortical representation Other CN palsies (but seen in 8% of Bell’s) Limb weakness Rashes Intracranial Vascular, MS, SOL Motor cortex causes UMN: Assoc. c¯ ipsilateral hemiplegia Brainstem nuclei causes LMN: Usually assoc. c¯ CN6 palsy + contralateral hemiplegia Cerebello-pontine angle: acoustic neuroma, meningioma: Both may be accompanied by 5th, 6th and 8th CN palsies and cerebellar signs Loss of corneal reflex Sensorineural deafness, tinnitus, vertigo DANISH Intra-temporal Cholesteatoma Ramsay Hunt Otitis media Trauma Infra-temporal Parotid tumours Trauma Systemic Peripheral neuropathy Demyelinating: GBS Axonal: DM, Lyme, HIV, Sarcoid Pseudopalsy: MG, botulism

Question 234

Bell’s Palsy - features , ix , mx, prognosis

Answer 234

Bell’s Palsy Inflammatory oedema from entrapment of CNVII in narrow facial canal Probably of viral origin (HSV1) 75% of facial palsy Features Sudden onset: e.g. overnight Complete, unilateral facial weakness in 24-72h Failure of eye closure causes dryness Bell’s sign: eyeball rolls up on attempted closure Drooling, speech difficulty Numbness or pain around ear decrease taste (ageusia) Hyperacusis: stapedius palsy Ix Serology: Borrelia or VZV Abs MRI: SOL, stroke, MS LP Mx Give prednisolone w/i 72hrs 60mg/d PO for 5/7 followed by tapering Valaciclovir if zoster suspected Otherwise antivirals don’t help Protect eye Dark glasses Artificial tears Tape closed @ night Plastic surgery may help if no recovery Prognosis Incomplete paralysis: recovers completely w/i wks Complete: 80% get full recovery Remainder have delayed recovery or permanent neurological / cosmetic abnormalities. Complications: Aberrant Neural Connections Synkinesis: e.g. blinking causes up-turning of mouth Crocodile tears: eating stimulates unilateral lacrimation, not salivation

Question 235

what is Ramsay Hunt Syndrome

Answer 235

Ramsay Hunt Syndrome American neurologist James Ramsay Hunt in 1907 Reactivation of VZV in geniculate ganglion of CNVII Features Preceding ear pain or stiff neck Vesicular rash in auditory canal ± TM, pinna, tongue, hard palate (no rash = zoster sine herpete) Ipsilateral facial weakness, ageusia, hyperacusis, May affect CN8 causing vertigo, tinnitus, deafness Mx If Dx suspected give valaciclovir and prednisolone w/i first 72h Prognosis Rxed w/i 72h: 75% recovery Otherwise: 1/3 full recovery, 1/3 partial, 1/3 poor

Question 236

what are mononeuropathies, causes etc

Answer 236

Lesions of individual peripheral or cranial nerves Usually local cause: trauma, entrapment Mononeuritis multiplex: 2 or more peripheral nerves affects Usually systemic cause: DM most commonly WAARDS PLC: Wegener’s, AIDS, Amyloid, RA, DM, Sarcoidosis, PAN, Leprosy, Carcinomatosis Electromyography (EMG) helps define site of lesion

Question 237

features of polyneuropathies

Answer 237

Features Generalised disorders of peripheral or cranial nerves Distribution is symmetrical and widespread Distal weakness and sensory loss (glove + stocking) Classification Time-course: acute, chronic Function: motor, sensory, autonomic, mixed Path: demyelination, axonal degeneration, both

Question 238

Hx and Ix for polyneuropathies

Answer 238

History Time-course Precise symptoms Assoc. events D&V: GBS decreased weight: Ca Arthralgia: connective tissue Travel, EtOH, drugs Ix LFTs, U+E, glucose, ESR, B12 TFTs B1, ANA, ANCA Genetic tests: e.g. PMP22 in CMT Nerve conduction studies EMG

Question 239

causes of polyneuropathies

Answer 239

Metabolic (mostly axonal) DM Renal failure / uraemia Hypothyroid decreased B1 or B12 (EtOH) Vasculitis PAN RA Wegener’s Inflammatory GBS Sarcoidosis Infection HIV Syphilis Leprosy Lyme Inherited CMT Refsum’s syndrome Toxins Lead Drugs Isoniazid EtOH Phenytoin Vincristine Other Amyloid Paraproteinaemias

Question 240

classification and causes of Guillain-Barré Syndrome

Answer 240

Classification AIDP: Acute autoimmune demyelinating polyneuropathy AMAN: Acute motor (± sensory) axonal neuropathy Miller-Fisher: Ophthalmoplegia + ataxia + areflexia Causes Abs cross-react to gangliosides No precipitant identified in 40% Bacteria: C. jejuni, mycoplasma Viruses: CMV, EBV, HSV, HIV, flu Vaccines: esp. rabies

Question 241

features Ix and Mx of GBS

Answer 241

Features and Ix: GBS=AIDP Growing Weakness Symmetrical, ascending flaccid weakness / paralysis LMN signs: areflexia, fasciculations may occur Proximal \> distal (trunk, respiratory, CNs [esp. 7]) Progressive phase lasts less than 4wks Breathing and Bulbar Problems Back Pain Back / limb pain is common Sensory disturbance Paraesthesia in extremities Sensory ataxia in Miller-Fisher Autonomic Neuropathy Arrhythmias, increased HR Labile BP Sweating Urinary retention Immune Serology for anti-ganglioside Abs Evidence of infection: e.g. stool sample Demyelinating Nerve Conduction Studies Slow conduction velocities Protein in CSF Protein often \> 5.5g/L Normal white cell count Mx Supportive Airway / ventilation: ITU if FVC \< 1.5L Analgesia: NSAIDs, gabapentin Autonomic: may need inotropes, catheter Antithrombotic: TEDS, LMWH Immunosuppression IVIg Plasma exchange Physiotherapy Prevent flexion contractures Prognosis 85% complete recovery 10% unable to walk alone at 1yr 5% mortality

Question 242

what is Charcot-Marie-Tooth Syndrome

Answer 242

Charcot-Marie-Tooth Syndrome = Peroneal Muscular Atrophy = Hereditary Motor and Sensory Neuropathy Pathophysiology Group of inherited motor and sensory neuropathies HMSN1 Commonest form Demyelinating AD mutation in the peripheral myelin protein 22 gene HMSN2 Second commonest form Axonal degeneration (therefore near normal conduction velocity)

Question 243

Charcot-Marie-Tooth Syndrome features, ix and mx

Answer 243

Clinical Features Onset at puberty Nerves Thickened, enlarged nerves: esp. common peroneal Motor Foot drop causes high stepping gait Weak ankle dorsiflexion and toe extension Absent ankle jerks Symmetrical muscle atrophy: mainly distal Peroneal muscles causes “Champagne Bottle” Hand and arm muscles causes “Claw Hand” Pes cavus (high-arched feet) Sensory Variable loss of sensation in a stocking distribution Neuropathic pain in some Ix Genetic tests: PMP22 gene mutation Nerve conduction studies: decrease conduction speed in CMT1 Mx Supportive Physio Podiatry Orthoses: e.g. ankle braces

Question 244

what is motor neurone disease

Answer 244

Characteristics Cluster of degenerative disease characterised by axonal degeneration of neurones in the motor cortex, CN nuclei and anterior horn cells. UM and LM neurones affected (cf. polyneuropathy) No sensory loss or sphincter disturbance (cf. MS) Never affects eye movements (cf. MG) Causes Unknown ~10% familial: SOD1 mutation in 20% of those

Question 245

motor neurone disease types

Answer 245

1 - Amyotrophic Lateral Sclerosis: 50% Loss of motor neurones in cortex and anterior horn causes UMN signs and LMN wasting + fasciculation 2 - Progressive Bulbar Palsy: 10% Only affects CN 9-12 causes bulbar palsy 3 - Progressive Muscular Atrophy: 10% Anterior horn cell lesion causes LMN signs only Distal to proximal Better prognosis cf. ALS 4 - Primary Lateral Sclerosis Loss of Betz cells in motor cortex causes mainly UMN signs Marked spastic leg weakness and pseudobulbar palsy No cognitive decline 2a - Bulbar Palsy Diseases of nuclei of CN 9-12 in the medulla LMN lesions of tongue, talking and swallowing Signs Flaccid, fasciculating tongue Speech: quiet or nasal (“Donald Duck” speech) Normal / absent jaw jerk Loss of gag reflex Causes MND GBS MG Central pontine myelinolysis (CPM) 2b - Pseudobulbar / Corticobulbar Palsy Commoner than bulbar palsy Bilateral lesions above mid-pons (e.g. corticobulbar tracts) causes UMN lesions of swallowing and talking CN motor nuclei have bilateral corticalbrepresentation except lower half of CN7 Signs Spastic tongue Slow tongue movements c¯ slow deliberate speech: “hot-potato” speech Brisk jaw jerk Emotional incontinence Causes MS MND Stroke CPM x - Polio RNA virus Affects anterior horn cells Fever, sore throat, myalgia 0.1% develop paralytic polio Asymmetric LMN paralysis No sensory involvement May be confined to upper or lower limbs or both Respiratory muscle paralysis can cause death

Question 246

diagnosis and Mx of motor neurone diseases

Answer 246

Diagnosis MRI to exclude structural lesions LP to exclude inflammation EMG shows acute denervation Use Revised El Escorial Diagnostic Criteria Mx MDT: neurologist, physio, OT, dietician, specialist nurse, GP, family Discussion of end-of-life decisions E.g. Advanced directive DNAR Specific Riluzole: antiglutamatergic that prolongs life by ~3mo Supportive Drooling: propantheline or amitriptyline Dysphagia: NG or PEG feeding Respiratory failure: NIV Pain: analgesic ladder Spasticity: baclofen, botulinum Prognosis Most die w/i 3yrs Bronchopneumonia and respiratory failure Worse prog: elderly, female, bulbar involvement Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy Riluzole prevents stimulation of glutamate receptors used mainly in amyotrophic lateral sclerosis prolongs life by about 3 months Respiratory care non-invasive ventilation (usually BIPAP) is used at night studies have shown a survival benefit of around 7 months Prognosis poor: 50% of patients die within 3 years

Question 247

myopathy vs neuropathy

Answer 247

Myopathy Gradual onset Symmetrical, proximal weakness: difficulty combing hair, climbing stairs, getting up from chairs Dystrophies usually affect specific muscle groups Preserved tendon reflexes Neuropathy Paraesthesia, bladder problems Distal weakness Other Pointers Rapid onset: neuropathy, or drug, toxic or metabolic myopathy Fatiguability: MG, LEMS Spontaneous pain and tenderness @ rest: inflam myopathy Pain on exercise: ischaemia or metabolic myopathy Oddly firm muscles: pseudohypertrophy in muscular dystrophies Fasciculation: anterior horn cell or root disease

Question 248

7 Muscular Dystrophies

Answer 248

Muscular Dystrophies Group of genetic disease c¯ progressive degeneration and weakness of specific muscle groups. DMD Commonest: 3/1000 male births X-linked recessive, 30% spontaneous cause non-functional dystrophin BMD (becker's) 0.3/1000 male births X-linked recessive Partially functioning dystrophin Presents later, is less severe and has better prognosis Facioscapulohumeral MD (Landouzy-Dejerine) Almost as common as DMD AD inheritance Presentation Onset @ 12-14yrs Difficulty puffing cheeks and raising arms above head Myotonic Dystrophy AD Cl- channelopathy Onset in 20s Tonic muscle spasm (myotonia) Acquired myopathies of late onset Usually part of systemic disease Hyperthyroidism, Cushing’s, up/downCa2+ Drugs: steroids, statins, EtOH Inflammatory Myopathies Inclusion body myositis Polymyositis Dermatomyositis Inclusion Body Myositis Asymmetric weakness affecting distal and prox muscles Early involvement of quads, ankle dorsiflexors and wrist/finger flexorsthen loss of grip strength and decreased dexterity Dysphagia is very common Myalgia is relatively uncommon

Question 249

pathophys of myasthenia gravis

Answer 249

Pathophysiology Autoimmune disease mediated by Abs vs. nicotinic Ach receptors. Interferes c¯ NM transmission via depletion of working post-synaptic receptor sites

Question 250

presentation and ix and dd of MG

Answer 250

Presentation increasing muscular fatigue: Extra-ocular: bilateral ptosis, diplopia Bulbar: voice deteriorates on counting to 50 Face: myasthenic snarl on attempting to smile Neck: head droop Limb: asymmetric, prox. weakness Normal tendon reflexes Weakness worsened by pregnancy, infection, emotion, drugs (beta-B, gent, opiates, tetracyclines) Investigations: Tensilon Test: Give edrophonium IV +ve if power improves w/i 1min Anti-AChR Abs: increased in 90%, MuSK Abs EMG: decreased response to a train of impulses Respiratory function: decreased FVC Thymus CT TFTs Differential of Muscle Fatigability Polymyositis SLE Botulism

Question 251

treatment of MG

Answer 251

Treatment Symptom Control Anticholinesterase: e.g. pyridostigmine. Cholinergic SEs = SLUDGEM Immunosuppression Rx relapses c¯ pred Steroids may be combined c¯ azathioprine or methotrexate Thymectomy Consider if young onset and disease not control by anticholinesterases Remission in 25%, benefit in further 50%. Complications Myasthenic Crisis Weakness of respiratory muscles during relapse may be lethal. Monitor FVC: vent support if \<20ml/kg Plasmapheresis or IVIg Rx trigger for relapse (drugs, infection…) Prognosis Relapsing or slow progression

Question 252

what happens in Lambert-Eaton Myasthenic Syndrome (LEMS)

Answer 252

Pathophysiology Abs to VGCC decrease influx of Ca2+ during presynaptic excitation cause presynaptic ACh-vesicle fusion. Causes Paraneoplastic: e.g SCLC Autoimmune Presentation As for MG except: LEMS Leg weakness early (before eyes) Extra: Autonomic and areflexia Movement improves symptoms Small response to edrophonium Anti-VGCC Abs

Question 253

what is Botulism

Answer 253

Botulinum toxin prevents ACh vesicle release Descending flaccid paralysis c¯ no sensory signs Anti-cholinergic effects: mydriasis, cycloplegia, n/v, dry mouth, constipation Rx: benpen + antiserum

Question 254

features of Neurofibromatosis 1 – von Recklinghausen’s

Answer 254

Features: CAFÉ NOIR Café-au-lait spots 1st yr of life increase in size and no. c¯ age Adults: \>6, \>15mm across Axillary Freckling in skin folds Fibromas, neuro-: Subcutaneous Small, gelatinous, violaceous nodules Appear @ puberty May itch Nos. increase c¯ age Plexiform Overgrowth of nerve trunk and overlying tissue Large cutaneous mass Complications Sarcomatous change Compression: Nerve roots: weakness, pain, paraesthesia GI: bleeds and obstruction Eye Lisch nodules - multiple little brown nodules. Brown/translucent iris hamartomas Use a slit lamp Optic N. glioma Neoplasia CNS: meningioma, ependyoma, astrocytoma Phaeochromocytoma Chronic or acute myeloid leukaemia Orthopaedic Kyphoscoliosis Sphenoid dysplasia IQdecreased and Epilepsy Renal RAS causes raised BP Mx MDT orchestrated by GP Yearly BP and cutaneous review Excise some neurofibromas Genetic counselling - AD

Question 255

what is Syringomyelia

Answer 255

Characteristics Syrinx: tubular cavity in central canal of the cervical cord. Onset @ 30yrs Symptoms may be static for yrs but then worsen fast e.g. on coughing, sneezing as increas pressure causes extension Commonly located in cervical cord Syrinx expands ventrally affecting: Decussating spinothalamic neurones Anterior horn cells Corticospinal tracts Causes Blocked CSF circulation c¯ decreased flow from posterior fossa Arnold-Chiari malformation (cerebellum herniates through foramen magnum) Masses Spina bifida secondary to cord trauma, myelitis, cord tumours and AVMs

Question 256

cardinal signs of syringomyelia

Answer 256

Cardinal Signs: 1. Dissociated Sensory Loss Absent pain and temperature so gets scars from burns Preserved touch, proprioception and vibration. Root distribution reflects syrinx location Usually upper limbs and chest: “cape” 2. Wasting/weakness of hands ± Claw hand 3. Loss of reflexes in upper limb 4. Charcot Joints: shoulder and elbow Other Signs UMN weakness in lower limbs c¯ upgoing plantars Horner’s syndrome Syringobulbia: cerebellar and lower CN signs Kyphoscoliosis Ix MRI spine Surgery Decompression at the foramen magnum for Chiari malformation

Question 257

what happens in Brown-Sequard Syndrome

Answer 257

Hemi-cord lesion Ipsilateral loss of proprioception and vibration sense Ipsilateral UMN weakness Contralateral loss of pain sensation

Question 258

what happens in Friedrich’s Ataxia

Answer 258

Friedrich’s Ataxia Auto recessive progressive degeneration of DRGs, spinocerebellar and corticospinal tracts and cerebellar cells Mitochondrial disorder Onset in teenage years Assoc. c¯ HOCM and mild dementia Presentation Pes cavus and scoliosis Bilateral cerebellar signs Ataxia Dysarthria Nystagmus Leg wasting + areflexia but extensor plantars Loss of lower limb proprioception and vibration sense Optic atrophy Cardiac: HOCM causes ESM + 4th heart sound DM then hyperglycaemia

Question 259

what happens in Hereditary Spastic Paraparesis

Answer 259

Lower limb spasticity Ataxia Extrapyramidal signs

Question 260

features ofHuman T-lymphotropic Virus-1

Answer 260

Human T-lymphotropic Virus-1 Retrovirus higher prevalence in Japan and Caribbean Features Adult T cell leukaemia / lymphoma Tropical spastic paraplegia / HTLV myelopathy Slowly progressing spastic paraplegia Sensory loss and paraesthesia Bladder dysfunction

Question 261

what happens in Wernicke’s encephalopathy

Answer 261

- Thiamine (B1) deficiency - Gaze palsies - Ataxia - Apathy - Confusion/delirium - Korsakoff’s psychosis (amnesia and confabulation) Wernicke's encephalopathy or now often Wernicke's disease refers to the presence of neurological symptoms caused by biochemical lesions of the central nervous system after exhaustion of B-vitamin reserves, in particular thiamine (vitamin B1). The condition is part of a larger group of diseases related to thiamine insufficiency, including beriberi in all its forms, and Korsakoff syndrome. When Wernicke's encephalopathy occurs simultaneously with Korsakoff syndrome it is known as Wernicke–Korsakoff syndrome.[1][2] Classically, Wernicke's encephalopathy is characterised by the triad ophthalmoplegia, ataxia, and confusion. However, only 10% of patients exhibit all three features, and other symptoms may also be present.[3] While it is commonly regarded as a condition peculiar to malnourished people with alcohol misuse, a variety of diseases can lead to Wernicke's encephalopathy.[1][4] Wernicke's encephalopathy (WE) is treated with thiamine supplementation, which can lead to improvement of the symptoms and often complete resolution, particularly in those where alcohol misuse is not the underlying cause. Often other nutrients also need to be replaced, depending on the cause.

Question 262

where can the brain herniate?

Answer 262

subfalcine herniation of the cingulate gyrus transtentorial herniation of the medial part of the temporal lobe coning

Question 263

define what an astrocyte is and does

Answer 263

Astrocytes (Astro from Greek astron = star and cyte from Greek "kyttaron" = cell), also known collectively as astroglia, are characteristic star-shaped glial cells in the brain and spinal cord. The proportion of astrocytes in the brain is not well defined. Depending on the counting technique used, studies have found that the astrocyte proportion varies by region and ranges from 20% to 40% of all glia.[1] They perform many functions, including biochemical support of endothelial cells that form the blood–brain barrier, provision of nutrients to the nervous tissue, maintenance of extracellular ion balance, and a role in the repair and scarring process of the brain and spinal cord following traumatic injuries. Research since the mid-1990s has shown that astrocytes propagate intercellular Ca2+ waves over long distances in response to stimulation, and, similar to neurons, release transmitters (called gliotransmitters) in a Ca2+-dependent manner. Data suggest that astrocytes also signal to neurons through Ca2+-dependent release of glutamate -

Question 264

prognosis of a glioblastoma multiforme

Answer 264

only 20% still alive after 1 year.

Question 265

prognosis of meningioma

Answer 265

around 90% five year survival

Question 266

brian tumours general classifications

Answer 266

primary vs secondary intrinsic vs extrinsic eg gliomas vs meningiomas.

Question 267

most common types of intrinsic brain tumours

Answer 267

astrocytic tumours diffusley infiltrating (glioblastoma) = incurable and in adults. cf with circumscribed (not infiltrating) = in children and much more curable

Question 268

why are meningiomas not so bad ?

Answer 268

they tend to be a bit benign and will press into the brain rather than invade and thus are a bit easier to remove. many are found incidentally at post mortem.

Question 269

why is extradural haemorrhage so important

Answer 269

requires urgent neurosurgical treatment. often skull frature and torn meningeal arteries.

Question 270

The most common primary intrinsic brain tumours are gliomas (tumours of glial cells). These include :

Answer 270

Diffuse astrocytomas: these form a group of tumours, astrocytoma (grade II), anaplastic astrocytoma (grade III) and glioblastoma multiforme (grade IV), of progressively increasing malignancy. They spread primarily by diffusely infiltrating through the brain parenchyma. Pilocytic astrocytoma: this is primarily a childhood tumour, with preference for the posterior fossa and midline structures and is low grade (WHO grade I). It is a circumscribed astrocytic tumour, and genetically and prognostically very different to the diffusely infiltrating astrocytic tumours. Oligodendrogliomas: these mainly occur in adults and are more chemosensitive than other glial tumours. There are two grades: grade II and grade III (anaplastic oligodendroglioma). Ependymomas: predominantly occur in young children and in the infratentorial compartment, or near to the ventricular system. Primitive neuroepithelial tumours (PNET) include medulloblastomas. They occur predominantly in children and tend to disseminate through the CSF and they have high mitotic rates. They can show mixed glial and neuronal differentiation. There are other rare CNS tumours including those containing a neoplastic neuronal element (e.g. gangliogliomas)

Question 271

As glial tumours become more malignant they show histological changes of

Answer 271

As glial tumours become more malignant they show histological changes of increasing anaplasia, in particular necrosis, pleomorphism and mitotic figures. The presence of mitotic activity, necrosis and vascular hyperplasia are features used to grade diffuse astrocytic tumours.

Question 272

central pontine myelinolysis - what is it?

Answer 272

over rapid correction of electrolytes in alcoholics causes pontine demyleination Central pontine myelinolysis (CPM), also known as Osmotic demyelination syndrome, is a neurological disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the area termed the pons, predominately of iatrogenic etiology. It is characterized by acute paralysis, dysphagia (difficulty swallowing), and dysarthria (difficulty speaking), and other neurological symptoms. It can also occur outside the pons.[1] The term "osmotic demyelination syndrome" is similar to "central pontine myelinolysis", but also includes areas outside the pons.[2] Central pontine myelinolysis presents most commonly as a complication of treatment of patients with profound, life-threatening hyponatremia (low sodium). It occurs as a consequence of a rapid rise in serum tonicity following treatment in individuals with chronic, severe hyponatraemia who have made intracellular adaptations to the prevailing hypotonicity.[3] Hyponatremia should be corrected at a rate of no more than 12-20 mmol/L of sodium per day to prevent central pontine myelinolysis.[3]

Question 273

overview of meningiomas

Answer 273

Meningiomas arise from the meningothelial cells (arachnoid cells) in the leptomeninges. These cells have both epithelial and mesenchymal properties They are usually tumours of adulthood with a female gender bias Common sites include the parafalcine region, orbitofrontal cortex, cerebello-pontine angle and the spinal dura. They are typically attached to the dura. They tend to produce nodular or plaque like masses which indent the underlying brain. The majority of meningiomas are low grade and benign but aggressive tumour types with recurrent behaviour can occur (WHO grades II and III).

Question 274

overview of Nerve sheath tumours

Answer 274

Nerve sheath tumours (Two main types : schwannoma and neurofibroma) Schwannomas are common cerebello-pontine (CPA) angle tumours Neurofibromas may arise as skin nodules or as local or plexiform tumours on nerve roots

Question 275

Common metastatic tumours to the CNS

Answer 275

Common metastatic tumours to the CNS are lung (50%), breast (15%), melanoma (10%), kidney (5%).

Question 276

what happens in Fetal Alcohol Syndrome

Answer 276

Fetal Alcohol Syndrome - poor intrauterine growth - poor postnatal development - craniofacial abnormalities - microcephaly - sometimes limb, cardiac and other malformations

Question 277

what is a penumbra in ischaemia

Answer 277

In pathology and anatomy the penumbra is the area surrounding an ischemic event such as an ischemic, thrombotic or embolic stroke. Immediately following the event, blood flow and therefore oxygen transport is reduced locally, leading to hypoxia of the cells near the location of the original insult. This can lead to hypoxic cell death (infarction) and amplify the original damage from the ischemia; however, the penumbra area may remain viable for several hours after an ischemic event due to the collateral arteries that supply the penumbral zone. As time elapses after the onset of stroke, the extent of the penumbra tends to decrease;[1] therefore, in the emergency department a major concern is to protect the penumbra

Question 278

what happens in a watershed infarction

Answer 278

Watershed cerebral infarctions (also known as border zone infarcts) occur at the border between cerebral vascular territories with no or little anastomosis. Pathology It has been proposed that both, episodes of hypoperfusion and microembolisms, play a role in pathophysiology of this entity. Clearance of the microemboli are most likely to be impaired in watershed zones due to poorer perfusion. Episodes of systemic hypotension along with severe stenosis or occlusion of the feeding arteries, in particular intra- and extracranial carotid arteries can also contribute to the infarction. Watershed zone infarct in an isolated zone is more likely to be secondary to microembolism in the absence of significant systemic hypotension. Prolonged hypotension such as cardiac surgery or cardiac arrest commonly give a bilateral pattern 5 , on the other hand, severe carotid stenosis lesions are usually unilateral on the side of the stenosis. WSI has been classified to: Cortical (external) border zones infarct: between ACA, MCA, and PCA territories and mostly due to microemboli Deep (internal) border zones infarct: between ACA, MCA, and PCA territories and perforating medullary, lenticulostriate, recurrent artery of Heubner and anterior choroidal arteries; mostly due to either severe hypotention or severe arterial stenosis

Question 279

middle meningeal artery anatomy

Answer 279

The middle meningeal artery (Latin: arteria meningea media) is typically the third branch of the first part (retromandibular part) of the maxillary artery, one of the two terminal branches of the external carotid artery. After branching off the maxillary artery in the infratemporal fossa, it runs through the foramen spinosum to supply the dura mater (the outermost meninges) and the calvaria. The middle meningeal artery is the largest of the three (paired) arteries that supply the meninges, the others being the anterior meningeal artery and the posterior meningeal artery. The anterior branch of the middle meningeal artery runs beneath the pterion. It is vulnerable to injury at this point, where the skull is thin

Question 280

what does an extradural ahaematoma look like on ct vs subdural vs subarachnoid

Answer 280

crescenteric - distends dura but not at the areas where the dura touches the skull sutures as it is particularly adhesive here, thus a crescenteric shape. subdural pushes out the meninges but not crescenteric, subdural can be CHRONIC so can be cause a couple of months before presentation. subarachnoid is below the level of the meninges and so distributes everywhere.

Question 281

headache red flags

Answer 281

Headache is one of the most common presenting complaints seen in clinical practice. The vast majority of these will be caused by common, benign conditions. There are however certain features in a history which should prompt further action. In the 2012 guidelines NICE suggest the following: ## Footnote compromised immunity, caused, for example, by HIV or immunosuppressive drugs age under 20 years and a history of malignancy a history of malignancy known to metastasis to the brain vomiting without other obvious cause worsening headache with fever sudden-onset headache reaching maximum intensity within 5 minutes new-onset neurological deficit new-onset cognitive dysfunction change in personality impaired level of consciousness recent (typically within the past 3 months) head trauma headache triggered by cough, valsalva (trying to breathe out with nose and mouth blocked), sneeze or exercise orthostatic headache (headache that changes with posture) symptoms suggestive of giant cell arteritis or acute narrow-angle glaucoma a substantial change in the characteristics of their headache

Question 282

what happens in Tuberous sclerosis

Answer 282

Tuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neuro-cutaneous Cutaneous features ## Footnote depigmented 'ash-leaf' spots which fluoresce under UV light roughened patches of skin over lumbar spine (Shagreen patches) adenoma sebaceum: butterfly distribution over nose fibromata beneath nails (subungual fibromata) café-au-lait spots\* may be seen Neurological features developmental delay epilepsy (infantile spasms or partial) intellectual impairment Also retinal hamartomas: dense white areas on retina (phakomata) rhabdomyomas of the heart gliomatous changes can occur in the brain lesions polycystic kidneys, renal angiomyolipomata \*these of course are more commonly associated with neurofibromatosis. However a 1998 study of 106 children with TS found café-au-lait spots in 28% of patients

Question 283

management of TIA based on results of the ABCDE score

Answer 283

This gives a total score ranging from 0 to 7. People who have had a suspected TIA who are at a higher risk of stroke (that is, with an ABCD2 score of 4 or above) should have: ## Footnote aspirin (300 mg daily) started immediately specialist assessment and investigation within 24 hours of onset of symptoms measures for secondary prevention introduced as soon as the diagnosis is confirmed, including discussion of individual risk factors If the ABCD2 risk score is 3 or below: aspirin specialist assessment within 1 week of symptom onset, including decision on brain imaging if vascular territory or pathology is uncertain, refer for brain imaging People with crescendo TIAs (two or more episodes in a week) should be treated as being at high risk of stroke, even though they may have an ABCD2 score of 3 or below. With regards to carotid artery endarterectomy: recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled should only be considered if carotid stenosis \> 70% according ECST\* criteria or \> 50% according to NASCET\*\* criteria

Question 284

Seizures: acute management

Answer 284

Most seizures are self-limiting and stop spontaneously but prolonged seizures may be potentially life-threatening. Basics check the airway and apply oxygen if appropriate place the patient in the recovery position if the seizure is prolonged give benzodiazepines BNF recommend dose for rectal diazepam, repeated once after 10-15 minutes if necessary Neonate 1.25 - 2.5 mg Child 1 month - 2 years 5 mg Child 2 years - 12 years 5 - 10 mg Child 12 years - 18 years 10 mg Adult 10 - 20 mg (max. 30 mg) Elderly 10 mg (max. 15 mg)

Question 285

what is Cataplexy

Answer 285

Cataplexy describes the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy. Features range from buckling knees to collapse.

Question 286

Causes of bilateral facial nerve palsy

Answer 286

Causes of bilateral facial nerve palsy ## Footnote sarcoidosis Guillain-Barre syndrome polio, Lyme disease

Question 287

causes of unilateral facial nerve palsy

Answer 287

Causes of bilateral facial nerve palsy sarcoidosis Guillain-Barre syndrome polio, Lyme disease Causes of unilateral facial nerve palsy - as above plus Bell's palsy Ramsay-Hunt syndrome (due to herpes zoster) acoustic neuroma parotid tumours HIV multiple sclerosis\* diabetes mellitus Upper motor neuron - stroke

Question 288

what are Pseudoseizures

Answer 288

Psychogenic non-epileptic seizures (PNES), also known as non-epileptic attack disorders (NEAD), are events superficially resembling an epileptic seizure, but without the characteristic electrical discharges associated with epilepsy. PNES are triggered by psychological problems, and frequently occur in conversion disorder. It is estimated that 20% of seizure patients seen at specialist epilepsy clinics have PNES. Factors favouring pseudoseizures pelvic thrusting family member with epilepsy more common in females crying after seizure don't occur when alone gradual onset Factors favouring true epileptic seizures tongue biting raised serum prolactin\* Video telemetry is useful for differentiating \*why prolactin is raised following seizures is not fully understood. It is hypothesised that there is spread of electrical activity to the ventromedial hypothalamus, leading to release of a specific prolactin regulator into the hypophyseal portal system

Question 289

what is Restless legs syndrome

Answer 289

Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present Clinical features ## Footnote uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest paraesthesias e.g. 'crawling' or 'throbbing' sensations movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS) Causes and associations there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy The diagnosis is clinical although bloods to exclude iron deficiency anaemia may be appropriate Management simple measures: walking, stretching, massaging affected limbs treat any iron deficiency dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole) benzodiazepines gabapentin

Question 290

Multiple sclerosis: features

Answer 290

Patient's with multiple sclerosis (MS) may present with non-specific features, for example around 75% of patients have significant lethargy. Visual ## Footnote optic neuritis: common presenting feature optic atrophy Uhthoff's phenomenon: worsening of vision following rise in body temperature internuclear ophthalmoplegia Sensory pins/needles numbness trigeminal neuralgia Lhermitte's syndrome: paraesthesiae in limbs on neck flexion Motor spastic weakness: most commonly seen in the legs Cerebellar ataxia: more often seen during an acute relapse than as a presenting symptom tremor Others urinary incontinence sexual dysfunction intellectual deterioration

Question 291

reduced vision and painful movements in the eye. what does this suggest?

Answer 291

likely due to optic neuritis, a common presenting feature of multiple sclerosis.

Question 292

overview of Atrial septal defects

Answer 292

Atrial septal defects (ASDs) are the most likely congenital heart defect to be found in adulthood. They carry a significant mortality, with 50% of patients being dead at 50 years. Two types of ASDs are recognised, ostium secundum and ostium primum. Ostium secundum are the most common Features ## Footnote ejection systolic murmur, fixed splitting of S2 embolism may pass from venous system to left side of heart causing a stroke Ostium secundum (70% of ASDs) associated with Holt-Oram syndrome (tri-phalangeal thumbs) ECG: RBBB with RAD Ostium primum present earlier than ostium secundum defects associated with abnormal AV valves ECG: RBBB with LAD, prolonged PR interval

Question 293

different types of MS

Answer 293

A variety of subtypes have been identified: Relapsing-remitting disease ## Footnote ``` most common form, accounts for around 80% of patients acute attacks (e.g. last 1-2 months) followed by periods of remission ``` Secondary progressive disease describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapses around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis gait and bladder disorders are generally seen Primary progressive disease accounts for 10% of patients progressive deterioration from onset more common in older people

Question 294

how big a problem are anti epileptic drugs in breastfeeding

Answer 294

The BNF states 'breast-feeding is acceptable with all antiepileptic drugs, taken in normal doses, with the possible exception of barbiturates'

Question 295

Trinucleotide repeat disorders

Answer 295

Trinucleotide repeat disorders are genetic conditions caused by an abnormal number of repeats (expansions) of a repetitive sequence of three nucleotides. These expansions are unstable and may enlarge which may lead to an earlier age of onset in successive generations - a phenomenon known as anticipation\*. In most cases, an increase in the severity of symptoms is also noted Examples - note dominance of neurological disorders Fragile X (CGG) Huntington's (CAG) myotonic dystrophy (CTG) Friedreich's ataxia\* (GAA) spinocerebellar ataxia spinobulbar muscular atrophy dentatorubral pallidoluysian atrophy \*Friedreich's ataxia is unusual in not demonstrating anticipation

Question 296

Causes of Parkinsonism

Answer 296

Causes of Parkinsonism ## Footnote Parkinson's disease drug-induced e.g. antipsychotics, metoclopramide - see below progressive supranuclear palsy multiple system atrophy Wilson's disease post-encephalitis dementia pugilistica (secondary to chronic head trauma e.g. boxing) toxins: carbon monoxide, MPTP

Question 297

Drugs causing Parkinsonism

Answer 297

Drugs causing Parkinsonism phenothiazines: e.g. chlorpromazine, prochlorperazine butyrophenones: haloperidol, droperidol metoclopramide Domperidone does not cross the blood-brain barrier and therefore does not cause extra-pyramidal side-effects so is a good antiemetic here in PD

Question 298

what structures does the facial nerve supply

Answer 298

Supply - 'face, ear, taste, tear' ## Footnote face: muscles of facial expression ear: nerve to stapedius taste: supplies anterior two-thirds of tongue tear: parasympathetic fibres to lacrimal glands, also salivary glands

Question 299

Medication overuse headache features and management

Answer 299

Medication overuse headache is one of the most common causes of chronic daily headache. It may affect up to 1 in 50 people Features ## Footnote present for 15 days or more per month developed or worsened whilst taking regular symptomatic medication patients using opioids and triptans are at most risk may be psychiatric co-morbidity Management (from 2008 SIGN guidelines) simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches) opioid analgesics should be gradually withdrawn

Question 300

what is Lhermitte's syndrome

Answer 300

Lhermitte's syndrome: paraesthesiae in limbs on neck flexion = a sensory feature of MS

Question 301

causes and management of Neuropathic pain

Answer 301

Neuropathic pain may be defined as pain which arises following damage or disruption of the nervous system. It is often difficult to treat and responds poorly to standard analgesia. Examples include: ## Footnote diabetic neuropathy post-herpetic neuralgia trigeminal neuralgia prolapsed intervertebral disc NICE updated their guidance on the management of neuropathic pain in 2013: first-line treatment\*: amitriptyline, duloxetine, gabapentin or pregabalin if the first-line drug treatment does not work try one of the other 3 drugs tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia) pain management clinics may be useful in patients with resistant problems \*please note that for some specific conditions the guidance may vary. For example carbamazepine is used first-line for trigeminal neuralgia

Question 302

subclassification of Peripheral neuropathies

Answer 302

Peripheral neuropathy may be divided into conditions which predominately cause a motor or sensory loss Predominately motor loss ## Footnote Guillain-Barre syndrome porphyria lead poisoning hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth chronic inflammatory demyelinating polyneuropathy (CIDP) diphtheria Predominately sensory loss diabetes uraemia leprosy alcoholism vitamin B12 deficiency amyloidosis Alcoholic neuropathy secondary to both direct toxic effects and reduced absorption of B vitamins sensory symptoms typically present prior to motor symptoms Vitamin B12 deficiency subacute combined degeneration of spinal cord dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

Question 303

Drugs causing peripheral neuropathy

Answer 303

Drugs causing a peripheral neuropathy ## Footnote antibiotics: nitrofurantoin, metronidazole amiodarone isoniazid vincristine tricyclic antidepressants

Question 304

what is Restless legs syndrome

Answer 304

Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present Clinical features ## Footnote uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest paraesthesias e.g. 'crawling' or 'throbbing' sensations movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS) Causes and associations there is a positive family history in 50% of patients with idiopathic RLS iron deficiency anaemia uraemia diabetes mellitus pregnancy The diagnosis is clinical although bloods to exclude iron deficiency anaemia may be appropriate Management simple measures: walking, stretching, massaging affected limbs treat any iron deficiency - a low serum ferritin is most likely to be a cause of secondary restless legs syndrome dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole) benzodiazepines gabapentin

Question 305

what is Meniere's disease

Answer 305

Meniere's disease is a disorder of the inner ear of unknown cause. It is characterised by excessive pressure and progressive dilation of the endolymphatic system. It is more common in middle-aged adults but may be seen at any age. Meniere's disease has a similar prevalence in both men and women. Features ## Footnote recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom a sensation of aural fullness or pressure is now recognised as being common other features include nystagmus and a positive Romberg test episodes last minutes to hours typically symptoms are unilateral but bilateral symptoms may develop after a number of years Natural history symptoms resolve in the majority of patients after 5-10 years the majority of patients will be left with a degree of hearing loss psychological distress is common Management ENT assessment is required to confirm the diagnosis patients should inform the DVLA. The current advice is to cease driving until satisfactory control of symptoms is achieved acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required prevention: betahistine may be of benefit

Question 306

what hapens in Trigeminal neuralgia

Answer 306

Trigeminal neuralgia is a pain syndrome characterised by severe unilateral pain. The vast majority of cases are idiopathic but compression of the trigeminal roots by tumours or vascular problems may occur The International Headache Society defines trigeminal neuralgia as: ## Footnote a unilateral disorder characterised by brief electric shock-like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve the pain is commonly evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas) the pains usually remit for variable periods Management carbamazepine is first-line. failure to respond to treatment or atypical features (e.g. \< 50 years old) should prompt referral to neurology eg for microvascular decompression.

Question 307

Acoustic neuroma overview

Answer 307

Acoustic neuromas (more correctly called vestibular schwannomas) account for approximately five percent of intracranial tumours and 90 percent of cerebellopontine angle Features can be predicted by the affected cranial nerves cranial nerve VIII: hearing loss, vertigo, tinnitus cranial nerve V: absent corneal reflex cranial nerve VII: facial palsy Bilateral acoustic neuromas are seen in neurofibromatosis type 2 MRI of the cerebellopontine angle is the investigation of choice

Question 308

traumatic brain injury basics

Answer 308

primary brain injury may be focal (contusion/haematoma) or diffuse (diffuse axonal injury) diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption and tearing of axons intra-cranial haematomas can be extradural, subdural or intracerebral, while contusions may occur adjacent to (coup) or contralateral (contre-coup) to the side of impact secondary brain injury occurs when cerebral oedema, ischaemia, infection, tonsillar or tentorial herniation exacerbates the original injury. The normal cerebral auto regulatory processes are disrupted following trauma rendering the brain more susceptible to blood flow changes and hypoxia the Cushings reflex (hypertension and bradycardia) often occurs late and is usually a pre terminal event

Question 309

what is Lesch-Nyhan syndrome

Answer 309

hypoxanthine-guanine phosphoribosyl transferase (HGPRTase) deficiency x-linked recessive features: gout, renal failure, neurological deficits, learning difficulties, self-mutilation

Question 310

what are Dystrophinopathies

Answer 310

Overview ## Footnote X-linked recessive due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21 dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleton in Duchenne muscular dystrophy there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form in Becker muscular dystrophy there is a non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form Duchenne muscular dystrophy progressive proximal muscle weakness from 5 years calf pseudohypertrophy Gower's sign: child uses arms to stand up from a squatted position 30% of patients have intellectual impairment Becker muscular dystrophy develops after the age of 10 years intellectual impairment much less common

Question 311

what is Von Hippel-Lindau syndrome

Answer 311

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3 Features ## Footnote cerebellar haemangiomas retinal haemangiomas: vitreous haemorrhage renal cysts (premalignant) phaeochromocytoma extra-renal cysts: epididymal, pancreatic, hepatic endolymphatic sac tumours

Question 312

Third nerve palsy

Answer 312

Features ## Footnote eye is deviated 'down and out' ptosis pupil may be dilated (sometimes called a 'surgical' third nerve palsy) Causes diabetes mellitus vasculitis e.g. temporal arteritis, SLE false localizing sign\* due to uncal herniation through tentorium if raised ICP posterior communicating artery aneurysm (pupil dilated) - Given the combination of a headache and third nerve palsy it is important to exclude a posterior communicating artery aneurysm cavernous sinus thrombosis Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes other possible causes: amyloid, multiple sclerosis \*this term is usually associated with sixth nerve palsies but it may be used for a variety of neurological presentations

Question 313

Epilepsy: pregnancy and breast feeding

Answer 313

The risks of uncontrolled epilepsy during pregnancy generally outweigh the risks of medication to the fetus. All women thinking about becoming pregnant should be advised to take folic acid 5mg per day well before pregnancy to minimise the risk of neural tube defects. Around 1-2% of newborns born to non-epileptic mothers have congenital defects. This rises to 3-4% if the mother takes antiepileptic medication. Other points ## Footnote aim for monotherapy there is no indication to monitor antiepileptic drug levels sodium valproate: associated with neural tube defects carbamazepine: often considered the least teratogenic of the older antiepileptics phenytoin: associated with cleft palate lamotrigine: studies to date suggest the rate of congenital malformations may be low. The dose of lamotrigine may need to be increased in pregnancy

Question 314

Seizures: acute management

Answer 314

Most seizures are self-limiting and stop spontaneously but prolonged seizures may be potentially life-threatening. Basics check the airway and apply oxygen if appropriate place the patient in the recovery position if the seizure is prolonged give benzodiazepines BNF recommend dose for rectal diazepam, repeated once after 10-15 minutes if necessary Neonate 1.25 - 2.5 mg Child 1 month - 2 years 5 mg Child 2 years - 12 years 5 - 10 mg Child 12 years - 18 years 10 mg Adult 10 - 20 mg (max. 30 mg) Elderly 10 mg (max. 15 mg)

Question 315

Levodopa overview

Answer 315

Overview ## Footnote usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to prevent peripheral metabolism of L-dopa to dopamine reduced effectiveness with time (usually by 2 years) no use in neuroleptic induced parkinsonism Adverse effects dyskinesia 'on-off' effect postural hypotension cardiac arrhythmias nausea & vomiting psychosis reddish discolouration of urine upon standing

Question 316

DVLA: neurological disorders

Answer 316

## Footnote • it is the PATIENT’s responsibility to inform DVLA For a driving license to be held, a patient must * be free from seizures completely for one year (includes all events, and auras) * Or only experienced sleep seizures for a period of at least three years • And the DVLA/DVLNI is satisfied that as a driver you are not likely to be a source of danger to the public You can drive on medication, as long as seizure free The guidelines below relate to car/motorcycle use unless specifically stated. For obvious reasons, the rules relating to drivers of heavy goods vehicles tend to be much stricter Specific rules first seizure: 6 months off driving\*. For patients with established epilepsy they must be fit free for 12 months before being able to drive stroke or TIA: 1 month off driving multiple TIAs over short period of times: 3 months off driving craniotomy e.g. For meningioma: 1 year off driving\*\* pituitary tumour: craniotomy: 6 months; trans-sphenoidal surgery 'can drive when there is no debarring residual impairment likely to affect safe driving' narcolepsy/cataplexy: cease driving on diagnosis, can restart once 'satisfactory control of symptoms' chronic neurological disorders e.g. multiple sclerosis, motor neuron disease: DVLA should be informed, complete PK1 form (application for driving licence holders state of health)

Question 317

what happens in Complex regional pain syndrome

Answer 317

Complex regional pain syndrome (CRPS) is the modern, umbrella term for a number of conditions such as reflex sympathetic dystrophy and causalgia. It describes a number of neurological and related symptoms which typically occur following surgery or a minor injury. CRPS is 3 times more common in women. There are two types of CRPS: ## Footnote ``` type I (most common): there is no demonstrable lesion to a major nerve type II: there is a lesion to a major nerve ``` Features progressive, disproportionate symptoms to the original injury/surgery allodynia temperature and skin colour changes oedema and sweating motor dysfunction the Budapest Diagnostic Criteria are commonly used in the UK Management ``` early physiotherapy is important neuropathic analgesia in-line with NICE guidelines specialist management (e.g. Pain team) is required ```

Question 318

what is amaurosis fugax

Answer 318

occlusion of the retinal artery. one eye's vision is progressively lost like a curtain descending over my field of view.

Question 319

alzheimer's disease pathology

Answer 319

Pathological changes macroscopic: widespread cerebral atrophy, particularly involving the cortex and hippocampus microscopic: cortical plaques due to deposition of type A-Beta-amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregation of the tau protein biochemical: there is a deficit of acetylecholine from damage to an ascending forebrain projection Neurofibrillary tangles paired helical filaments are partly made from a protein called tau in AD are tau proteins are excessively phosphorylated

Question 320

alzheimer's disease management

Answer 320

Management NICE now recommend the three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer's disease memantine (a NMDA receptor antagonist) is reserved for patients with moderate - severe Alzheimer's targeting beta amyloid has been disappoiinting. - apparent role for folic acid and B vitamins. HUGE number of social care and practical things eg social services, develop routines, planning ahead, day services to provide breaks for carers, manage depression of carers and patients.

Question 321

sodium valproate side effects

Answer 321

V - A - INCREASED APPETITE, WEIGHT GAIN L - liver failure P - pancreatitis R - reversible hair loss O - oedema A - taxia T - teratogenic, tremor, thrombocytopenia E - encephalopathy

Question 322

what is cervical spondylosis

Answer 322

compression of the cord (myelopathy) and nerve roots. the leading cause of progressive spastic quadriparesis with sensory loss below the neck. signs - limited painful neck movement. can cause lhermitte's. think about the muscles and sensation provided by a particualr root. can cause root compression - radiculopathy. dermatomal distribution. do an MRI to investigate. surgical root decompression. transformaminal steroid injection to reduce swelling.

Question 323

what is a pyramidal distribution of symptoms

Answer 323

weakness involving the arm extensors the small muscles of the hand, lower limb flexors . no wasting. spasticity developes in stronger muscles. UMN weakness affects muscle groups not individual muscles.

Question 324

nerve and root that supplies biceps, triceps and brachioradialis

Answer 324

triceps = c6,7,8 radial nerve brachioradialis = c5,6 radial nerve biceps = Musculocutaneous nerve (C5–C6)

Question 325

dermatome to the nipples

Question 326

dermatome to the umbilicus

Question 327

dermatome to the inguinal ligament

Answer 327

L1

Question 328

where is L5 and S1

Answer 328

l5 = medial side of the big toe s1 = lateral margin of the foot and little toe.

Question 329

tinnitus - causes and managemetn

Answer 329

causes - hearing loss, wax, viral, head injury, excess noise. etc managemet - psychological support. cognitive therapy help. medications are useless. hypnotics at night for sleep. cochlear nerve resection butse is deafness.

Question 330

what is charles bonnet syndrome

Answer 330

Visual release hallucinations, also known as Charles Bonnet syndrome (CBS), is the experience of complex visual hallucinations in a person with partial or severe blindness. First described by Charles Bonnet in 1760,[1][2] it was first introduced into English-speaking psychiatry in 1982. Sufferers, who are mentally healthy people with often significant vision loss, have vivid, complex recurrent visual hallucinations (fictive visual percepts). One characteristic of these hallucinations is that they usually are "lilliputian" (hallucinations in which the characters or objects are smaller than normal). The most common hallucination is of faces or cartoons.[4] Sufferers understand that the hallucinations are not real, and the hallucinations are only visual, that is, they do not occur in any other senses, e.g. hearing, smell or taste.[5][6] Among older adults (\> 65 years) with significant vision loss, the prevalence of Charles Bonnet syndrome has been reported to be between 10% and 40%; a recent Australian study has found the prevalence to be 17.5%

Question 331

types of gait disturbance

Answer 331

Parkinson’s disease Flexed posture, loss of arm swing when walking, small steps, lean forward, difficulty in stopping and changing direction to command. Hemiparesis (stroke) Arm may be flexed and adducted across body (no arm swing). Leg is extended with circumduction when the patient has to swing the leg around (cannot flex the knee – foot would catch on floor if leg brought straight through). 70 Cerebellar disease Wide-based posture (sway with eyes open). Needs to hold on to something to walk. Foot drop (lateral popliteal palsy) High-stepping gait on that side to prevent the foot catching as person brings leg through. Foot drop can be observed (lesser degrees brought out by asking patient to stand on heels). Peripheral neuropathy High stepping ‘stomping’ gait as patient is unaware of position of feet. Very careful as they walk. Arthritis Painful gait (antalgic). Does not want to weight bear through affected joint. Quickly steps through to transfer weight through other leg.

Question 332

Neurological focal deficits

Answer 332

10 S's: Sugar (hypo, hyper) Stroke Seizure (Todd's paralysis) Subdural hematoma Subarachnoid hemorrhage Space occupying lesion (tumor, avm, aneurysm, abscess) Spinal cord syndromes Somatoform (conversion reaction) Sclerosis (MS) Some migraines

Question 333

Peripheral neuropathies: differential

Answer 333

DANG THERAPIST: Diabetes Amyloid Nutritional (eg B12 deficiency) Guillain-Barre Toxic (eg amiodarone) Heriditary Endocrine Recurring (10% of G-B) Alcohol Pb (lead) Idiopathic Sarcoid Thyroid

Question 334

Wernicke-Korsakoff's psychosis: findings

Answer 334

COAT RACK: Wernicke's encephalopathy (acute phase): Confusion Ophthalmoplegia Ataxia Thiamine tx. Korsakoff's psychosis (chronic phase): Retrograde amnesia Anterograde amnesia Confabulation Korsakoff's psychosis

Question 335

rx of myasthenia gravis

Answer 335

neostigmine or pyridostigmine edrophoniumm is the test = tensilon test

Question 336

what is INO

Answer 336

Internuclear ophthalmoplegia (INO) is a disorder of conjugate lateral gaze in which the affected eye shows impairment of adduction. When an attempt is made to gaze contralaterally (relative to the affected eye), the affected eye adducts minimally, if at all. The contralateral eye abducts, however with nystagmus. Additionally, the divergence of the eyes leads to horizontal diplopia. The disorder is caused by injury or dysfunction in the medial longitudinal fasciculus (MLF),

Question 337

common cause of death in motor neurone disease

Answer 337

Motor neurone disease is a progressive degenerative disorder of the nervous system, affecting the anterior horn cells of the spinal cord, the motor nuclei of the brain stem, and the corticospinal tracts. Respiratory failure is a common cause of death in MND patients. The most important cause of respiratory failure and alveolar hypoventilation is the loss of negative intrathoracic pressure, developed by the inspiratory action of the diaphragm, as a consequence of cervical anterior horn cell loss. Inadequacy of inspiratory muscle function leads to global alveolar hypoventilation and respiratory acidosis. Respiratory failure usually develops gradually in the advanced stages of the disease, but occasionally is precipitated. Basically, you get respiratory muscle weakness. In this scenario type two respiratory failure and therefore a respiratory acidosis was precipitated by the patient being given high-flow oxygen. The correct answer is: Respiratory acidosis

Question 338

features of meniere's disease and cause

Answer 338

d) Triad of Meniere’s disease = Vertigo, tinnitus,deafness. It is a disorder of the inner ear thought to be due to swelling of the endolymphatic space.

Question 339

different PCs for extradural, subdural and subarachnoid haemorrhage

Answer 339

Extradural haematomas follow linear skull vault fractures (due to direct injury to the head such as being hit by a baseball bat). There is characteristically a brief moment of unconsiousness followed by a lucid interval and then progressive hemiparesis with subsequent coning. d) Subarachnoid haemorrhages present acutely with severe sudden onset headache at the back of the head. e) This patient has had a subdural haematoma which presents typically with headache, drowsiness and fluctuating confusionfollowing a mild head injury days, weeks or months previously. Subdural haematomas should be considered in the elderly, alcoholicsand patients with a history of falls or patients on warfarin.

Question 340

some serious causes of bilateral papilloedema

Answer 340

Idiopathic intracranial hypertension occurs more commonly in overweight young women. It is associated with headaches and blurred vision. Bilateral papilloedema is often present. Neck stiffness and photophobia are associated with meningitis. Sub-arachnoid haemorrhage usually occurs in association with a thunderclap headache, and can cause bilateral papilloedema. However, is not the most likely diagnosis in a 24 year old female. Malignant hypertension can present with headaches and blurred vision, and is associated with bilateral papilloedema, but again, it is not the most likely diagnosis in this scenario.

Question 341

sudden onset of facial numbness, double vision, unsteadiness of gait and left arm and leg weakness. What is the single most likely diagnosis?

Answer 341

The correct answer is: right posterior inferior cerebellar artery infarction

Question 342

classical description of DLB - diffuse Lewy body disease

Answer 342

A 93 year old man has a series of falls. He has been progressively forgetful and has had episodic hallucinations over the past year. Which is the single most likely diagnosis? This is a classical description of DLB – drop attacks and neuropsychiatric symptoms occur early in the disease. The correct answer is: diffuse Lewy body disease

Question 343

what is Kernig's sign

Answer 343

One of the physically demonstrable symptoms of meningitis is Kernig's sign. Severe stiffness of the hamstrings causes an inability to straighten the leg when the hip is flexed to 90 degrees.

Question 344

what is Brudzinski's sign

Answer 344

The Brudziński neck sign or Brudziński's symptom is a clinical sign in which forced flexion of the neck elicits a reflex flexion of the hips. It is found in patients with meningitis, subarachnoid haemorrhage and possibly encephalitis. It is not very commonly seen.

Question 345

what is De quervain's tenosynovitis

Answer 345

a tenosynovitis of the sheath or tunnel that surrounds two tendons that control movement of the thumb Tenosynovitis is the inflammation of the fluid-filled sheath (called the synovium) that surrounds a tendon. Symptoms of tenosynovitis include pain, swelling and difficulty moving the particular joint where the inflammation occurs Finkelstein's test[11] is used to diagnose de Quervain syndrome in people who have wrist pain. = place your thumb in a closed fist and tilt your hand towards the little finger

Question 346

what is cubital tunnel syndrome

Answer 346

‘cubital tunnel syndrome’, which has been caused by intraoperative compromise and compression of the ulnar nerve at the elbow. The second commonest entrapment neuropathy to carpal tunnel syndrome, the ulnar nerve is particularly vulnerable around the elbow. cross your middle finger over the dorsal surface of the index finger.

Question 347

whta CN lesion causes a person to tilt their head to reduce the diplopia and why

Answer 347

This man’s head tilt is characteristic of a trochlear nerve lesion: patients usually tilt away from the side of the lesion in order to reduce their diplopia. The trochlear nerve has three roles: intorsion, depression, and abduction of the globe. It is most commonly disturbed by head trauma, but can be aff ected—as here—in microvasculopathies such as diabetes. The diplopia is worse on downward gaze and gaze away from the aff ected muscle.

Question 348

what antithrombotic drugs are given after a TIA or stroke?

Answer 348

for the first 2 weeks after both, aspirin 300mg is given then: TIA = aspirin and dipyridamole Stroke = clopidogrel

Question 349

drugs given in status epilepticus

Answer 349

their normal antiepileptic meds first line : maximum 2 doses of PR diazepam/buccal midazolam/ IV lorazepam or diazepam if this fails then start a phenytoin infusion. if this fails call an anaesthetist and warn the ITU. induce general anaesthesia and monitor EEG.

Question 350

common peroneal nerve lesion

Answer 350

This man has a foot drop and weak eversion—symptoms of a common peroneal nerve lesion. The common peroneal nerve is commonly damaged by trauma to the lateral side of the knee. It is here that it winds around the head of the fi bula covered only by skin and subcutaneous tissue. After entering the peroneus longus muscle, it divides into deep and superfi cial branches. It is the superfi cial branch that provides sensory innervation to the skin of the lower lateral calf and dorsum of the foot. The deep branch is primarily a motor nerve.

Question 351

A 30-year-old woman has had diffi culty walking for the past 12h. She fi rst noticed it when she left work the previous night: her walk to the station, which usually takes only a few minutes, on this occasion took over an hour. She has grade 3 power in her lower limbs and an extensor plantar response bilaterally. Her range of eye movements is normal but painful. Which is the single most likely cause of her symptoms?

Answer 351

This woman has acute lower limb weakness and what sounds like an optic neuritis. Although MS can present in a variety of ways, these are two of the most common initial symptoms. More commonly, patients present with one symptom that improves, only for another diff erent problem to develop some time later. Anyone in this demographic who gives a history of fl itting, seemingly unlinked (by time and space) neurological problems should raise suspicions and prompt an in-depth history and careful examination.

Question 352

relevance of the OCP to migrane

Answer 352

migrane can be triggered by the OCP

Question 353

what is battle's sign

Answer 353

ecchymosis (bruising) of the mastoid process whcih along with periorbital ecchymosis, CSF rhino/otorrhoea and haemotympanum should prompt urgent imaging in case of basal skull fracture.

Question 354

damage to broca's area causes

Answer 354

slow and non-fluent speech. an expressive dysphasia. Broca’s area ( Figure 8.8 ) is the area responsible for the production of speech. Although this man understands what is being said to him (Wernicke’s area is intact), he cannot generate words fl uently, which leads to expressive dysphasia.

Question 355

what happens if the tibial nerve is damaged

Answer 355

This man is unable to plantar fl ex or invert his foot due to an injury to the tibial nerve. The tibial nerve supplies a sensory branch to the sole of the foot and a motor branch to the hamstrings, tibialis posterior, gastrocnemius, fl exor digitorum longus, and the small muscles of the foot.

Question 356

A 65-year-old woman has fallen at home. She has had increasingly regular headaches for several weeks. She has hypertension, type 2 diabetes, and Crohn’s disease and takes bendrofl umethiazide, amlodipine, metformin, gliclazide, prednisolone, and azathioprine. A CT head scan is carried out, you see multiple possibly ring enhancing lesions, whats going on?

Answer 356

The brief history is suggestive of cerebral space-occupying masses. The MRI scan (coronal view) shows multiple discrete ring-enhancing lesions. Diff erentials for these appearances include metastases (most commonly from lung, kidney, breast, melanoma, and colon), demyelination, multiple infarcts, and, in patients who are HIV positive, lymphoma. However, in those who have been on long-term immunosuppression like this woman, the most likely cause are abscesses. They are diff erent from metastases in that they cause surrounding oedema and have thinner walls. The most common infections that can cause multiple small lesions are toxoplasmosis, cryptococcosis, and cysticercosis.

Question 357

mortality rates for wernicke's and korsakoff's

Answer 357

The acute onset of ophthalmoplegia (nystagmus as here or lateral rectus or conjugate gaze palsies), an ataxic gait, and global confusion is known as Wernicke’s encephalopathy: it results from thiamine defi ciency (which is common in heavy alcohol users) and can proceed to the more serious Korsakoff ’s syndrome (characterized by a retrograde amnesia resulting in confabulation). Untreated, mortality rates are 20% in Wernicke’s and 85% in Korsakoff ’s. Apart from arresting the decline into Korsakoff ’s, thiamine has been shown variously to reverse all three clinical problems within hours. Therefore, in any patient with one or more of the three symptoms and no other more likely cause, give two pairs of thiamine ampoules IV in 50–100mL 0.9% saline over 30min three times a day for 3–7 days before converting to oral thiamine.

Question 358

presentation of acute alcohol withdrawal

Answer 358

Confusion is common following surgery, especially in the elderly. This woman has a few features particular to the diagnosis: alcohol withdrawal. This classically presents between 10 and 72h after admission with hypotension, tachycardia, and visual/tactile hallucinations. She should be given generous amounts of chlordiazepoxide (a benzodiazepine) for the fi rst 3 days, which is then gradually reduced.

Question 359

a patient has had a TIA and is in AF - what do you do?

Answer 359

This woman has had a transient ischaemic attack (TIA) and is in atrial fi brillation (AF). According to NICE guidelines, she needs to be anticoagulated, but before this can happen, a recent infarct and a haemorrhagic cerebral event need to be excluded, hence the CT. If she had had a stroke, then the guidance is that a CT scan is performed to exclude The decision to anticoagulate is not based on neurological fi ndings ( D ) or the rate of AF ( E ) and should not be postponed ( B ). All those in AF should be risk stratifi ed for stroke. As this woman has just had a TIA, she immediately becomes high risk. Other high-risk criteria are: 1. Previous ischaemic stroke or thromboembolic event. 2. Age \>75 years with hypertension, diabetes, or vascular disease. 3. Clinical evidence of valve disease, or heart failure of left ventricular dysfunction on echocardiography. Unless there are contraindications, all those in the high-risk group should be anticoagulated with warfarin to a target INR of 2.5. haemorrhage, and warfarin is started 2 weeks later.

Question 360

when should antiepileptic treatment be started?

Answer 360

In most cases, anti-epileptic treatment does not start until after a second seizure. However, NICE guidance highlights four situations in which it should be started after the fi rst seizure: 1. The individual has a neurological defi cit. 2. The electroencephalogram (EEG) shows unequivocal epileptiform activity. 3. The individual considers risk of further seizures unacceptable. 4. Imaging shows a structural abnormality. Although this woman’s defi cit is likely to be temporary (Todd’s paresis following a seizure involving the motor cortex), it is a sign that the seizure was likely to be epileptic in origin and that the risk of recurrence is high

Question 361

SE of cyclizine in patients with parkinson's disease

Answer 361

Cyclizine will exacerbate the extra-pyramidal symptoms of Parkinson’s disease due to its central anti-cholinergic eff ects. This manifests as confusion, diffi culty walking, and an increase in tone.

Question 362

The ‘swinging fl ashlight test’ reveals

Answer 362

The ‘swinging fl ashlight test’ reveals an abnormal response in the right pupil. The fact that the right eye produces less pupillary constriction suggests an aff erent defect on that side. Although it may appear that both pupils are dilating, this is just relative: they are, in fact, both trying to constrict, but failing to do so fully due to the partial aff erent nerve damage that distinguishes this condition. In this case—a young woman with concurrent headache—the damage may have occurred following an optic neuritis and may be indicative of a history of multiple sclerosis. Full dilation and lack of a subsequent response would suggest a total CN II lesion.

Question 363

use of urinary alkalinisation

Answer 363

This uses sodium bicarbonate to produce urine with a pH between 7.5 and 8. This can enhance elimination of weak acids such as cocaine, tricyclic antidepressants, and salicylates.

Question 364

management of benzodiazepine overdose

Answer 364

Treatment of benzodiazepine overdose is supportive with maintenance of the airway, regular assessment of consciousness level, and IV fl uids Although fl umazenil is a benzodiazepine antagonist, it is not used to reverse purposeful overdose because there is no way of being sure how much of the hypnotic has been taken. Flumazenil can trigger seizures via the benzodiazepine receptor as a result of the reduction in the seizure threshold. It is only used if the overdose has been caused during a procedure in the hospital environment.

Question 365

An 82-year-old woman has rapidly become unable to speak. Her family report that she has been increasingly tired over the last 3 weeks and latterly has been restricted to sitting in a chair. Prior to this, she walked with a stick and was independent with regard to all activities of daily living. She is awake but not able to follow commands and is therefore diffi cult to examine. T 38.4 ° C, HR 105bpm, BP 100/65mmHg. Tone and refl exes are globally increased with equivocal plantars but no focal neurology. Which single investigation is the most likely to produce a defi nitive diagnosis

Answer 365

This is viral encephalitis. Cerebrospinal fl uid examination obtained by a lumbar puncture is the most appropriate option. It usually shows a raised protein and lymphocyte count with a normal glucose concentration but can be normal.

Question 366

rx of benign paroxysmal positional vertigo (BPPV).

Answer 366

This is a classic history for benign paroxysmal positional vertigo (BPPV). Displaced otoconia can be relocated by the Epley manoeuvre or Brandt– Daroff exercises and provide a cure in up to 80–90% of cases in some studies

Question 367

presentation of cervical spondylosis

Answer 367

painful neck and symptoms consistent with nerve root compression This man has cervical spondylosis associated with a radiculopathy—pain, reduced refl exes, dermatomal sensory disturbance and lower motor neurone (LMN) weakness.

Question 368

use of ABCDE score

Answer 368

This man has had a TIA and needs to be assessed to establish his risk for a subsequent stroke. A reliable way of assessing this risk is using the ABCD2 score: A ge \>60 years (1 point), B P \>140/90 mmHg (1 point) C linical features— speech disturbance without unilateral weakness (1 point), unilateral weakness (2 points), D uration 10–59 minutes (1 point), \>60min (2 points), D iabetes (1 point). Notably, the presence of family history and sensory symptoms play no part in his risk assessment for a subsequent stroke. A score of 3 or below: aspirin 300mg daily starting immediately and specialist assessment within 7 days. A score of 4 or more: aspirin 300mg daily starting immediately with specialist assessment within 24h. The man scores 2 for his symptoms and therefore requires 2 further points to trigger an urgent admission, which he would get if his symptoms had lasted for more than 1h.

Question 369

HHS management

Answer 369

Reduced consciousness level in a patient with type 2 diabetes is initially suggestive of a hyperosmolar state. This is clearly confi rmed by the laboratory fi ndings which show not only hyperglycaemia but also hypernatraemia, considerable dehydration and a raised serum osmolality (2 × (Na + K) + Ur + glucose). There are several important management steps at this time: 1. Insulin therapy (1U/mL of 0.9% saline at a rate of 6U/h). 2. Anticoagulation therapy: as these patients are at increased risk of venous thrombosis. 3. Fluid replacement: most patients are fl uid depleted by 5L or more. Normal-strength saline is eff ective at rapidly restoring circulating volume and does so without dropping the serum osmolality too quickly, which is a risk for the development of cerebral oedema. If the sodium remains high after 2–3L of infusion fl uids, then half-strength normal saline can be used. Clearly it would be dangerous to use any glucose products, while sodium bicarbonate is usually discouraged unless in the setting of extreme acidosis and under the guidance of experienced renal or intensive care clinicians. Given the amount of fl uid that needs to be replaced, it is worth bearing in mind that these patients—especially if they are over 65 years old and/ or have ischaemic heart disease—will likely need a CVP line and urinary catheter.

Question 370

presentation of vasovagal syncope

Answer 370

A 42-year-old woman lost consciousness while standing on a busy train. She became hot and nauseated and her vision ‘closed in’ before she collapsed. She was witnessed to have jerked a few times on the ground before she regained consciousness. She has never had anything like this before and feels fi ne now. This is a classical description of a simple faint followed by some hypoxic ‘jerks’, which can be mistaken for epilepsy. However, the preceding symptoms and recovery afterwards are typical of a vasovagal syncope.

Question 371

A 42-year-old woman talks in short, clipped sentences. She tries hard to enunciate every syllable but does run some together— particularly the last sounds of longer words. Her speech is irregular in volume but of a regular, almost automated rhythm and tone.

Answer 371

This woman has vocal symptoms typically experienced in multiple sclerosis. It is caused by ataxic muscles: dysarthria with scanning, staccato speech.

Question 372

what happens in a stokes- adams attack

Answer 372

This man has a classical history for Stokes–Adams attacks: syncope due to transient ventricular fi brillation or asystole. This might be captured on a 24h Holter monitor rather than a one-off ECG A 72-year-old man has lost consciousness and has fallen a number of times in the last month. He has no chest pain and gets no warning that these falls are going to occur. His wife says he goes white and his arms twitch and about 30s later he goes pink again.