Neurology

Question 1

Describe neurofibromatosis, it’s types, and features and diagnosis.

Answer 1

There are two types NF1 and NF2 both are automsomal dominant.

NF1 features with cafe-au-lait spots, axillary/groin freckles, peripheral neurofibromas, iris hamartomas, scoliosis, phaeochromocytomas.

NF2 features with bilateral acoustic neuromas.

Diagnosis of NF1 assessed by seven clinical criteria at least two of which should be present:

• Six or more Cafe-au-lait spots or hyper pigmented macules greater than of equal to 5mm in diameters in pre-pubertal individuals and 15mm in post-pubertal individuals.
• Two or more neurofibromas or one plexiform neurofibroma
• Axillary of inguinal freckles
• Two or more iris hamartomas (Lisch nodules), often identified only thought slit-lamp examination by an ophthalmologist
• optic nerve glioma
• Sphenoid dysplasia or typical long-bone abnormalities such as psuedouarthrosis
• First-degree relative with NF1.

Question 2

What are the features of a UMN lesion?

Answer 2

Affects muscle groups
Upgoing plantar reflex (+ve babinski)/clonus
Hyperreflexia
No fasciculations
Spasticity/hypertonia

Question 3

What are the featured of a LMN lesion?

Answer 3

Caused by damage from anterior horn cells, nerve roots, plexi, or peripheral nerves

Wasting and fasciculations
Hyporeflexia
Downward going plantars (-ve babinski)
Hypotonia (flaccidity)

Question 4

What does the anterior cerebral artery supply and what symptoms arise from its occlusion?

Answer 4

Supplies the frontal and medial part of the cerebrum.

Occlusion may cause a weak,numb contralateral leg +/- similar but often milder picture in the arms. The face is spared.

Question 5

What does the middle cerebral artery supply and what symptoms arise from its occlusion?

Answer 5

Supplies the lateral part of each hemisphere

Occlusion may cause a weak contralateral hemiparesis, Hemisensory loss (esp arm + face), contralateral homonymous hemianopia.

Question 6

What does the posterior cerebral artery supply and what symptoms arise from its occlusion?

Answer 6

Supplies occipital lobe

Occlusion gives contralateral homonymous hemianopia (often with macular sparing)

Question 7

Describe subclavian steal syndrome

Answer 7

Subclavian artery stenosis proximal to the origin of the vertebral artery may cause blood to be stolen by retrograde flow down this vertebral artery down into the arm, causing brain stem ischaemia typically after use of the arm.

Suspect if the BP in each arm differs by >20mmHg

Question 8

What are some causes of an acute single episode headache?

Answer 8

• With meningism: meningitis, encephalitis, subarachnoid haemorrhage
• Head injury
• Venous sinus thrombosis
• Sinusitis
• Tropical illness
• Low pressure headache
• Acute glaucoma

Question 9

What are some causes of recurrent acute attacks of headache?

Answer 9

• migraine
• cluster headache
• trigeminal neuralgia
• recurrent (mollaret’s) meningitis

Question 10

What are some causes of subacute onset headaches?

Answer 10

Giant cell arteritis should be excluded in all >50yrs old with a headache that has lasted a few weeks.

Question 11

What are some causes of chronic headache?

Answer 11

• Tension Headache
• raised ICP
• Medication overuse (analgesic rebound) headache

Question 12

Describe tension headache, its symptoms, and management

Answer 12

Symptoms:
Usual cause of bilateral, non pulsating headache +/- scalp tenderness, but without vomiting or sensitivity to head movement.

Management:

• Stress relief, massage, or antidepressant (low dose amitryptilline) may help.
• Consider seeing optician

Question 13

Describe raised intracranial pressure, its symptoms and tests.

Answer 13

Symptoms:
Typically worse on waking, lying, bending forward, or coughing. Also vomiting, papilloedema, seizure, false-localising signs, or odd behaviour

Tests: Do imaging to exclude a space-occupying lesion, and consider idiopathic intracranial hypertension. LP is contraindicated until after imaging!

Question 14

Describe Acute Glaucoma, it’s presentation, symptoms, and management.

Answer 14

Presentation: Typically elderly, long-sighted people. Constant, aching pain develops rapidly around one eye, radiating to the forehead. Attacks may be precipitated by dilating eye-drops, emotional upset or sitting in the dark.

Symptoms: markedly reduced vision, visual haloes, nausea/vomiting.

Signs: red congested eye, cloudy cornea, fixed dilated non-responsive pupil (may be oval), decreased acuity.

Management:
seek expert help, if delay of treatment of >1h start acetazolamide 500mg IV over several minutes.

Question 15

Describe Cluster Headaches, its features, and management

Answer 15

Unilateral severe headache that occurs once or twice a day for 15-180mins and is often nocturnal, usually lasting several weeks, with the clusters themselves occuring typically once a year. Clusters may be episodic or chronic. 5 times more common in men, can occur at any age and more common in smokers.

Features:
-Rapid-onset of excruciating stabbing pain around one eye that may become watery and bloodshot with lid swelling, lacrimation, facial flushing, rhinorrhoea, miosis+/- ptosis.

Management:

• acute attack: 100% O2 (80% response rate within 15 minutes) + Sumatriptan SC 6mg (75% response rate within 15 minutes)
• Prophylaxis: Verapamil is the drug of choice, some evidence supports a tapering dose of prednisolone
• Neurology referral

Question 16

Describe epilepsy and its suggestive features.

Answer 16

Epilepsy is a recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifesting as seizures.

Features suggestive of epilepsy: attacks when asleep or lying down, aura, identifiable triggers (e.g. tv) altered breathing, cyanosis, typical tonic-clonic movements, incontinence, tongue-biting, prolonged post-ictal drowsiness (slow recovery), confusion, amnesia, and transient focal paralysis (Todd’s palsy)

Question 17

What are some causes of seizures?

Answer 17

• Idiopathic ~ 2/3
• Structural: (cortical scaring, developlmental, space-occupying lesion, stroke, hippocampal sclerosis, vascular malformations)
• Others: tuberous sclerosis, sarcoidosis, SLE, PAN
• Non-epileptic causes: trauma, stroke, haemorrhage, increased ICP, alcohol or benzodiazepine withdrawal, metabolic disturbance (e.g. hypoxia, hypo/hypernatraemia, hypocalcaemia, hypo/hyperglycaemia, uraemia) liver disease, infection, hyperthermia, drugs (tricyclics, cocaine, tramadol, theophylline), pseudoseizures.

Question 18

What are the classifications of seizures?

Answer 18

The basic classification of epilepsy has changed in recent years. The new basic seizure classification is based on 3 key features:

• where seizure begin in the brain
• level of awareness during a seizure
• other features of seizures

Focal seizures:

• previously termed partial seizures
• these start in specific area, on one side of the Brain.
• the level of awareness can vary, the terms focal aware (previously simple partial) or focal awareness impaired (previously complex partial) and awareness unknown are used to further differentiate focal seizures
• Further to this focal seizures can be classified as being motor (e.g. Jacksonian march), non-motor ( e.g. dejavu, jamais vu). or having other features such as aura.

Generalised seizures:

• these engage or involve networks job both sides of the brain at onset.
• Consciousness is lost immediately.
• Generalised seizures can be further subdivided into motor (e.g. tonic-clonic) and non-motor (e.g. absence)
• specific types include, tonic-clonic, tonic, clonic, absence, myoclonic, atonic.

Unknown onset: a term reserved for when origin of the seizure is unknown.

Focal to bilateral:

• starts on one side in a specific area before spreading to both lobes
• previously termed secondary generalised seizures.

Question 19

Define a focal aware seizure

Answer 19

Awareness is unimpaired. with focal motor, sensory, autonomic or psychic symptoms. no post-ictal symptoms.

Question 20

Define a focal impaired awareness seizure

Answer 20

awareness is impaired. may have a simple partial onset or impaired awareness at onset. most commonly arise from the temporal lobe. Post-ictal confusion is common with seizure arising from the temporal lobe, whereas recovery is rapid after seizures in the frontal lobe.

Question 21

Define a focal to bilateral seizure

Answer 21

occurs in ~ 2/3 of patients with partial seziures, electrical disturbances begin focally as either complex or simple seizures but then spread widely, causing a secondary generalised seizure which is typically convulsive.

Question 22

Describe Absence seizures, and its management

Answer 22

Brief (less than 10s) pauses, e,g, suddenly stops talking mid-sentence then continues where they left off. Presents in childhood 4-8yrs.

Management:

• Sodium Valproate, Ethosuximide
• Good prognosis 90-95% become seizure free in adolescence.

Question 23

Define tonic-clonic seizures

Answer 23

loss of consciousness. limbs stiffen (tonic), the jerk (clonic) may have one without the other. post-ictal confusions and drowsiness occur.

Question 24

Define myoclonic seizures

Answer 24

sudden jerk of a limb, face of trunk. the patient may be thrown suddenly to the ground or have a violently disobedient limb.

Question 25

Describe atonic (akinetic) seizures

Answer 25

sudden loss of muscle tone causing a fall, no loss of consciousness

Question 26

What are some localising features of partial focal temporal lobe seizures?

Answer 26

- Automatisms (brief complex motor phenoma with impaired awareness/ no recollection e.g. may be oral or manual. - abdominal rising sensation +/- pain - dysphasia - memory phenonema e.g. dejavu or the opposite jamais vu - hippocampal involvement may cause emotional disturbance e.g. terror, anger - Uncal involvement may cause hallucinations fo smell or taste - Auditory hallucinations - delusional behaviour

Question 27

What are some localising features of partial focal frontal lobe seizures?

Answer 27

- Motor features - motor arrest - subtle behavioural distubances - dysphasia or speech arrest - post-ictal todd's palsy

Question 28

What are some localising features of partial focal parietal lobe seizures?

Answer 28

- sensory disturbances | - motor symptoms

Question 29

What are some localising features of partial focal occipital lobe seizures?

Answer 29

visual phenomena such as spots lines or flashes

Question 30

Define pseudo- or psychogenic seizures

Answer 30

these are not infrequent suspect if there are uncontrollable symptoms, no learning disabilities, and CNS, CT, MRI and EEG are all normal. May be part of Munchausen's syndrome

Question 31

What are the drug used in treatment of epilepsy?

Answer 31

Generalised tonic-clonic seizures - sodium valproate or lamotrigine are 1st line, then carbamazepine or topiramate. Absence seizures - ethosuximide, sodium valproate, lamotrigine. Tonic, atonic, and myoclonic seizures same as tonic-clonic but no carbamazepine as it may worsen seizures. Focal seizures - carbamazepine is first line then lamotrigine and valproate

Question 32

What are the side effects of sodium valproate

Answer 32

``` V alproate side effects A ppetite increased leading to weight gain L iver failure (LFT monitoring) P ancreatitis R eversible hair loss (grows back curly) O edema A taxia T eratogenicity, tremor, thrombocytopenia (Monitor bloods) E ncephalopathy ```

Question 33

How do you assess risk of stroke in patients presenting with TIA?

Answer 33

ABCD2 score - >6 strongly predicts stoke, >4 should be assed within 24 hours all suspected TIA should be seen within 7 days. ``` A ge>60 = 1 point B lood pressure >140/90 = 1 point C linical features unilateral weakness = 2 points speech disturbance without weakness = 1 point D uration of symptoms lasting > 1h = 2 points lasting 10-59 mins = 1 point D iabetes = 1 point ```

Question 34

Describe Cauda equina syndrome and its symptoms.

Answer 34

A neurosurgical emergency! Symptoms: Features include back pain and radicular pain down the legs, assymetrical (alternating or bilateral), atrophic, areflexic paralysis of the legs. Sensory loss in a root distribution (saddle anaesthesia) and decreased sphincter tone causing bladder and bowel incontinence (Do PR)

Question 35

What would you expect to see on CSF analysis from LP of suspected bacterial meningitis?

Answer 35

Appearance: cloudy Glucose: low less than half plasma glucose Protein: high >1g/L White cells: 10-5000 polymorphs/mm

Question 36

What would you expect to see on CSF analysis from LP of suspected tuberculous meningitis?

Answer 36

Appearance: slightly cloudy, fibrin web Glucose: low less than half plasma glucose Protein: high >1g/L White cells: 10-1000 lymphocytes/mm

Question 37

What would you expect to see on CSF analysis from LP of suspected viral meningitis?

Answer 37

Appearance: clear/cloudy Glucose: 60-80% plasma glucose Protein: normal/raised White cells: 15-1000 lymphocytes/mm

Question 38

What are the main features of Parkinsonism?

Answer 38

* Tremor (worst at rest, often 'pill-rolling') * bradykinesia (diagnostic feature) * rigidity/ increased tone (tremor + rigidity gives cogwheel rigidity felt during pronation and supination of arm)

Question 39

What are some causes of Parkinsonism?

Answer 39

- idiopathic Parkinson's, - drug induced (anti-psychotics and anti emetics (domperidone does not cross the blood brain barrier) - Progressie supranuclear palsy - Multiple system atrophy - Dementia Pugilistica (secondary to trauma/boxing) - Wilson's disease - Post-encephalitis - HIV - vascular - Toxins e.g. Carbon monoxide, MPTP

Question 40

Describe idiopathic Parkinson's disease, it's presentation, and management..

Answer 40

Incurable progressive disease characterised by degeneration of neurones in the substantia Nigra pars compacta associated with lewy bodies which leads to a reduction in striatal-like dopamine Presentation: bradykinesia + one or more of: resting tremor, postural instability, rigidity. Typically one side is worst often right side. Also micrographia, depression, decreased cognition (Lewy body dementia), slow festinating gait, simian posture, Management: levodopa, dopamine agonists (ropinirole), anticholinergics, MAO-B inhibitors (selegiline), COMT inhibitors (entacapone)

Question 41

What are some causes of nystagmus?

Answer 41

* horizontal nystagmus may be due to vestibular or cerebellum lesions. If it occurs more in the eye abducting cause may be MS. If also deafness and tinnitus may be peripheral cause e.g. VIIIth nerve lesion * vertical nystagmus requires specialist review, upbeat nystagmus classically occurs in lesions of midbrain or base of 4th ventricle. Downbeat nystagmus in foramen magnum lesions.

Question 42

What are the symptoms of a cerebellum lesion?

Answer 42

``` D ysdiadokokinesis and dysmetria A taxia S lurred speech/stoccato speech H ypotonia I ntention tremor N ystagmus G ait abnormality ```

Question 43

Describe Rhomberg's test and meaning of its results

Answer 43

Rhomberg's test is used to assess a patient proprioception when standing with their eyes closed. A loss of balance is a positive test In the context of ataxia a patient with a positive Rhomberg's suggest the ataxia is sensory in nature if it is negative it suggest the ataxia is of cerebellum origin

Question 44

Describe subarachnoid haemorrhage, its symptoms, and management, and complications

Answer 44

80% are due to rupture of saccular aneurysms. Common sites for berry aneurysms are the junction of posterior communicating artery with the internal carotid (may present with pupil changes indicating a IIIrd nerve palsy) or the junction of anterior communicating artery with the anterior cerebral artery or bifurcation of the middle cerebral artery. Symptoms: Usually presents with sudden onset devastating occipital headache, vomiting, collapse,seizures and coma often follow. May report a sentinel headache. Signs include Kernigs sign, neck stiffness. Management: - Do CT within 4 hours of presentation and if positive refer to neurosurgeons for endovascular coiling or less preferred clipping. - If CT negative and no contraindications do LP 12 hours after presentation. Xanthachromia confirms Subarachnoid haemorrhage. - Maintain BP and nimodipine to reduce vasospasm. Complications: - Hydrocephalus (occurs a few days after) - Raised Intracranial pressure - Rebleeding (Occurs in first 1-2 days or after 2 weeks) - Delayed Ischaemia (Occurs around day 10)

Question 45

What is Kernig's sign?

Answer 45

Kernig's sign is a positive when the hip is flexed to 90 degrees and there is paining resistance of extension of the knee. It is indicative of subarachnoid haemorrhage or meningitis

Question 46

What is battle's sign?

Answer 46

Bruising of the mastoid process a sign of basal skull fracture

Question 47

What is Panda or Raccoon eyes a sign of?

Answer 47

It is bruising of the eyes a sign of basal skull fracture

Question 48

Describe IIIrd Cranial Nerve palsy and its causes

Answer 48

Ptosis, large pupil, eye down and out. If lesion present on it own causes may be: - Medical (pupil sparing) e.g. Diabetes, HTN, GCA, syphillis idiopathic. - Surgical (early pupil involvement) e.g. posterior communicating artery aneurysm, Increased ICP, tumours.

Question 49

Describe IVth Cranial Nerve palsy and its causes.

Answer 49

Diplopia on looking down and in (often noticed on descending stairs) head tilting compensates for this (ocular torticollis). Lesion rarely occurs on its own but possible after trauma to orbit. More commonly presents with other lesions.

Question 50

Describe VIth Cranial Nerve palsy and possible causes.

Answer 50

The Abducens nerve. Horizontal diplopia on abduction. False localising sign due to long intracranial course in Raised ICP, SOL,

Question 51

Describe acoustic neuroma and its presentation, investigations, and management

Answer 51

It is a schwannoma arising from the vestibular nerve. They account for 80% of cerebello-pontine angle tumours. Commoner in females and also in neurofibromatosis. Presentation: progressive unilateral tinnitus +/- sensorineural hearing loss, with vertigo occuring later. With progresion, ipsilateral Vth,VIIth, VIIIth, (IXth, Xth nerves may be affected later). Signs of increased ICP occur late, indicating a large tumour. Investigations: MRI in all with unilateral tinnitus and deafness Management: Surgery

Question 52

Describe the red flags present in parkinson's-plus syndrome

Answer 52

- Early postural instability and vertical gaze palsy +/- falls, rigidity of trunk > in limbs, symmetrical onset, speech and swallowing problems, little tremor = Progressive Supranuclear Palsy (PSP) - Early autonomic features e.g. impotence/incontinence, postural BP drop, cerebellar + pyrimidal signs, rigidity>tremor = Multiple System Atrophy (MSA) - Fluctuating cognition with visual hallucinations and early dementia = lewy-body dementia - akinetic rigidity involving one limn and cortical sensory loss e.g. astereognosis (inability to recognise objects by touch alone) = cortico-basal degeneration - Pyramidal signs e.g. in a diabetic/hypertensive patient who falls or has gait problems with no festination = vascular parkinsonism

Question 53

Describe the pull test

Answer 53

a sharp tug on the back of a patients shoulders to test for postural instability a positive result is one in which a patient is unable to correct there balance and continues falling backwards after the tug.

Question 54

What are some causes of cerebellar syndrome?

Answer 54

``` Posterior Fossa Tumour Alcohol Sclerosis (Multiple) Trauma Rare e.g. Iithium Inherited - Friedrich's ataxia Epilepsy medications e.g. Phenytoin, carbamazepine Stroke ```

Question 55

Describe hemispatial neglect (hemiagnosia)

Answer 55

Damage to one hemisphere of the brain leads to a deficit in attention to and awareness of one side of observed space. It is usually contralateral to the lesion. Usually due to a lesion of the parietal cortex but in particular neglect is closely related to damage of the temporo-parietal junction and posterior parietal cortex.

Question 56

Describe Visual extinction

Answer 56

Due to damage to the parietal lobe, similar but distinct from hemispatial neglect (hemiagnosia). Visual extinction describes the symptom in which patients have difficulty perceive contralesional stimuli e.g. moving fingers, when presented simultaneously wth an ipsilesional stimulus; but able to identify both stimuli when presented individually.

Question 57

What is the difference between spasticity and rigidity

Answer 57

Spasticity is velocity dependant the initial fast movement is resisted and then gives way, usually uni-directional whereas rigidity is independent of velocity and is bidirectional.

Question 58

Describe lateral medullary syndrome

Answer 58

Occurs after occlusion of most commonly the vertebral artery but also posterior inferior cerebellar artery. Present with: - cerebellar features e.g. ataxia, nystagmus - brainstem features e.g. ipsilateral dysphagia, facial numbness, cranial nerve palsy e.g. Horner's and contralateral limb sensory loss

Question 59

Describe Dysarthria

Answer 59

Dysarthria is a disorder of speech due to disturbance of muscular control. Can be caused by UMN lesions of the cerebral hemisphere or LMN of the brain stem. There may be some variation depending on the site of the lesion: - slurred and weak articulation with a weak voice is typical of pseudobulbar palsy from a stroke. - slurred, scanning and staccato speech caused by cerebellar lesions is typical of MS. - Dysrhythmic, dysphonic, monotonous voice can be due to disease of the extrapyramidal system in Parkinson's.

Question 60

Describe dysphasia

Answer 60

Dysphasia is impaired ability to understand or use language.it is due to a lesion of the dominant hemisphere and may include impaired ability to read, write and use gestures. Wernicke's dysphasia - lesions are located in temporal lobe specifically though to be in posterior section of superior temporal gyrus. Patients are unable to understand language speech is fluent but lacking content. Patients lack awareness of the speech difficulties and my get agitated Broca's dysphasia - lesions are located in the frontal lobe, also known as expressive dysphasia as patient are unable to speak fluently, difficult to understand but comprehension is good. patients are aware of their speech difficulties. Conduction dysphasia - lesions around the arcuate fasciculus, posterior parietal and temporal regions. leads to naming deficits inability to repeat non meaningful words, although there is apparently normal speech comprehension and production. Deep dysphasia - lesions are in the temporal lobe, symptoms are word repetition problems. -Global dysphasia - occurs with extensive damage to basal ganglia, thalamus, symptoms are extensive with generalised deficits in comprehension, repetition, naming and speech production.

Question 61

Name some of the visual fields defects and their causes

Answer 61

Bitemporal hemianopia - if upper quadrant affected more than lower = inferior chiasmal compression commonly due to pituitary tumour, if opposite way then superior chiasmal compression, commonly a cranipharyngioma. Homonymous hemianopia with macular sparing = lesion of occipital cortex. Homonymous hemianopia with incongruous defects tends to be optic tract lesion where as congruous defects point towards optic radiation of occipital lesion. Homonymous quadrantopias: -superior = lesion of temporal lobe -inferior = lesion of parietal lobe Remember PITS Posterior-Inferior, Temporal-Superior

Question 62

Describe the spinothalamic tracts and their journey through the CNS.

Answer 62

Spinothalamic tracts carries information related to pain, temperature, non-discriminative touch, and pressure. Primary afferent fibres synapse in the contralateral dorsal horn, secondary neurones decussate through the ventral white commissure to the spinothalamic tract which lies ventral and lateral to the ventral horn. From here the tract ascend, and in the brainstem fibres run in close proximity to the medial lemniscus and are known as the spinal lemniscus. The majority of secondary fibres terminate in the ventral posterior nucleus of the thalamus, contacting third-order thalamocortical neurones that project to the somatosensory cortex.

Question 63

Describe the dorsal columns and their journey through the CNS.

Answer 63

The dorsal columns are split into two tracts, the fasciculus gracilis (medial) and the fasciculus cuneatus (lateral). The tracts carry impulse concerned with proprioception and discriminative touch. The dorsal columns contain axons of primary afferent neurones that have entered the cord through the dorsal roots. The fasciculus gracilis consits of fibres that join the cord at the sacral, lumbar and lower thoracic levels (lower limb) and the fasciculus cuneatus those from upper thoracic and cervical roots (upper limb). They travel on the ipsilateral side to the medulla oblongata where they terminate upon second-order neurones in the nucleus gracilis and cuneatus. Second-order accons then decusste in the medulla as internal arcuate fibres and thereafter ascend through the brain stema s the medial lemniscus to terminate in the ventral posterior nucleus of the thalamus. Third-order thalamocortical neurones in turn project to the somatosensory cortex located in the postcentral gyrus of the parietal lobe.

Question 64

Describe the corticospinal tracts and their journey through the CNS.

Answer 64

A descending spinal tract concerned with the control of voluntary, discrete, skilled movements, especially those of the distal parts of the limbs Primary neurones arise from motor and sensory parts of the cortex but the largest axons arise from betz cells in the premotor cortex in the precentral gyrus. Corticospinal axons leave the cerebral hemisphers by passing through the corona radiate and internal capsule to entre the crus cerebri of the midbrain. Having passed through the the ventral portion of the pons, fibres reach the medulla oblongata where they form the pyrmiadal tracts on the ventral surface. In the caudal medulla majority of fibres decussate and travel down to cord in lateral corticospinal tracts however some remain ipsilateral as the ventral corticospinal tract which decussate lower down the cord.

Question 65

Describe normal pressure hydrocephalus, its signs and symptoms, investigations, and treatment

Answer 65

Type of brain malfunction caused by decreased absorption of CSF typically presenting with the triad, ataxia, urinary incontinence and dementia. The dementia is predominantly frontal lobe in nature i.e. presents in the form of apathy, forgetfulness inertia, inattention. Investigations: - CT or MRI to exclude SOLs, shows enlarged ventricles - lumbar puncture and evaluation of response to CSF removal. Treatment: -Surgical implantation of a ventriculoperitoneal shunt if clinically fit for surgery.

Question 66

Describe stroke, its causes, risk factors, signs, and management

Answer 66

Strokes result from ischaemia infarction of bleeding into part of the brain, and manifest by rapid-onset of focal CNS signs and symptoms Causes: - Small vessel occlusion, cerebral microangiopathy of thrombosis insitu - Cardiac emboli e.g. AF, endocarditis, MI - Atherothromboembolism e.g. from carotids - CNS bleeds e.g. hypertension, trauma, aneurysm rupture, anticoagulation, thrombolysis. - sudden BP drop - carotid artery dissection - vasculitis Risk factors: hypertension, DM, heart disease, PVD, past TIA, carotid bruit, the pill, hyperlipidaemia, alcohol abuse, pro-thrombotic disorders Signs: sudden onset with progression over hours - Cerebral infarcts: the following criteria should be assessed 3 of the below defines a TACI (middle and anterior cerebral arteries) and 2 of the below defines a PACI (smaller arteries of anterior circulation) 1) Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg. 2) Homonymous hemianopia 3) high cognitive dysfunction e.g. Dysphasia. - Brainstem infarcts: quadriplegia, locked-in syndrome - Lacunar infarcts: in basal ganglia, thalamus, and pons. ataxic hemiparesis, pure motor, pure sensory, sensorimotor and dysarthria/clumsy hand. Management: - Protect airway to avoid hypoxia/aspiration - Pulse, BP, ECG - exclude AF, haemodynamically stable treat hypo not hypertension - BM - Urgent CT/MRI if thrombolysis considered, cerebellar stroke, unusual presentation, - Consider thrombolysis if haemorrhagic stroke excluded and onset of symptoms less than 4.5hrs ago - NBM keep hydrated assess speech and swallow - Antiplatelet agents once haemorrhagic stroke excluded, aspirin 300mg for 2 weeks mono therapy then after 2 weeks dual antiplatlets. - do not routinely anti-coagulate in ischaemic stroke due to risk of haemorrhagic transformation - refer to stroke unit asap - consider statin if hyperlipidaemia, controlling BP but not acutely unless thrombolysed in which case aim BP less than 180/110. In Haemorrhage aim BP less than 160/110. If no thrombolysis do not routinely treat BP unless over 220mmHg systolic. Seek Stroke nurse/specialist support.

Question 67

Describe Guillain-Barre syndrome, its presentation, triggers, and management

Answer 67

Also known as acute inflammatory demyelinating polyneuropathy. Presentation: Typically presents a few weeks after an infection with a symmetrical ascending muscle weakness. The trigger causes antibodies which attack nerves, though in 40% no cause is found. It may advance quickly, affecting all four limbs at once, and can lead to paralysis. There is a progressive phase of up to 4 weeks followed by recovery. Management: - lumbar puncture, CSF shows increased protein in absence of raised WCC. - FVC monitoring 4 hourly with transfer to ITU if respiratory involvement. - Cardiac monitoring required for risk of bradycardia or SVT - Ventilate sooner rather than later e.g. if FVC less than 1.5L or signs of respiratory failure - IV immunoglobulin 0.4g/kg/24h for 5d Triggers include campylobacter jejuni, CMV, mycoplasma, zoster, HIV, EBV, vaccinations.

Question 68

What is apraxia

Answer 68

A motor disorder cause by damage to the posterior parietal cortex leading to difficulty with motor planning to perform tasks or movements when asked.

Question 69

Describe Vaso-vagal syncope, typical history,

Answer 69

Due to reflex bradycardia +/- peripheral vasodilatation provoked by emotion, pain, fear or standing too long. Onset is over seconds (not instantaneous), and is often preceded by nausea, pallor, sweating and closing in of visual fields (pre-syncope). It cannot occur when lying down. LOC for around 2 mins. Brief clonic jerking of the limbs may occur but there is no stiffening or tonic/clonic sequence. Urinary incontinence is uncommon but may occur, and there is no tongue-biting. Recovery is rapid.

Question 70

Describe a circumducting gait and its causes

Answer 70

Patient stands with unilateral weakness on the affected side, arm held in flexion, adducted and internally rotated, leg extended on same side with plantar flexion of foot and toes. When walking the patient will hold his or her arm to onside and drag their affected leg in a semicircle (circumduction) due to weakness of distal muscles and extensor hypertonia. This is most commonly seen in stroke.

Question 71

describe a high-stepping gait and its causes

Answer 71

Seen in patients with foot drop (weakness of foot dorsiflexion), the cause of this gait is due to an attempt to lift the leg high enough during walking so that the foot does not drag on the floor. If unilateral, causes include peroneal nerve palsy and L5 radiculopathy. If bilateral, causes include amyotrophic lateral sclerosis, Charcot-Marie-Tooth disease and other peripheral neuropathies including diabetic neuropathy.

Question 72

Describe a myopathic (waddling) gait and its causes.

Answer 72

Hip girdles muscles are responsible for keeping the pelvis level when walking. If you have weakness on one side, this will lead to a drop in the pelvis on the contralateral side of the pelvis while-walking (Trendelenburg sign). with bilateral weakness, you will have drooping of the pelvis on both sides during walking leading to waddling This gait is seen in patients with myopathies such as muscular dystrophy.

Question 73

Describe the role of the hippocampus

Answer 73

Located in the medial temporal lobe of the brain, and part of the limbic system, the hippocampus plays important role in short-term memory, especially the conversion of short-term memory to long term, and spatial navigation

Question 74

What is allodynia and name some causes?

Answer 74

Refers to triggering of pain response from stimuli which do not normally provoke pain. Causes: Neuropathies, complex regional pain syndrome, fibromyalgia, migraine, spinal cord injury.

Question 75

Describe Migraine, it's symptoms, treatment, and prevention

Answer 75

A common cause of recurrent acute headaches. Symptoms: Diagnostic criteria requires at least 3 attacks, of which last 4-72hours duration, and have at least 2 of the following characteristics; Unilateral, Moderate pain, pulsating quality, avoidance of physical activity. They must also have at least one of the following symptoms N+V, photophobia, phonophobia. And finally must not be attributable to another disorder. Classically headaches are preceded by an aura lasting 15-20minutes with onset of headache within an hour. Aura: Precedes headache by minutes and may persist during. May be visual such as chaotic lines or dots, visual distortion or scotomata, or hemianopia. May also be somatosensory such as paraesthesiae spreading from fingers to face. Motor aura such as dysarthria, ataxia, opthamolplegia or hemiparesis. Treatment: - NSAIDS (e.g. Ketoprofen 100mg PO, or dispersive aspirin 900mg/6h) are good as there is reduced chance of developing medication-misuse headache. - Triptans such as rizatriptan may be used. They are CI if IHD, coronary spans, uncontrolled hypertension, recent lithium, SSRIs, or ergot use. SEs include arrhytmias and angina+/- MI - Ergots may also be used such as ergotamine 1mg PO can cause gangrene and vascular damage. CI in those taking pill, PVD, IHD, pregnancy, breastfeeding, hemiplegic migraine, raynauds. - Botulinum toxin type A injection may be used in chronic migraine headaches on more than 14days a month if no response to 3prophylactic drugs. Prevention: - Remove triggers I.e. CHOCOLATE, Hangovers, Orgasms, Cheese, Oral contraceptives, Lie-ins, Alcohol, Tumult, Exercise. - If frequent I.e. More than 2 a month 1st line is propanolol 40-120mg/12h, amitriptyline 10-75mg nocte, or topiramate 25-50mg/12h - 2nd line = valproate, pizotifen, gabapentin, pregabalin,

Question 76

Describe Meralgia Paraesthetica, its symptoms, and management.

Answer 76

An entrapment syndrome of the lateral femoral nerve. Symptoms: causes burning or numbness down the upper lateral aspect of the thigh. Pain can be reproduced by deep palpation just below the upper lateral aspect of the thigh. Management: - weight loss may cure the condition in the case of obesity - otherwise NSAIDs may be trialled

Question 77

What is cushings triad?

Answer 77

It is a sign of increased Intracranial pressure, it is a triad of Hypertension, bradycardia and irregular breathing

Question 78

Describe Cerebral Oedema, is warning signs, and management.

Answer 78

It is a big threat and is a complication of childhood DKA. Usually presents as sudden CNS deterioration after an initial improvement. Warning signs: headache, cushings triad (Bradycardia, hypertension, irregular breathing), restlessness, irritability, focal neurology such as CN palsies, papilloedema. Management: -Call your senior, exclude hypoglycaemia, mannitol 0.25-1.5g/Kg IVI, retrial IV maintenance fluids by 1/2 and replace deficit over 72 hours. Move to PICU and do CT.

Question 79

Describe Tuberous Sclerosis and some of its features.

Answer 79

Also known as bourneville's disease, it is a genetic condition of autosomal dominant inheritance. Like Neurofibromatosis the majority of features seen are neuro-cutaneous. Cutaneous features: - depigmented 'ash-leaf' spots which fluoresce under UV light - roughed patches of skin over lumbar spine (shagreen patches) - adenoma sebaceum (angiofibromas), butterfly distribution over nose - subungual fibromyalgia - cafe-au-lait spots may be seen Neurological features: - developmental delay - epilepsy, infantile spasms - intellectual impairment. Other features include, retinal haramtomas, rhabdomyomas of heart, polycystic kidney disease, renal angiomyolipomata, polyps, peutz-Jeghers, lymphagniomyomatotsis

Question 80

Describe Cerebral Palsy, it's presentation, causes, classification, and management

Answer 80

A chronic disorder of posture and movement caused by non-progressive CNS lesions sustained before 2yrs old, resulting in delayed motor development and evolving CNS signs, learning disability and epilepsy. Presentation: Abnormal tone in early infancy, delayed motor milestones, abnormal gait, feeding difficulties. Associated non-motor problems include learning difficulties, epilepsy, squinted and hearing impairment. Causes: Antenatal e.g. Cerebral malformation and congenital infection, intrapartum e.g. birth asphyxia/trauma, postnatal e.g. IVH, meningitis, trauma, Kernicterus. Classification: - spastic: hemiplegic, diplegic, quadriplegic - dyskinetic - ataxic - mixed Management: - MDT approach - treatments for spasticity include oral diazepam, intrathecal baclofen - anti-epileptics as required.

Question 81

Describe Multiple System Atrophy and its symptoms.

Answer 81

A rare neurodegenerative disorder caused by cell loss in certain areas of the brain and spinal cord. Characterised by widespread Glial cytoplasmic inclusions. Typically occurs between 50-70yrs. Symptoms: Parkinsonism, autonomic disturbance (atonic bladder, postural hypotension, constipation), cerebellar signs

Question 82

Describe Duchenne's Muscular Dystrophy, its symptoms, investigations and management.

Answer 82

Commonest of the muscular dystrophies, it is a genetic disease with progressive degeneration and weakness of specific muscle groups. It is an X-linked recessive mutation in the dystrophin gene. Patients tend to die due to cardiomyopathy and respiratory failure. Can affect females 10-20% developing full blown phenotype with some having cardiomyopathy only. Symptoms: Presents around 4yrs with clumsy walking and then difficulty in standing, and respiratory failure. Pseudo hypertrophy is seen especially in calves. Investigations: CK is raised 40-fold. Management: - no specific treatment - physio and occupational input - ventilation may be needed.

Question 83

Describe Multiple Sclerosis, the types, its symptoms, and management.

Answer 83

Chronic autoimmune demyelinating disorder. 3 times more common in women, most commonly diagnosed between 20-40. Types: - relapsing-remitting disease (most common 85%) acute attack (last 1-2mnths) followed by periods of remission. - secondary progressive disease, relapsing remitting patients who have deteriorated and have developed neurological signs between relapses. Gait and bladder disorders are generally seen. - primary progessive, deterioration from onset, more common in older people. Symptoms: usually monosymptomatic: Lethargy, unilateral optic neuritis (pain on eye movement and rapid loss of central vision), optic atrophy, ophthalmoplegia, pins/needles, numbness, Trigeminal neuralgia, spastic weakness, cerebellar ataxia, tremor, urinary incontinence, sexual dysfunction, intellectual deterioration. Management: - vitamin D status related to prevention of MS and fewer symptoms and lesions ensure levels above 50nmol/L - Acute relapse, high dose steroids may be given for 5 days to shorten the length of relapse (PO methylprednisolone has been shown to be non-inferior to IV methylprednisolone) - Beta-interferon reduce relapse rate by up to 30%, also dimethyl fumarate - other drugs include, galatiramir acetate, natalizumab, fingolimod

Question 84

Describe Myotonic Dystrophy, its types, and features.

Answer 84

AKA dystrophia myotonica, an inherited autosomal dominant, myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscles. Types: There are two main types DM1 and DM2. - DM1 present with more distal weakness caused by trinucleotide CTG repeat at the end of DMPK gene on chromosome 19 - DM2 is more proximal, caused by a tetranucleotide repeat expansion of the (CNBP) ZNF9 gene on chromosome 3. Symptoms: -DM1 (Distal weakness, Autosomal Dominant, Diabetes, Dysarthria) General Features: Myotonic fancies (long, haggard appearance), frontal balding, bilateral ptosis, cataracts, dysarthria, myotonia (tonic spasm of muscle), weakness of arms and legs, mild mental impairment, diabetes Mellitus, testicular atrophy, heart block, cardiomyopathy, dysphagia.

Question 85

Describe Trigeminal Neuralgia, its symptoms, and management.

Answer 85

Symptoms: Paroxysms of intense, stabbing pain, lasting seconds, in the Trigeminal nerve distribution. It is unilateral, typically affecting mandibular or maxillary divisions. The face screws up with pain. May be triggered by washing affected area, shaving, eating, talking, dental prostheses. Management: - MRI is required to exclude secondary causes e.g. Aneurysm, tumour, MS - Carbamazepine (100mg 1-2times a day), lamotrigine, phenytoin or gabapentin may be tried.

Question 86

Describe Syringomyelia, its symptoms, and management.

Answer 86

A condition in which there is a fluid-filled tubular cyst (syrinx) within the central usually cervical spinal cord. It can elongate and expand to affect the Brainstem (syringobulbia). Symptoms: Dissociated sensory loss ( absent pain and temperature, with preserved light touch, vibration and joint position sense) in a root distribution. Horner's syndrome, UMN leg signs. Body asymmetry, If extension into Brainstem nystagmus, tongue atrophy, dysphagia, palatal weakness, Vth sensory loss. Management: - MRI imaging - Neurosurgical review.

Question 87

Describe insominia, its causes, and management

Answer 87

Causes: - self limiting e.g. Jet lag, stress, shift work, old age - psychological e.g. Depression, anxiety, mania, grief, agitation/psychosis - Organic e.g. Drugs (caffeine, nicotine withdrawal), nocturia, pain, itch, tinnitus, sleep apnoea, restless leg syndrome (check ferritin). Management: - advise re sleep hygiene, no daytime naps, into turn in until feel sleepy, regular bedtime routines, keep a room for sleep do not work in it, less caffeine. - Hypnotic drugs for a few nights only, e.g. Zopiclone warn risk of addiction and tolerance. And rebound insomnia on withdrawal, no heavy machinery use or driving.

Question 88

What movements are the different myotomes responsible for?

Answer 88

Shoulder: Abduction = C5, Adduction = C5-C7 Elbow: Flexion = C5-C6, Extension = C7 Wrist: Flexion = C7-C8, Extension = C7 Fingers: Flexion = C8, Extension = C7, Abduction = T1 Hip: Flexion = L1-L2, Adduction = L2-L3, Extension = L5-S1 Knee: Flexion = L5-S1, Extension = L3-L4 Ankle: Dorsiflexion = L4, Eversion = L5-S1, Plantarflexion = S1-S2 Toe: Big toe extension = L5

Question 89

Describe Myasthenia Gravis, its features, investigations, and management.

Answer 89

An autoimmune disease mediated by antibodies to nicotinic acetylcholine receptors. More common in women under 50 or men over 50, associated with thyimic tumour/atrophy, RA and SLE. Features: Increasing muscular fatigue, typically affecting (in order) ocular, bulbar, face, neck, limb girdle, trunk. Look for ptosis, Diplopia, myasthenic snarl on smiling, 'peek sign' (fatigue on trying to keep eyes closed). Reflexes are normal. Investigations: Anti-AChR (90%) if negative look for Anti-MuSK (Muscle specific tyrosine kinase). CT of thymus. Neurophysiology if unsure. Management: - Weakness is made worse by pregnancy, hypokalaemia, infection, exercise, gentamicin, opiates, tetracycline, quinine, procainamide, beta-blockers. - Symptom control with anticholinesterase e.g. Pyridostigmine (SEs increased salivation, lacrimation, sweats, vomiting, miosis - Immunosuppression (Prednisolone, Azathioprine, cyiclosporin, mycophenolate) treat relapses with prednisolone, give osteoporosis prophylaxis, For treatment resistant Tacrolimus, cyyclophoshamide or rituximab, or periodic IVIg - in myasthenia crisis, monitor FVC, treat with plasmapheresis or IV Ig

Question 90

Describe Lambert-Eaton Myasthenic Syndrome, its features, and management.

Answer 90

Can be a paraneoplastic syndrome (from small cell lung cancer) or autoimmune. Caused by an antibody to pre-synaptic membranes voltage gated Ca-channels (Anti P/Q type VGCC) Features: Similar to MG but there is gait difficulty before eye signs, autonomy involvement (dry mouth, constipation, impotence), hyporeflexia and weakness, which improve after exercise. Management: - 3,4-diaminopyridine - IV Ig - regular CXR/ HRCT as symptoms may precede cancer by over 4 years.

Question 91

What are the causes of mixed UMN and LMN lesions

Answer 91

Fred's Tabby Cat Seeks Mice ``` Friedrich's Ataxia Tabes dorsalis (syphilis) Cervical spondylitis Subacute degeneration of the cord Motor neurone disease ```

Question 92

Describe Bulbar Palsy, its signs, and causes.

Answer 92

Bulbar palsy is a LMN lesion of cranial nerve IX, X and XII Signs: flaccid, fasiculating tongue, jaw jerk is normal or absent, speech is quiet, hoarse, or nasal, gag reflex is absent. Causes: MND, Syringobulbia, Guillian-Barre, poliomyelitis, central poninte myelinolysis, brainstem tumours, myasthenia gravis

Question 93

Describe pseudobulbar palsy, its signs and causes.

Answer 93

Pseudobulbar palsy is an UMN lesion of cranial nerves IX, X and XII. Signs: slow tongue movements, increased jaw jerk, increased gag reflex, Donald Duck speech, pseudobulbar affect causing weeping unprovoked or sudden giggling. Causes: - bilateral CVAs affecting internal capsule - MS - MND - High brainstorm tumours - Head injury

Question 94

What are the signs of an anterior communicating artery aneurysm?

Answer 94

Most common aneurysm of the circle of Willis can cause visual field defects such as bitemporal hemianopia due to compression of the optic chiasm, psyhycopathology and frontal lobe pathology.

Question 95

Describe Charcot-Marie-Tooth disease, its features, and management.

Answer 95

Hereditary motor and sensory neuropathy, most commonly inherited neurological disorder affecting approx 1/2500. Features: Foot drop, hammer toe, wasting of muscle of lower prt of legs (inverted champagne bottle), weakness in hands and forearms, loss of touch sensation in distal limbs. Pes cavus and pes planus are also seen. Management: - incurable - ankle-foot orthoses to help with foot drop

Question 96

Describe Mono-neuritis multiplex and its causes

Answer 96

A term used for neuropathies if two or more peripheral nerves are affected. ``` Causes: WARDS PLC Wegner's granulomatosis (Granulomatosis with Polyangitis) Amyloidosis/ AIDS Rheumatoid Arthritis Diabetes Mellius Sarcoidosis Polyarteritis Nodosa Leprosy Carcinomatosis ```

Question 97

What are the causes of a motor peripheral neuropathy?

Answer 97

Guillian Barre, Lead poisoning Charcot-Marie-tooth Chronic Inflammatory Demyelinating Polyneuropathy

Question 98

Describe Huntington's disease, its features and management.

Answer 98

Autosomal dominant, progressive neurodegenerative disorder presenting in middle age. Due to expansion of CAG repeat on chromosome 4. 10% are new mutations. Features: Often prodroml pahse of mild symptoms (irritability, depression, incoordination), progresses to chorea (random, jerky uncontrollable movements), dementia, seizures and death. Management: Dopamine antagonists to control chorea No treatment prevents progression Counselling for families and patient, anticipation (apparent at earlier age with successive generations)

Question 99

Describe FascioScapuloHumeral muscular Dystrophy and its features.

Answer 99

Almost as common as duchenne's. Autosomal dominant inheritance onset is around 12-14yrs. Around 20% require a wheelchair by 40yrs. Features: inability to puff out cheeks, difficulty raising arms above head, weakness of face, shoulder and upper arms, bilateral foot drop, scapular winging

Question 100

What are the causes of a sensorimotor peripheral neuropathy?

Answer 100

``` Alcohol B12/folate deficiency CKD Diabetes Everything else e.g. Vasculitis, HIV, trace elements (e.g. zinc, copper, chromium deficiencies) ```

Question 101

Describe Intranuclear opthalmoplegia, its features and causes.

Answer 101

Causes by a lesion in the medial longitudinal fasciculus. Features: When patients gaze is directed away from the side of the lesion, the ipsilateral (adducting) eye will not adduct and the contralateral (abducting) eye demonstrates horizontal nystagmus. Patients usually do not complain of diplopia, and when the eyes are tested independently medial rectus function is shown to still be present. Causes: - MS (likely cause in adulthood/middle age often bilateral) - Vascular brainstem lesion (likely in elderly or with vascular RFs) - Pontine glioma (likely cause in children) - Inflammatory encephalitis

Question 102

What are some causes of bilateral facial nerve palsy?

Answer 102

Sarcoidosis Vasculitis Lyme disease Guillian-Barre

Question 103

Describe Miller-fisher syndrome and its features

Answer 103

A variant of Guilliam barre that present in a descending fashion, associated with Anti-GQ1B. Features: classical triad of opthalmoplegia, ataxia, areflexia

Question 104

Describe Lumbar Canal Stenosis and its features.

Answer 104

Compression of the lumbar spinal cord due to stenosis. Features: Low back pain and leg weakness that is relived when bending forward, walking uphill or stairs, or cycling.

Question 105

Describe Weber's Syndrome and its features

Answer 105

Midbrain stroke syndrome, (Brainstem strokes have crossed finding with motor or sensory finding on the contralateral side of the body and cranial nerve findings ipsilateral) caused by occlusion of the paramedian branches of the basilar artery Features: Ipsilateral surgical IIIrd nerve palsy, and contralateral hemiplegia or hemiparesis

Question 106

Describe Von Hippel-Lindau syndrome and its features.

Answer 106

Autosominal dominant condition due to mutations in the VHL gene on chromosome 3. Results in the development of haemangioblastomas, which are CNS tumours that originate form the vascular system. Can also develop renal cell carcinoma and cysts in the liver, kidney and pancreas. Features: Cerebellar syndrome, Ectopic EPO production (polycythaemia), phaeochromocytoma (flushing, headache, palpitations)

Question 107

Describe Motor Neuron Disease, the types, features, and managment.

Answer 107

Cluster of major degenerative disease characterised by selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells. UMN + LMN with no sensory loss never affecting eye movements. Types + Features: - Amyotrophic lateral sclerosis: affects motor cortex and anterior horn cells. weakness, UMN + LMN signs worse prognosis if bulbar onset, increased age, decreased FVC - progressive bulbar palsy: only affect cranial nerves IX-XII - progressive muscular atrophy: anterior horn cell only thus no UMN signs, affects distal muscle groups before proximal. - Primary lateral sclerosis: los of betz cells in motor cortex, thus mainly UMN signs, spastic leg weakness and pseudobulbar palsy Management: - MDT approach, neurologist, palliative nurse, hospice, physio, speech therapist, OT, dietician, social services. - Treat symptomatically - Riluzole prevents stimulation of glutamate receptors used mainly in amyotrophic lateral sclerosis and prolongs life by around 3 months.

Question 108

Describe Post-lumbar puncture headache, its features, factors which contribute to headache, and management.

Answer 108

Features: - usually develops within 24-48hr following LP but may occur up to one week later - may last several days - worsens with upright position - improves with recumbent position Aggravating Factors: - Increased needle size - Direction of bevel - not replacing the stylet - Increased number of LP attempts Management: - Supportive, rest and analgesia - if pain continues more than 72 hours then treatment is needed to prevent subdural haematoma, treatment options include, blood patch (A surgical procedure that uses autologous blood in order to close one or many holes in the dura mater), epidural saline, and intravenous caffeine.

Question 109

Describe Transient Global Amnesia

Answer 109

Present widths Transient loss of memory function Patients may appear anxious and repeatedly ask the same question Patients have no recall of events after the attack Aetiology is unknown thought to be due to Transient ischaemia to the thalamus in particular the amygdala and hippocampus.

Question 110

What are the neurological features of a Parietal Lobe lesion?

Answer 110

Parietal Lesions depend on whether the lesion is dominant (Left), Non-dominant (Right) or bilteral: Unilateral lesions: (right or left) may cause: - Contralateral hemisensory loss and sensory inattention - Inferior homonymous quadrantopia - Abolotion of optokineticnystagmus. Unilateral dominant (usually left) may cause: - Apraxia (Difficulty with motor planning to perform tasks) - Astereognosis aka Tactile agnosia (the inability to identify an object by active touch of the hands) - Gerstmann's Syndrome (Lesion of dominant parietal): alexia (Difficulity reading) , acalculia (difficulty performing calculations), finger agnosia (difficulty recognising the fingers), right-left disorientation. Unilateral non-dominant (usually right) may cause: - Predominantly visuospatial dysfunction - Constructional apraxia (Ability to copy a drawing) - Anosognosia and dressing apraxia.

Question 111

What are the neurological features of a Occipital lobe lesion?

Answer 111

Homonymous hemianopia (with macula sparing) Cortical Blindness Visual Agnosia

Question 112

What are the neurological features of a Temporal Lobe lesion?

Answer 112

Wernicke's Aphasia (Receptive aphasia) Superior Homonymous Quadrantopia Auditory Agnosia Prosopagnosia (difficulty in recognising faces)

Question 113

What are the neurological features of a Frontal Lobe lesion?

Answer 113

``` Broca's Aphasia (Expressive aphasia) Disinhibition Perseveration Anosmia Inability to generate a list ```

Question 114

What are the neurological features of a cerebellum lesion?

Answer 114

Midline lesions causes gait and truncal ataxia | Hemisphere lesions causes intention tremor, past-pointing, dysdiadokokinesis and nystagmus

Question 115

Describe Korsakoff's syndrome and its features.

Answer 115

Marked memory disorder often seen in alcoholics, thiamine deficiency causes damage and haemorrhage to the mammillary bodies of the hypothalamus and the medial thalamus. It often follows on from untreated Wernicke's encephalopathy Features: - anterograde amnesia (inability to acquire new memories) - Retrograde amnesia - confabulation

Question 116

Describe Reye's syndrome and its features.

Answer 116

Reye's syndrome is a severe progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys and pancreas. There is a known association with aspirin use and a viral cause has been postulated. Peak incidence is 2 years Features: - May be history of preceding viral illness - Encephalopathy: confusion, seizures, cerebral oedema, coma - Fatty infiltration of liver, kidneys and pancreas - hypoglycaemia

Question 117

Describe Friedrich's ataxia and its features.

Answer 117

Most common of the early-onset hereditary ataxias. Autosomal Recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9. Unusual in trinucleotide repeat disorders in not demonstrating anticipation. Typical age of onset is 10-15yrs. Gait ataxia and kyphoscoliosis are most common presenting features: Neurological features: - Absent ankle jerks/extensor plantars - Cerebellar ataxia - Optic atrophy - Spinocerebellar tract degeneration - Decreased vibration and proprioception, pyramidal weakness Other features: - HOCM (90%, most common cause of death) - Diabetes Mellitus (10-20%) - High-arched palate - deafness

Question 118

Describe Cervicial Spondylosis, its features, and management.

Answer 118

Aka Degenerative cervical myelopathy. Features: - Pain (affecting the neck or upper or lower limbs) - Loss of motor function (loss of digital dexterity, arm of leg weakness/stiffness) - loss of sensory function causing numbness - loss of autonomic function (urinary or faecal incotinence or impotence) Management: - MRI of cervical spine is gold standard test. - Urgent Referral for to neurosurgery or orthopaedic spinal surgeons - decompressive surgery is only effective treatment.

Question 119

What are Dawson Fingers?

Answer 119

A radio graphic feature depicting demyelinating plaques through the corpus callosum arranged at right angles along medullary veins (Callososepal location). They are a relatively specific sign for Multiple Sclerosis which presents at T2 Hyperintensities.

Question 120

Describe Kluver-Bucy syndrome and its features.

Answer 120

A rare neurological condition caused by a brain lesion in the Amygdala. Features: Hypersexuality, Hyperorality, hyperphagia, visual agnosia.

Question 121

Describe Osmotic demyelination syndrome and its features.

Answer 121

Aka Central pontine myelinolysis, a demyelination Syndrome caused by the rapid correction of chronic hyponatraemia. Features: Quadriparesis and bulbar palsy, seizures, confusion, movement disorders. Diagnosis with MRI Brain which can show Tripod sign.

Question 122

Describe Intracranial Hypotension, its features and causes.

Answer 122

Aka Craniospinal hypotension, defined as CSF pressure less than 7cmH20. Features: Postural headache better when lying flat, nausea + vomiting, neck pain, visual and hearing disturbances, vertigo. Causes: - Primary - Spontaneous intracranial hypotension - secondary: iatrogenic (lumbar puncture or surgery), over shunt due to diversion devices, traumatic.

Question 123

Describe Marchiafava-bignami disease and its features.

Answer 123

Mostly seen in chronic alcoholics it is attributed to deficiency’s in all eight types of vitamin B leading to neurosis and demyelination of the corpus callosum Features: Non specific e.g. motor or cognitive disturbances, hemispheric disconnection syndrome or seizures. Radiologically is classically involves the central layers of the corpus callosum with relative sparing of the dorsal and ventral extremes which may be seen as a sandwhich sign on Sagittal MRI.

Question 124

Describe Copper deficiency and its causes and its features

Answer 124

A rare haematological and neurological disorders. May manifest in parallel with vitamin B12 and other nutritional deficiencies. Copper is involved in normalised function of many enzymes such as cytochrome C oxidase a complex in mitochondrial electron transport chain (catalyses reactions for oxidative phosphorylation), Ceruloplasmin (catalyses reactions for iron transportation), Cu/Zn Superoxide dismutase (catalyses reactions for antioxidant and free radical scavenging and neutralisation) and amine oxidises (catalyses neurotransmitter synthesis) Causes: - most common cause is malabsorption e.g. gastric bypass surgery - Zinc toxicity (zinc found in denture cream) - Menkes Disease genetic disorder of copper deficiency Features: - Haematolgical manifestations e.g. myelodysplasia, anaemia, leukopenia, neutropenia. - Neurological manifestations e.g. sensory ataxia, spasticity, muscle weakness, optic neuropathy, myelopathy. - Bone marrow aspirate shows dysplasia, ring sideroblasts (Features shared with myelodysplastic syndrome) uniquely shows cytoplasmic vacuoles within red and white cell precursors.

Question 125

Describe Balint's Syndrome and its features

Answer 125

An uncommon triad of severe neuropsychological impairments: - Simultanagnosia (Inability to perceive the visual fields as a whole) - Oculomotor ataxia (difficulty fixating the eyes) - Optic ataxia (inability to move the hand to a specific object by using vision)

Question 126

Describe Opsoclonus Myoclonus Syndrome, its features

Answer 126

Rare neurological disorder, thought to be autoimmune process. Affects 2-3% of children with neuroblastoma (accounts for 50% of cases) and has been reported to occur with coeliac disease. May be associated with Anti-Ri. Features: -Opsoclonus (Rapid, involuntary, multivectorial, unpredictable conjugate fast eye movements without intersaccadic intervals. -Myoclonus (brief, involuntary twitching of a muscle or a group of muscles) -cerebellar ataxia, both truncal and appendicular -Aphasia (impairment of speech and comprehension of speech) -Mutism -Lethargy

Question 127

Describe Isaac’s Syndrome (Neuromyotonia), its causes and features.

Answer 127

A form of peripheral nerves hyperexcitability that causes spontaneous muscular activity resulting from resistive motor unit action potentials of peripheral origin. Symptoms are most prominent in the Calves, legs, trunk, and soft ice the face and neck. Causes: - Acquired (Most common), typically caused by antibodies that bind to potassium channels on the motor nerve. - Paraneoplastic - Hereditary Features: -Muscle cramps, stiffness, myotonia-like symptoms (slow relaxation), associated walking difficulties, hyperhidrosis (excessive sweating), myokymia (quivering of a Muscle), fasciculations, fatigue, exercise intolerance, Myoclonus jerks.

Question 128

What is Hoffmann’s reflex?

Answer 128

Elected by a reflex test which can help verify the presence of absence of issues arising from the corticospinal tract. Also known as the finger flexor reflex. The test involves loosely trapping the middle finger at the distal inter-phalangeal joint and then scratch down the terminal phalanx nail plate allowing the phalanx to flick up naturally. A positive response is seen when there is flexion of the terminal phalanx of the thumb on the same appendage. Sign of UMN lesion (can be normal) e.g. MS, Stroke, Cervical myelopathy, cord compression

Question 129

Describe Herpes Simplex encephalitis, its features, investigations, and management.

Answer 129

HSV encephalitis characteristically affects the temporal an inferior frontal lobes. HSV-1 is responsible for 95% of cases. Features: Fever, headache, psychiatric symptoms, seizures, vomiting, focal features e.g. aphasia, peripheral lesions have no relation to presence of HSV encephalitis. Investigations: - CSF: Lymphocytosis, elevated protein - PCR for HSV - CT: Medial temporal and inferior frontal changes (e.g. petechial haemorrhages) normal in 1/3rd of patients. - MRI is betters - EEG Pattern: Lateralised periodic discharges at 2 Hz Treatment: Intravenous aciclovir

Question 130

Describe Creutzfeldt-Jakob disease, its features, investigation.

Answer 130

CJD is a rapidly progressive neurological condition caused by prion proteins. These proteins induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to pro teases. Features: Rapid onset dementia, myoclonus. Types: - Sporadic CJD accounts for 85% of cases, 10-15% are familial, mean age of onset is 65yrs. - New Variant CJD occurs in younger patients (average onset 25yrs) psychological symptoms such as anxiety, withdrawal, and dysphonia are most common presenting features. The prion protein is encoded on chromosome 20. Methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD. Investigations: - CSF is usually normal - EEG shows biphasic, high amplitude sharp waves (only in sporadic CJD) - MRI shows hyper intense signals in the basal ganglia (Hockey stick sign) and thalamus (Pulvinar sign). - Real-time Quaking Induced Conversion (RTQuIC) is a new sensitive and specific test for detecting protein 14.3.3 in sporadic CJD

Question 131

Describe Chorea and its causes

Answer 131

Chorea describe 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: -Hungtindon’s disease, Wilson’s disease, Ataxic telangiectasia -SLE, anti-phospholipid syndrome -Rheumatic fever: Sydenham’s chorea -Drugs e.g. oral contraceptive pill, L-dopa, antipsychotics -Neuroacanthocytosis -Pregnancy: Chorea gravidarum -Thyrotoxicosis Polycythaemia Rubra Vera -Carbon monoxide poisoning -cerebrovascular disease ```

Question 132

What is Parkinsonian Malignant Syndrome?

Answer 132

A life-threatening condition that occasionally occurs in patients with abrupt cessation of anti-parkinsonian medications. It is a cause of neuroleptic malignant syndrome

Question 133

Describe the Reticular Activating System, its structure, and function

Answer 133

The ascending reticular activating system is a set of connected nuclei in the brains of vertebrates that is responsible for regulating wakefulness and sleep-wake transitions. It is composed of several neuronal circuits connecting the dorsal part of the posterior midbrain and anterior pons to the cerebral cortex via distinct pathways through the thalamus and hypothalamus.

Question 134

Describe Froin's Syndrome and its causes.

Answer 134

Co-existance of xanthocrhomia, high proteins level, and marked coagulation of CSF. Stagnation of the CSF within the thecae sac facilities exudation from the tumour itself and activation of coagulation factors. Causes: - meningeal irritation e.g. Spinal meningitis - CSF flow blockage e.g. tumour mass or abscess

Question 135

Describe Acute Disseminated Encephalomyelitis

Answer 135

ADEM is an autoimmune Demyelinating disease of the central nervous system. It may also be termed post-infectious encephalomyelitis. Common infections include measles, mumps, rubella, varicella and small pox. After a lag time of between a few days to 2 months there is an acute onset of multifocal neurological symptoms with rapid deterioration. Non specific signs such as headache, fever, nausea and vomiting may also accompany the onset of illness. Motor and sensory deficits are frequent and there may also be brainstem involvement including occulomotor defects. There are no specific bio markers for the diagnosis of ADEM. MRI imaging may show areas of supra and infra-tectorial demyelination. Management involves IV Glucocorticoids and the consideration of IVIG

Question 136

Describe Progressive Supranuclear Palsy and its features

Answer 136

AKA Steele-Richardson-Olszewski syndrome. A 'Parkinson-plus' syndrome. Features: - Impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficulty reading or descending stairs) - Parkinsonism - Falls - Slurring of speech, pseudobulbar palsy - Cognitive impairment - subcortical dementia - Axial rigidity - Signe d’applause - ask patient to clap hands three times they proceed to clap more than 3

Question 137

Describe Limbic (Autoimmune) Encephalitis, it types, features,

Answer 137

A form of encephalitis caused by autoimmunity it may be paraneoplastic or non-paraneoplastic (e.g. secondary to infection, autoimmune disorder, idiopathic). It is often classified according to the auto-antibody which causes the disease: - Anti-Hu (associated with small-cell lung carcinoma) - Anti-Ma2 (Associated with germ-cell tumours of the testis) - Anti-NMDAR (associated with tumour of the ovaries, commonly teratomas) - Anti-VGKC(LGI1, CASPR2, Contactin-2) (Not associated with tumours) Features: - Subacute short-term memory deficit - Headache, irritability, sleep disturbance, delusions, seizures (Fascio-brachial dystonic seziures (characteristic of LGI1 autoantibodies)) Investigations: - CSF Analysis - Elevated Protein, Normal Glucose, Elevated Lymphocytes (But usually less than 100). - Auto-antibodies - MRI Brain - Increased T2 signal in one or both temporal lobes. Management: - IVIG - Plasmapheresis - Corticosteroids, cyclophosphamide, rituximab.

Question 138

What are the different Prion diseases?

Answer 138

Human Prion Diseases: - Creutzfeldt-Jakob disease - Variant Creutzfeldt-jakob disease - Gerstmann-straussler-Scheinker Syndrome - Fatal Familial Insomnia - Kuru Animal Prion Diseases: - Bovine spongiform Encephalopathy - Chronic Wasting Disease - Scrapie - Tranmissible Mink Encephalopathy - Feline spongiform encephalopathy - Ungulate spongiform encephalopathy

Question 139

Describe Refsum disease and its features

Answer 139

aka Phytanic acid deficiency, an autosomal recessive neurological disease that results in the over accumulation of phytanic acid in cells and tissues. Features: cerebellar ataxia, peripheral neuropathy, Retinitis Pigmentosa, sensorineural hearing loss, ichthyosis

Question 140

Describe Hiriyama disease and its features

Answer 140

aka Monomyelic Amyotrophy or Non-progressive Juvenile Spinal Muscular Atrophy. An untreatable focal motor neurone disease that primarily affects young males in India and Japan. A type of cervical myelopathy Features: Insidious onset muscular atrophy usually of the forearms and hands, which stabilises at a plateau after 2-5 years with no progression. There is no pain or sensory loss. Sparing of the brachioradialis

Question 141

Describe Kennedy's Diseaseand its features

Answer 141

Aka Spinal and Bulbar Muscular Atrophy, a progressive neurodegenerative disease resulting in muscles cramps and progressive weakness due to degeneration of motor neurone in the brainstem and spinal cord. Associated with Mutation of Androgen Receptor Gene and is inherited in X-linked recessive fashion, caused by a CAG trinucleotide repeat in the first exon of the gene. Features: (Lower motor neurones signs) Muscle cramps, progressive weakness. Early signs include weakness of the tongue and mouth muscles fasciculations and gradually increasing weakness of the limb muscles with wasting.

Question 142

Describe Trigeminal Autonomic Cephalgia

Answer 142

A group of primary headache disorders characterised by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features. The group comprises of: - Cluster Headache - Paroxysmal Hemicrania - Hemicrania Continua - Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT syndrome).

Question 143

What are the different pathologies and causes of peripheral neuropathy?

Answer 143

Demyelinating pathology: - GBS - CIDP - Amiodarone - Hereditary sensorimotor neuropathies (HSMN) Type I - Paraprotein neuropathy Axonal Pathology: - Alcohol - Diabetes Mellitus (May also be demyelinating) - Vasculitis - B12 Deficiency (may also be demyelinating) - Hereditary sensorimotor neuropathies (HSMN) type II

Question 144

Describe Neuromyelitis Optica, its features, investigations

Answer 144

aka Devic's disease is monophonic or releasing-remitting demyelinating CNS disorder. Although previously thought to be a variant of multiple sclerosis it is now recognised to be a distinct disease, particularly prevalent in asian populations. It typically involves the optic nerves and cervical spine, with imaging of the brain frequently normal (A distinguishing feature between MS). Vomiting is also the most common presenting complaint. Diagnosis requires bilateral optic neuritis, myelitis and 2 of the following 3 criteria: - Spinal cord lesion involving 3 or more spinal levels - Initially normal MRI Brain - Aquaporin 4 +Ve serum antibody

Question 145

Describe Dural Ectasia, its symptoms, and causes.

Answer 145

Widening or ballooning of the dural sac surrounding the spinal cord. Usually occurs in the lumbosacral region as this is where the cerebrospinal fluid pressure is greatest. Features: - Lower back pain - Headaches - weakness and numbness above and below the involved limb. - Incontinence - symptoms are exacerbated by upright posture and relieved by lying down. Causes: - Marfans syndrome - Ehlers-danlos syndrome - Neurofibromatosis type 1 - Ankylosing spondylitis - trauma

Question 146

Describe autonomic dyreflexia, its symptoms, and management

Answer 146

A medical emergency, most often occurs in individuals with spinal cord injuries with spinal lesions above the T6 level (although has been known to occur in patients with a lesion as low as T10. thought to be triggered by afferent stimuli below the level of the lesion. Acute AD is a reaction of the autonomic nervous system to overstimulation. It is characterised by paroxysmal hypertension, associated with throbbing headaches, profuse sweating, nasal stuffiness, flushing of the skin above the lesion, bradycardia, confusion. Management: - Catheterisation, or relief of blocked catheter - Manual Rectal evacuation - GTN Spray or oral clonadine if precipiting trigger cannot be found.

Question 147

Which cranial nerves are sensory, motor or both?

Answer 147

Some Say Marry Money But My Brother Says Big Brains Matter Most ``` I - Sensory II - Sensory III - Motor IV - Motor V - Both VI - Motor VII - Both VIII - Sensory IX - Both X - Both XI - Motor XII - Motor ```

Question 148

Describe Mediation Overuse Headache, its features, and management

Answer 148

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

Question 149

What is Bruns syndrome?

Answer 149

A sudden onset of severe headaches and vomiting associated to a vestibular syndrome provoked by abrupt change in head position. It is related to an episodic obstructive hydrocephalus caused by an intraventricular mass that act like a ball-valve mechanism

Question 150

What is Cerebellar Ataxia with neuropathy and bilateral vestibular areflexia (CANVAS) syndrome?

Answer 150

An adult onset slowly progressive neurologic disorder comprised of Cerebellar Ataxia, Neuropathy, and vestibular areflexia. Features: - Characteristic sign of impaired visually enhanced vestibule-ocular reflex ( a impairment of three compensatory eye movement reflects, vestibule-ocular reflex (Opposing movement of the eyes to movement on the head in order to keep focussed imaged centred), smooth pursuit (smooth movement of eyes when following an object, as opposed to saccadic) and optokinetic reflex (when following an object and it move out of the line of vision the eyes centre back on the point at which the object first appeared) - Non length-dependant neuropathy

Question 151

Describe Narcolepsy and its features.

Answer 151

Associated with HLA-DR2, it is associated with low levels of orexin (hypocretin), a protein which is responsible for controlling appetite and sleep patterns. Early onset of REM sleep. Features: Typical onset in teenage years, hypersomnolence, cataplexy (sudden loss of muscle tone often triggered by emotion), sleep paralysis, vivid hallucinations on going to sleep or waking up. Investigations: -Multiple sleep latency EEG.

Question 152

Describe Parinaud's Syndrome and its features

Answer 152

aka dorsal midbrain syndrome, vertical gaze palsy, and sunset sign. It is an inability to move the eyes up and down. It is caused by compression of the vertical gaze centre at the rostral interstitial nucleus of the medial longitudinal fasiculus. Features: - Paralysis of upwards gaze (vertical palsy is supranuclear, so Doll's head maneucer should elevate the eyes, but eventually all upward gaze mechanism fail - Pseudo-argyll robertson pupils, accomodative paresis ensures, and pupils becomes mid-dilated and show high-near dissociation. - convergence-retractino nystagmus (on fast up gaze the eyes pull in and the globes retract) - eyelid retraction (collier's sign)

Question 153

Describe Malignant MCA syndrome

Answer 153

A condition whereby blood supply from MCA is restricted leading to a widespread infarct. This leads to cerebral oedema which causes raised ICP and may lead to herniation and/or brainstem death. Mannitol or hypertonic saline is used to manage, emergency hemicraniotomy may be needed.

Question 154

Describe Corticobasal Degeneration syndrome, and its features

Answer 154

Rare progressive neurodegenerative disease involving the cerebral cortex and the basal ganglia. It is classified as one of the Parkinson Plus syndromes. Also associated with Frontotemporal dementia and Tau pathology. Features: Parkinsonism (asymmetric), Alien hand syndrome a characteristic feature wear a limb appears to move on its own, Apraxia, or dystopia, Progressive non-fluent Aphasia, Myoclonus

Question 155

Describe the management of neuropathic pain.

Answer 155

- First line treatment: amitriptyline, duloxetine, gabapentin or pregabalin - If the first line drug foes not work try one of the other three. - Tramadol may be used for breakthrough pain - Topical Capsaicin may be used for localised neuropathic pain.

Question 156

Describe Cubital Tunnel Syndrome

Answer 156

Due to the compression of the ulnar nerve Features: - Intially intermittent tingling in the 4th and 5th finger - may be worse when the elbow is resting on a firm surface or flexed for extended periods. - Later numbers in the 4th and 5th finger with associated weakness

Question 157

Describe Radial tunnel syndrome

Answer 157

Most commonly due to compression of the posterior interosseous branch of the radial nerve. It is though to be a result of overuse. Features: - Symptoms are similar to lateral epicondylitis making it difficult to diagnose. - However, the pain tends to be around 4-5cm distal to the lateral epicondyle - Symptoms may be worsened by wanted the elbow and pronating the forearm.

Question 158

Describe Brown-Séquard syndrome, its features, and causes.

Answer 158

Caused by damage to one hand of the spinal cord. Features: - Ipsilateral loss of proprioception - Contralateral loss of pain + temperature - Ipsilateral limb paralysis Causes: - Spinal cord tumour (Menigioma) - Trauma (most common) - Ischaemia - Infections e.g. TB - Inflammation e.g. MS - Decompression sickness.

Question 159

What are the different features of axonal vs demyelinating neuropathy in nerve conduction studies?

Answer 159

Axonal: Decreased Amplitude but normal velocity Demyelinating: Normal Amplitude but decreased velocity

Question 160

Describe Cavernous Sinus thrombosis, its features, and management.

Answer 160

Usually caused by spreading infection in the nose, sinuses, ears or teeth, most commonly Staph Aureus and Strep. Features: Visual disturbance, chemosis (Conjunctival swelling), exophthalmos, headaches, cranial nerve palsy III-VI (VI most common). Management: - MR Venogram most sensitive imaging - Broad-spectrum antibiotics - Anticoagulation - Surgery may be indicated if neurological deterioration.

Question 161

Describe Chronic Paroxysmal Hemicrania, and its features

Answer 161

Aka Sjaastad syndrome, it falls under the Trigeminal Autonomic Cephalgia category of headache. Features: Debilitating unilateral headache affecting the area around the eye. Usually consists of multiple (often more than 5/day) severe, yet short (5-30mins), headache attacks affecting only one side of the cranium. No neurological features (unlike migraine), may be associated with redness and watering of the eye but no nausea or vomiting. Management: - NSAIDs especially Indomethacin. (Thought that if not responsive to indomethacin is unlikely correct diagnosis) - Topiramate may have a role. OR ?Lamotrigine.

Question 162

Describe Tolosa-Hunt syndrome, its features, and management,

Answer 162

A rare disorder characterised by severe and unilateral headaches with orbital pain, along with weakness and paralysis of certain eye muscles. Thought to be due to inflammation of the areas behind the eyes (Cavernous sinus and superior orbital fissure). Diagnosis of exclusion. Features: Usually unilateral intense sharp pain and paralysis of muscles around the eye. Other features include, double vision, fever, fatigue, vertigo, arthralgia. Management: - Corticosteroids - Steroid-sparing immunosuppressants.

Question 163

Describe Transverse myelitis and its features

Answer 163

A rare neurological condition in which the spinal cord is inflamed. Features: Weakness and numbers of the limbs, deficits in sensations and motor skills. Autonomic dysfunction. Exact symptoms variable and reflect the level of the affected spinal cord. Typically develop over the course of hours or days. Often associated with a sensory level.

Question 164

What are the features of a posterior communicating artery aneurysm?

Answer 164

Most common of all intracranial aneurysms presents with ipsilateral third nerve palsy up to 60% of the time.

Question 165

Categorise Antiepileptics drugs according to their enzyme inducing activity

Answer 165

Enzyme Inducing AEDs: - Carbamazepine - Phenobarbital - Phenytoin - Topiramate Non-Enzyme Inducing AEDs: - Acetazolamide - Ethosuximide - Levetiracetam - Sodium Valproate Lamotrigine is non-enzyme inducing but there is evidence that OCP lowers its levels.

Question 166

Describe Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) and its features

Answer 166

Most common form of hereditary stroke disorder thought to be due to mutations of the Notch 3 gene on chromosome 19 Features: Recurrent migraines, TIAs or strokes, usually occurring between 40 -50yrs.

Question 167

Describe Wolfram (DIDMOAD) syndrome and its features

Answer 167

Rare Autosomal-Recessive disorder. Two genetic forms WFS1 + WFS2 (Dysfunction of CISD2 gene) Feature: Childhood onset Diabetes Insipidus Diabetes Mellitus, Optic Atrophy and Deafness

Question 168

Describe Paraneoplastic Cerebellar Degeneration and its features.

Answer 168

A paraneoplastic syndrome associated with lung, ovarian, breast, Hodgkins lymphoma.. It is believed to be due to a autoimmune reaction target Purkinje cells. Thought to be triggered when tumour cells express proteins normally expressed in the cerebellum. Features: Dysarthria, truncal,limb and gait ataxia, and nystagmus. Symptoms develop sub acutely and progress rapidly over a period over weeks or months to a plateau period which could last for months to years.. May involve anti-purkinje cell or anti-Yo antibodies.

Question 169

Describe Chiari Malformations and its features.

Answer 169

A structural defect in the cerebellum characterised by a downward displacement of one of both or the cerebellar tonsils through the foramen magnum. There are four types: -Type 1 is the most commonly observed in children, in this type the lower part of the cerebellum but not brainstem extends into the foramen magnum. It is also the only type that can be acquired -type 2 is usually only even in children with spinal bifida it also known as classic or Arnold Chiari malformation, Involves both the cerebellum and brain stem extending into the foramen magnum. - Type 3 is the most serious form and involves protrusion or herniation of cerebellum and brain stem through foramen magnum - Type 4 involves an underdeveloped cerebellum. Features: Headache, Dysphagia, Dizziness, Vomiting, Neck pain, Ataxia, Paraesthesia. Downbeat nystagmus

Question 170

What is MELAS syndrome?

Answer 170

Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS), a mitochondrial cytopathy (A group also included MERRF and Leber's Hereditary optic neuropathy).

Question 171

Describe Reflex sympathetic dystrophy, its symptoms, and management.

Answer 171

Aka Complex regional pain syndrome type 1, a disorder which manifests as extreme disproportional pain which occurs after an illness or injury thought to be due to alteration of pain perception and inflammatory molecules released from peripheral nerves leading to inappropriate cross talk between sensory and motor fibres. Features: Pain, swelling, limited range of motion, and changes to skin and bones. Management: - Physical and occupational therapy. - Psychological treatments - Neuromodulation (mirror box therapy, graded motor imagery) - Bisphosphonates, calcitonin, ketamine.

Question 172

What are the distinguishing features of Botulinsm that helps differentiate it from myasthenia gravis?

Answer 172

There is autonomic involvement in botulism e.g. mydriatic eyes and dry mouth.

Question 173

Describe Orbital apex syndrome, its features and causes.

Answer 173

aka Jacod syndrome, a collection of cranial nerve deficits associated with a mass lesions near the apex of the orbit of the eye. Most commonly optic nerve is involved. Features: Most common finding is oculomotor nerve dysfunction leading to opthalmoplegia. Often accopanied by thalamic nerve dysfunction leading to hypoeasthetsia of the upper face. Optic nerve may also be involved resulting in visual impairment. Causes: Head and neck cancer, neural tumours, haematological cancer, sarcoid, SLE, GPA, Churg-strauss, GCA, Thyroid disease, Iatrogenic, carotid aneurysm

Question 174

Describe Sturge-Weber syndrome and its types and features.

Answer 174

A rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomengingeal angioma (cerebral malformations and tumours. Types: - Type 1 include facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal lesions, Normally only ones side of the brain is affected, this type is the most common - Type 2 involvement includes a facial angioma (Port wine stain) with a possibility of glaucoma developing. There is no envidence of brain involvement. Symptoms can show at any time beyond the initial diagnosis of the facial angioma. The symptoms can include glaucoma, cerebral blood flow abnormalities and headaches. - Type 3 has leptomengingeal angioma involvement exclusively. The facial angioma is absent and glaucoma rarely occurs. This type can only be diagnosed via brain scans.

Question 175

Describe Hereditary Neuralgic amyotrophic and its features

Answer 175

An Autosomal Dominant disorder associated in 50% of cases with a mutation of the SEPT9 gene on chromosome 17. Characterised by nerve damage, muscle atrophy, preceded by severe pain. Features: An episodic disorder characterised by episodes generally lasting 1-6 weeks. During an episode nerves of the brachial plexus are targeted by the body as antigens and are degenerated. Leading to loss of function and pain, if phrenic nerve is involved there can be difficulty with breathing and the recurrent laryngeal nerve leads to dysphonia. Management: -Prednisone. Nerve regenerates after an episodes though in some cases permanent damage may occur.

Question 176

Describe Familial Amyloid Polyneuropathy and its features.

Answer 176

Aka Transthyretin amyloidosis. An Autosomal Dominant neurodegenerative disease. Characterised by systemic deposition of amyloidogenic variants of the transthyretin protein, especially in the peripheral nervous system causing a progressive sesnory and motor polyneuropathy. Caused by a mutation of the TTR gene on Chromosome 18q12.1-11.2 a replacement of valaline by methionine at potion 30 is the most common mutation. Features: Usually manifesting between 20-40yrs. Characterised by pain, paraesthesia, muscular weakness, and autonomic dysfunction. In its terminal state the kidneys and heart are affected. Management: Liver transplantation.

Question 177

Describe Idiopathic Intracranial Hypertension, its features, investigations, and management.

Answer 177

Characterised by increased intracranial pressure without a detectable cause. More common in overweight can be triggered by tetracyclines. Features: Headache, (usually worst in the mornings generalised throbbing, worsened by coughing.) N&V, visual disturbance, pulsatile tinnitus, shoulder pain. Investigations: - Fundoscopy (Papiloedema - CT Head r/o SOL - LP Management: - Weight loss, exercise, salt restriction - Acetazolamide - rarely neurosurgery (optic nerve sheath fenestration)

Question 178

Describe Conus Medullaris Syndrome, its features.

Answer 178

A collection of signs and symptoms associated with injury to the conus medullaris (The tapered lower end of the spinal cord occur near L1/L2). Features: Sudden bilateral distal paresis. Urinary retention and faecal incontinence. Back pain more severe than radicular pain. Symmetrical sensory loss or perianal region. Preserved knee eras but absent ankle keys. Fasciculations.

Question 179

What are the differences between cauda equina and conus medullaris syndromes?

Answer 179

Presentation: Sudden and bilateral in conus and gradual and often unilateral in cauda. Reflexes: Knee jerks preserved and ankle jerks hyperreflexic in conus whereas both affected in cauda and are absent. Radicular pain: less severe in conus more in cauda Back pain: More severe in conus less in cauda Sensory symptoms: Bilateral perianal sensory loss, sensory dissociation occurs in conus whereas in cauda numbness is more saddle area and asymmetrical and may be unilateral. There is also loss of sensation in dermatomes of lower extremities. Motor strength: Distal hyper-Reflex is apreasis of lower limbs. Fasciculations may be present in conus. In cauda assymteric paraplegia areflexic. Atrophy more common. Sphincter dysfunction: Early urinary retention and faecal incontinence in conus, whereas urinary retention in cauda is a late feature

Question 180

Describe Multifocal motor neuropathy, its features, and management.

Answer 180

A progressive pure motor neuropathy often mistaken for amyotrophic lateral sclerosis. MMN only involves the lower motor neurones. Thought to be caused by autoantibodies against GM1. Features: Usually beginning in one or both hands MMN is characterised by weakness, muscle atrophy, cramping, and often profuse fasciculations. Sensory nerves are usually unaffected. Wrist drop and foot drop are common symptoms. Cold and Hot temperatures exacerbates MMN symptoms. Management: - IVIg - Cyclophosphamide, rituximab. - Prednisolone can exacerbate symptoms.

Question 181

Describe Idiopathic Brachial Plexopathy

Answer 181

Aka Parsonage-Turner syndrome Features: abrupt onset of shoulder pain (Usually unilateral) followed by progressive neurological deficits of motor weakness and paraesthesia may be associated with recent viral illness.

Question 182

Describe X-linked adrenoleukodystrophy, its features

Answer 182

An X-linked disease that results from fatty acid build up caused by dysfunction of peroxisomal fatty acid beet oxidation which leads to damage to the myelin sheath of nerves resulting in seizures and hyperactivity. Caused by mutations in the ABCD1 gene. All patients with ALD are at risk for adrenal insufficiency. Features: emotional instability, hyperactivity, disruptive behaviour at school. Later muscle stiffness, paraparesis and sexual dysfunction. Adrenal insufficiency.

Question 183

Describe Hemiballismus and its management

Answer 183

A movement disorder which manifests as unilateral involuntary flinging movements of the Proximal upper limbs. The lesion is in the contralateral subthalamic nucleus of Lucy’s Management: - Stroke TIA prevention - Haloperidol Lowe dose and tirated May help - Tetrabenazine (A catecholamine-depleting agent) may be considered when long-term therapy is required.

Question 184

What factors increase the risk of seizure recurrence on withdrawal of AED’s?

Answer 184

Older age at diagnosis Use of multiple antivconvulsants History of Myoclonic or tonic-clonic seizures. Previous abnormal imaging or EEG

Question 185

Describe Becker’s muscular dystrophy, its features

Answer 185

X-linked recessive disorder characterised by slowly progressing muscle weakness of the legs and pelvis. Caused by mutations in the dystrophin gene, similar to Duchenne muscular dystrophy but due to partial function of dystrophin is less severe and presents later. Features: Muscular weakness (Gradually increasing difficulty walking), Upper extremity muscle weakness, Toe-walking, SOB, Pseudohypertrophy of calf muscles, cardiomyopathy, Elevated CK.

Question 186

What are the distinguishing features between Duchenne and Becker’s Muscular dystrophy?

Answer 186

Duchenne: - Absent/Non functional dystrophin gene - 1/3600 Male births incidence - Onset 3-5 yrs - Fast disease progression - Mean life expectancy mid 20s - Onset of cardiomyopathy is after skeletal progression. Becker’s: - Partially functional dystrophin gene. - 6/100 000 male births incidence - Onset 12 yrs - Slow disease progression - Mean life expectancy 40s - Onset of cardiomyopathy May present before skeletal symptoms.

Question 187

Describes Spinal Muscular Atrophy, its features, and types.

Answer 187

A rare neuromuscular disorder characterised by loss of motor neurones and progressive muscle wasting often leading to early death. Genetic defect in the SMN1 gene. Types: - SMA1: aka Werdnig-Hoffman disease, infantile SMA presenting 0-6 months with floppy limbs and respiratory impairment - SMA2: aka Dubowitz disease, intermediate SMA, presents at 6-18months when able to sit up but not stand, Longer life expectancy. - SMA3: aka Kugelberg-Welander disease, Juvenile SMA, occurs after 12 months, able to walk without support when disease occurs. Life expectancy near normal. - SMA4: Adult onset, 3rd decade of life with gradual proximal muscle weakness. Normal life expectancy.

Question 188

Describe Vertebral Artery Dissection and its features

Answer 188

May originate in the neck and extend to the intracranial portion of the vessel or remain isolated to either segment. Ehlers-Danilo’s and Fibromuscular dysplasia are risk factors. Triggers: Rapid and extreme rotation of the neck, such as chiropractic manipulation or extension of the neck to have ones hairs washed, forceful coughing. Features: Symptoms may fluctuate over minutes to hours. Symptoms consist of posterior circulation deficits (Vertigo, diplopia, dysarthria) or infarction of the cervical spinal cord from occlusion of the anterior spinal artery. Axial MRI T1 weigted images show a doub le unmentioned in the dissected vessel and may reveal areas of infarction.

Question 189

Describe Bickerstaff’s Encapahlitis and its features, and management.

Answer 189

Brainstem Encephalitis, it may be related to Gillian-Barre syndrome. And in particular the Miller-fisher variant with which is shares the opthalmoplegia and ataxia. However in miller-fisher there is often areflexia and no disturbance of conciousness but similarly there is positive anti-GQ1b antibodies. It is normally an antecedent illness following LRTI. Features: progressive fairly symmetrical Opthalmoplegia, ataxia, disturbance of conciousness, and extensor plantar response +/- hyperreflexia. Management: -Steroids, immunoglobulins and plasma exchange.

Question 190

Describe Lance-Adams syndrome and its management

Answer 190

A rare condition describing post-hypoxic intention myoclonus usually following cerebral hypoxia e.g. cardiac arrest. Management: -Levetiracetam, clonazepam, valproate are first line treatments.

Question 191

Describe the HINTS exam

Answer 191

A bedside test used to rule out dangerous vestibular symptoms e.g. central causes. Comprises of Head Impulse test, Nystagmus, Test of Skew. A benign exam is defined as an ABNORMAL Head impulse test, Direction Fixed horizontal Nystagmus, and absent skew A dangerous HINTS exam is any one of the follow - Normal head impulse or untestable. - Direction changin horizontal nystagmus - Skew deviation present or untestable A dangerous HINTS results was found to be 100% sensitive and 96% specific for the presence of a central lesion when applied to a patient with acute vestibular syndrome with at least one stroke risk factor.

Question 192

Describe Subacute Degeneration of the Cord, its features and management.

Answer 192

Degeneration of the posterior and lateral columns of the spin cord as a result of Vitamin B12 defiency, Vitamin E and Copper deficiency. Most often associated with Pernicious anaemia. Features: Gradual and uniform onset of weakness of legs and arms, and trunk. Tingling and numbness that progressively worsens. Visual changes and confusion may occur. Bilateral spastic paresis can occur with associated pressure, vibration and touch sensory loss. Management: -Treat the cause i.e vitamin b12 replacement and neuro rehab,

Question 193

What is the helicopter sign?

Answer 193

A murmur sounding like a helicopter when auscultating the calves in orthostatic tremor