Neuro

Question 1

Anterior Spinal Artery infarct

Answer 1

Anterior spinal artery infarctions can occur after damage to the aorta, including both aneurysm repair and dissection.

single anterior spinal artery running down the midline of the spinal cord (in the anterior median fissure), blockage causes loss of pain, temperature, motor and autonomic function below that level.

Dorsal column function is preserved.

Question 2

Causes of autonomic neuropathy

Answer 2

Diabetes mellitus

Infections (including HIV, lyme and chagas)

Autoimmune disease (including SLE)

Amyloidosis

Question 3

Bell’s Palsy

Answer 3

Acute (but not sudden) onset, unilateral, lower motor neuron facial weakness, sparing the extraocular muscles and muscles of mastication.
Mild-moderate postauricular otalgia (which may precede paralysis).
Hyperacusis (actually quite uncommon).
Nervus intermedius symptoms (altered taste and dry eyes/mouth).

Question 4

Bell’s Palsy management

Answer 4

he central component of Bell’s Palsy management is the prompt administration of oral steroids: 50mg OM for 10 days, followed by a taper.

Supportive treatments including artificial tears and ocular lubricants, and an eye patch/tape (especially if protective Bell’s phenomenon is absent) should be considered

Question 5

Erb’s palsy

Answer 5

Involves the C5-6 nerve roots with corresponding dermatomal sensory loss, and the so-called “waiter’s tip” sign with shoulder adduction, elbow extension, forearm pronation and wrist flexion. It is most typically associated with shoulder dystocia and traumatic childbirth.

Question 6

Klumpke’s palsy

Answer 6

Involves the C8-T1 nerve roots with corresponding dermatomal sensory loss, and weakness of the intrinsic muscles of the hand. Uncommonly, T1 involvement may also result in an ipsilateral Horner’s syndrome

Question 7

Brown-Sequard Syndrome

Answer 7

Disruption of descending lateral corticospinal tracts, ascending dorsal column and ascending spinothalamic tracts leads to the following findings below the level of the injury:

Ipsilateral hemiplegia
Ipsilateral loss of proprioception and vibration
Contralateral loss of pain and temperature sensation

Question 8

Common causes of BSS include:

Brown-Sequard Syndrome

Answer 8

Cord trauma (penetrating injuries being the most common)
Neoplasms
Disk herniation
Demyelination
Infective/ inflammatory lesions
Epidural hematomas

Question 9

Bulbar palsy

Answer 9

bulbar palsy is a ‘lower motor neurone’ lesion affecting cranial nerves 9, 10 and 12

Clinical signs include an absent or normal jaw jerk reflex, an absent gag reflex, a flaccid fasciculating tongue, nasal quiet speech, and signs suggestive of the cause e.g. limb fasciculations of motor neurone disease.

Question 10

Cauda Equina Syndrome

management

Answer 10

Management

Patient with clinical features suggestive of spinal cord compression or cauda equina syndrome should have an urgent WHOLE spine MRI, with an aim (in appropriate cases) to surgically decompress within 48 hours.

In patients where malignancy is demonstrated on MRI, or in patients where clinical suspicion is high, administration of dexamethasone 16 mg daily in divided doses (with PPI cover) is indicated.

Question 11

he features of cerebellar dysfunction can be remembered by the acronym DANISH:

Answer 11

Dysdiadochokinesia (an inability to perform rapid alternating hand movements)
Ataxia (a broad-based, unsteady gait)
Nystagmus (involuntary eye movements)
Intention tremor (seen when the patient is asked to perform the ‘finger-nose test’)
Slurred speech
Hypotonia

Question 12

Cervical Spondylosis

Answer 12

Manifestations include:

Neck pain

Radiculopathy due to compression of nerve roots at the site of foraminal exit

Myelopathy, probably due to dynamic stretch of the spinal cord over impinging spinal osteophytes.

On examination, neck pain is accompanied by flaccid upper limb paresis (due to radiculopathy), variable sensory changes (sometimes including the Lhermitte phenomenon), and spastic paraparesis (with variable involvement of the upper limbs depending on the site of the lesion, and degree of radiculopathy).

Question 13

Charcot-Marie-Tooth Syndrome

Answer 13

most common being an autosomal dominant mutation in the peripheral myelin protein 22 gene (PMP22..

There are two broad groups of disease: type 1 which is a demyelinating condition which is more common (and includes the PMP22 subtype), and type 2 which is axonal.

Clinical features

As disease progresses (most commonly over decades) some key clinical features may be noted: (1. thickening and enlargement of the nerves themselves, (2. symmetrical distal muscular atrophy (champagne bottle legs, and claw hand), and (3. pes cavus (high-arched feet).

Diagnosis

Diagnosis is made with a combination of nerve conduction studies and genetic testing. Patients with type 1 have reduced conduction velocity, whereas this is normal in type 2. While the disease may be incurable, most have a normal lifespan with expert supportive management. Majority require walking aids eventually, though requiring a wheelchair is uncommon.

Question 14

Clinical features of Huntington’s disease

Answer 14

It is characterised by the triad of

Dominant inheritance
Choreoathetosis
Dementia

MRI and CT scans in moderate to severe disease can show loss of striatal volume and increased size of the frontal horns of the lateral ventricles.

Question 15

Management of Huntington’s disease

Answer 15

Patients and their families require a significant amount of physical and emotional support from a multidisciplinary team.

Chorea can be managed with a number of medications, with tetrabenazine having the most evidence-base.

Depression can be treated with Selective serotonin reuptake inhibitors (SSRIs) as a first choice.

Psychosis can be managed with antipsychotics, preferably newer atypical agents due to lower rates of extrapyramidal side effects.

Question 16

most common cause of Encephalitis

Answer 16

herpes simplex virus type 1

Question 17

Investigating encephalitis

Answer 17

Encephalitis should be suspected in any patient with sudden onset behavioural change, new seizures and unexplained acute headache with meningism.

Standard work up for encephalitis is similar to that for meningitis, with a routine panel of blood tests, blood cultures and viral PCR. CSF should also be sent for viral PCR in addition to standard tests, and malaria blood films should be considered if exposure is suspected.

CNS imaging may also be helpful – HSV has a predilection for the temporal lobes and bilateral multifocal haemorrhage is typical.

Question 18

treatment of encephalitis

Answer 18

Treatment of suspected encephalitis is empirical and should involve broad spectrum antimicrobial cover with 2g IV ceftriaxone BD and 10 mg/kg aciclovir TDS for two weeks.

Supportive management of complications including prompt termination of seizure activity with anticonvulsants (such as phenytoin) also important.

Question 19

clinical presentation - Absence seizures

Answer 19

Patients, often children, pause briefly, for less than 10 seconds, and then carry on where they left off.

Treatment: Ethosuximide 1st line Sodium Valproate (SE: weight gain, hair loss which grows back curly, oedema, ataxia, tremor, teratogenicity)

Question 20

Tonic-clonic seizures:

Answer 20

Patients lose consciousness; their limbs stiffen (tonic) and start jerking (clonic). Post-ictal confusion is common.

Treatment: Sodium Valproate or Lamotrigine is first-line.

Question 21

Myoclonic seizures:

Answer 21

udden jerk of a limb, trunk, or face.

Treatment: Sodium Valproate is first-line unless the patient is a female of childbearing age where Levetiracetam or Topiramate should be used instead as first-line; avoid Carbamazepine as it worsens seizures.

Question 22

Atonic seizures:

Answer 22

Atonic seizures: Sudden loss of muscle tone, causing the patient to fall, whilst retaining consciousness. Treatment: Sodium Valproate or Lamotrigine is first-line.

Question 23

Essential tremor

Answer 23

mainly the upper limbs distally and is postural or kinetic.

amplitude increases with time, and patients experience difficulty in writing, eating, holding objects and doing fine motor tasks, dressing, and speaking

increares with stress

Question 24

Essential tremor management

Answer 24

Management

The management of a patient with ET includes:

Behavioural techniques and physical therapy
Medical (pharmacological) therapy (with agents such as propranolol, primidone, topiramate, gabapentin, clonazepam etc.)
Surgical treatment (such as deep brain stimulation, focused ultrasound thalamotomy and radiosurgical (Gamma Knife) thalamotomy)

Question 25

Extradural Haemorrhage

Answer 25

brief loss of consciousness, followed by regaining of normal consciousness level (the lucid interval), with subsequent deterioration of consciousness and headache.

Question 26

Other causes of peripheral nerve disease include:

Answer 26

Other causes of peripheral nerve disease include: Physical lesions of the cerebellopontine angle (including the classical acoustic neuroma) Basal meningitis (which is often bilateral and may be infective or inflammatory, including lyme disease and sarcoidosis). Ramsay Hunt syndrome (herpes zoster of the facial nerve) Trauma Diseases of the middle/inner ear Mononeuritis multiplex

Question 27

Fibromuscular dysplasia

Answer 27

Fibromuscular dysplasia (FMD), formerly called fibromuscular fibroplasia, is a group of non-atherosclerotic, non-inflammatory arterial diseases that most commonly involve the renal and carotid arteries.

Question 28

Causes of Foot Drop

Answer 28

L5 root lesion (radiculopathy) – distinguished by loss of inversion (inversion is a tibial nerve function and is not lost in patients with a common peroneal nerve lesion). Sensory loss is over the L5 dermatome, and sciatica-type shooting leg pain may also be present. Lumbosacral disc herniation is the most common cause. Distal motor neuropathy – often associated with glove and stocking sensory disturbance, and loss of all movements of the foot. Small cortical lesions – often with though other upper neuron features may be present. Uncommonly: intrinsic cord disease, partial sciatic nerve disease and myopathy may also mimic foot drop.

Question 29

Friedreich's ataxia

Answer 29

autosomal recessive trinucleotide repeat disorder resulting in reduced level or function of the frataxin protein. Patients typically present in their teenage years with lower limb weakness, gait abnormalities, or falls. On physical examination there may be cerebellar signs (due to involvement of the spinocerebellar tracts) or mixed upper and lower motor neurone signs e.g. absent knee/ankle reflexes but upgoing plantars (due to involvement of the corticospinal tracts and peripheral nerves). Involvement of the dorsal columns can lead to impaired joint/vibration sense. Other clues on physical examination include a high-arched palate, pes cavus, and kyphoscoliosis. Non-motor features include: hypertrophic obstructive cardiomyopathy, reduced visual acuity, type 1 diabetes mellitus, and deafness.

Question 30

Miller-Fisher syndrome

Answer 30

(a variant of GBS) presents with ataxia, ophthalmoplegia, and areflexia. The condition is typically positive for anti-GQ1b antibodies.

Question 31

Acute management of haemorrhagic stroke

Answer 31

Neurosurgical and neurocritical care evaluation due to the potential for surgical intervention (e.g. decompressive hemicraniectomy). Admission to the neuro ICU or stroke unit (the patient may require intubation and ventilation or invasive monitoring of ICPs). Aim to keep blood pressure <140/80 as poor blood pressure control in the acute stage is associated with poorer outcomes later on

Question 32

Diagnosis of Migraine

Answer 32

Presence of an aura confirms the diagnosis. Otherwise, in headaches with no aura, the following criteria is required - at least 5 headaches lasting 4-72 hours, with nausea/vomiting or photo/phonophobia AND 2 of: unilateral headache, pulsating character, impaired or worsened by daily activities.

Question 33

Management of Migraine

Answer 33

Avoid triggers; prophylaxis with Propranolol (CI: asthma) or Topiramate; managing an acute attack with an oral triptan such as Sumatriptan (CI: ischaemic heart disease), in addition to Paracetamol or an NSAID. Ensure that female patients are not taking combined oral contraceptive pills as it increases their risk of ischaemic stroke.

Question 34

Management of Cluster Headaches

Answer 34

Avoid triggers; prophylaxis with Verapamil; managing an acute attack with 100% oxygen via non-rebreathable mask (CI: COPD) with a subcutaneous or nasal Triptan (CI: ischaemic heart disease).

Question 35

Symptoms of Raised intracranial pressure

Answer 35

Headaches which are worse in the morning and upon bending over, and improve after vomiting and lying down. May be associated with neurological deficits due to compression of cranial structures by a space-occupying lesion, such as a tumour or haemorrhage.

Question 36

Hereditary spastic paraparesis d

Answer 36

main pathological feature is axonal degeneration of the corcisopinal tracts in the spinal cord. Diagnostic criteria include: a family history, progressive gait disturbance, spasticity of lower limbs, hyper-reflexia of lower limbs, and extensor plantar responses. Typically the power in lower limbs muscles is normal/only mildly reduced.

Question 37

Idiopathic Intracranial Hypertension

Answer 37

Classical clinical features include headache and visual disturbance. The headache is classically non-pulsatile, bilateral, and worse in the morning (after lying down or bending forwards). Patients may even report morning vomiting. A key differential diagnosis for morning headache in these obese patients is sleep apnoea. Visual disturbances include transient visual darkening or loss, probably due to optic nerve ischaemia, and bilateral papilloedema is seen on fundoscopy. If left untreated, permanent visual damage may result (untreated, 1-3% of patients may lose vision by year).

Question 38

Drug associationsIdiopathic Intracranial Hypertension

Answer 38

It is associated with a number of drugs including: ``` Oral contraceptive pill Steroids Tetracycline Vitamin A Lithium ```

Question 39

Management Idiopathic Intracranial Hypertension

Answer 39

First line management of idiopathic intracranial hypertension (and the only intervention supported by good evidence) is weight loss. Failing this, patients often try carbonic anhydrase inhibitors, such as acetazolamide, but its extensive profile of side effects (peripheral paraesthesia, anorexia and metallic dysgeusia) mean that it is poorly tolerated. Topiramate and furosemide are also commonly tried. More invasive strategies to lower CSF pressure including therapeutic lumbar punctures and surgical CSF shunting are tried in resistant cases. In patients with prominent visual symptoms (but otherwise manageable headache), optic nerve sheath fenestration may protect against visual loss.

Question 40

Internuclear ophthalmoplegia

Answer 40

caused by a lesion of the medial longitudinal fasciculus = Ipsilateral impaired adduction in the eye Nystagmus in the abducting eye

Question 41

Intracranial Venous Thrombosis

Answer 41

Intracranial venous thrombosis refers to occlusion of venous vessels in the cranial cavity. common symptoms include headache, confusion/drowsiness, impaired vision, and nausea/vomiting. Other signs include seizures, reduced consciousness, focal neurological deficitis, cranial nerve palsies, and papilloedema. Investigations Non-contrast CT reveals a hyperdensity in the affected sinus. CT venogram is used to look for a filling defect ('the empty delta sign'). Treat LMWH

Question 42

Subdural haemorrhage

Answer 42

tend to occur in older patients. Other risk factors include historic head trauma, alcoholism, and anticoagulation. On CT, a crescent-shaped haematoma is diagnostic. (hyper dense = acute and hypodense = chronic))

Question 43

The key steps in secondary stroke prevention can be remembered by the mnemonic HALTSS:

Answer 43

Hypertension: studies show there is no benefit in lowering the blood pressure acutely (as this may impair cerebral perfusion) unless there is malignant hypertension (systolic blood pressure >180 mmHg). Anti-hypertensive therapy should, however, be initiated 2 weeks post-stroke. Antiplatelet therapy: patients should be administered Clopidogrel 75 mg once daily for long-term antiplatelet therapy. In patients with ischaemic stroke secondary to atrial fibrillation, however, warfarin (target INR 2-3. or a direct oral anticoagulant (such as Rivaroxaban or Apixiban) is initiated 2 weeks post-stroke. Lipid-lowering therapy: patients should be prescribed high dose atorvastatin 20-80 mg once nightly (irrespective of cholesterol level this lowers the risk of repeat stroke). Tobacco: offer smoking cessation support. Sugar: patients should be screened for diabetes and managed appropriately. Surgery: patients with ipsilateral carotid artery stenosis more than 50% should be referred for carotid endarterectomy.

Question 44

Lambert-Eaton Myasthenic Syndrome

Answer 44

auto-immune disorder characterised by antibodies against the pre-synaptic voltage-gated calcium channels. Clinical features Patients typically present with limb weakness and autonomic features (xerostomia, orthostatic hypotension, and impotence). Clues on examination include reduced or absent tendon reflexes that are potentiated by brief contraction of the relevant muscle (due to the build up of acetylcholine in the synaptic cleft with repeated contraction, overcoming the inhibitory effect of pre-synaptic voltage-gated calcium channel blockade). Investigations Bloods for anti-voltage-gated calcium channels Nerve conduction studies will show a doubling of muscle action potential amplitude with exercise. It is important to carry out further investigation, as guided by the history, to rule out underlying malignancy or autoimmune disease. Chest x-ray and CT chest, for example, should be carried out to rule out small cell lung cancer.

Question 45

LEMS management

Answer 45

The mainstay of management is to address the underlying cause e.g. small cell lung cancer. Amifampridine has been used to manage LEMS. Amifampridine blocks pre-synaptic potassium channels in the nerve terminals. This prevents potassium efflux, prolonging the action potential in the nerve terminal. This prolongs the opening time of voltage-gated-calcium channels, augmenting the release of acetylcholine in the synaptic cleft. In severe respiratory impairment or bulbar weakness intubation and ventilation and plasma exchange/intravenous immunoglobulin (IVIG) may be necessary.

Question 46

Medical third nerve palsy

Answer 46

ptosis (due to impaired innervation to levator palpebrae superioris) and a 'down and out' pupil (due to unopposed activation of lateral rectus and superior oblique). In a 'medical' third nerve palsy there is pupil sparing. This is because the parasympathetic (constrictive) fibres run on the outside of the nerve. Medical lesions will affect the blood supply to the inner (motor) fibres of the nerve (the vasa vasorum), leaving the outer parasympathetic fibres intact. The most common cause of a medical third nerve palsy is diabetes. Other causes include those that increase the risk of atherosclerosis, such as hypertension, and vasculitis.

Question 47

Meralgia paresthetica

Answer 47

compression of the lateral cutaneous nerve of the thigh underneath the inguinal ligament. It is characterised by shooting pains along the outer aspect of the upper leg.

Question 48

The four clinical groups are: MND

Answer 48

Spinal ALS (the classic MND syndrome) Bulbar ALS (with early tongue and bulbar involvement) Progressive muscular atrophy (with only lower motor neuron features) Primary lateral sclerosis (with only upper motor neuron features).

Question 49

Acute management of MS

Answer 49

An acute attack of multiple sclerosis should be treated with glucocorticoids. 1g of intravenous methylprednisolone every 24 hours for 3 days is a typical regimen, however oral courses are probably equally as effective. Infections must first be excluded. Severe acute attacks who do not respond to glucocorticoids should be covered with plasma exchange. While these interventions does not appear to affect long term outcome, it does appear to reduce the duration and severity of individual attacks. In practical terms, it is usually necessary to involve the local neurology team for guidance on when to start steroids for patients with an acute flare of multiple sclerosis. Always ensure to check routine bloods and urine dip to rule out any intercurrent infection.

Question 50

Chronic management of MS

Answer 50

First-line injectables such as beta-interferon and glatiramer New oral agents such as dimethyl fumarate, teriflunomide and fingolimod Biologics such as natalizumab and alemtuzumab.

Question 51

Myasthenia Gravis

Answer 51

antibodies against the nicotinic acetylcholine receptors on muscle fibres weakness affecting limb muscles, extra-ocular muscles (drooping eyelids, diplopia), facial muscles (difficulty smiling or chewing), and bulbar muscles (change in speech or difficulty swallowing).

Question 52

Myasthenia Gravis investigations

Answer 52

Investigations Blood tests for serum acetylcholine receptor antibody (detected in 80-90% with generalised myasthenia gravis, >90% sensitivity in generalised myasthenia gravis, 99% specificity), muscle-specific tyrosine kinase antibodies (should be ordered if the acetylcholine receptor antibody is negative or equivocal). Imaging investigations include CT of the chest (65% have thymic hyperplasia, 12% have a thymoma. This will inform the decision regarding consideration for thymectomy). Special tests include repetitive nerve stimulation (in myasthenia gravis there is a >10% fall in muscle action potential between the 1st and 4th action potential in a series of 10 stimulations of the alpha motor neurone).

Question 53

Myasthenia Gravis management

Answer 53

nservative management includes regular review in neurology outpatients, with involvement of the MDT as required (occupational therapist, physiotherapist, speech and language therapist if the patient suffers from dysarthria etc.) Medical management is with immunosuppressive therapy (such as steroids, given acutely) and anticholinesterase inhibitors (such as pyridostigmine or neostigmine). Acetylcholinesterase inhibitors (AKA anticholinesterases) inhibit the breakdown of acetylcholine in the synaptic cleft, increasing acetylcholine concentrations in the synaptic cleft, and increasing activation of nicotinic acetylcholine receptors at the neuromuscular junction. Acutely, intravenous immunoglobulin or plasmapheresis may be considered in severe, steroid-refractory, cases. Side effects of acetylcholinesterase inhibitors include diarrhoea, salivation, lacrimation, and urinary frequency. Surgical management with thymectomy is considered in some patients.

Question 54

Neuromyelitis optica (Devic's disease)

Answer 54

Optic neuritis Transverse myelitis, Positive NMO-IgG (an antibody targeting aquaporin 4.. Management Management of Devic's disease is with immunosuppression (e.g. with the anti-CD20 agent rituximab).

Question 55

Optic neuritis

Answer 55

Visual loss Periocular pain Dyschromatopsia first line of treatment for ON is intravenous methylprednisolone

Question 56

simple first-step in the management of patients with raised ICP

Answer 56

Head elevation to 30º

Question 57

typically spared in motor neurone disease

Answer 57

Eye movements

Question 58

MS investigation

Answer 58

MR with contrast

Question 59

tremor seen in Parkinson's disease

Answer 59

unilateral tremor that improves with voluntary movement

Question 60

Core features of Parkinson's disease

Answer 60

Its core motor features include bradykinesia, asymmetric 3-5Hz "pill-rolling" tremor, and lead pipe rigidity. The combination of lead pipe rigidity and tremor results in the phenomenon known as cogwheeling, a jerkiness felt when testing a patient's tone.

Question 61

Side effects of L-dopa

Answer 61

Common side effects include: Hypotension Restlessness Gastrointestinal upset In rare cases, dopamine excess can result in psychiatric reactions including acute psychosis. Peripheral side effects are reduced by the co-administration of a peripheral dopa decarboxylase inhibitor, such as carbidopa.

Question 62

A pseudobulbar palsy

Answer 62

bilateral lesion affecting the corticobulbar tracts (running from the motor cortex to the motor nuclei of cranial nerves 9, 10, and 12 in the medulla) Clinical features Clinical signs include a spastic tongue, a slow thick ("hot-potato") speech, a brisk jaw jerk reflex, and emotional lability. Other upper motor neurone features in the limbs may be present e.g. spastic hypertonia, pyramidal weakness, and hyper-reflexia.

Question 63

Features of pseudoseizures

Answer 63

Pseudoseizures (now known as psychogenic nonepileptic attacks, PNEAs) are often prolonged episodes (rarely <1 minute, often >30 minutes), usually in front of a witness during which the patient exhibits fluctuating motor activity and seizure-like convulsions. Tongue biting is rare. In equivocal cases, video EEG recording may be indicated to differentiate PNEAs from true seizures. The extended differential diagnosis includes hemiplegic migraine, and cerebrovascular events, though consciousness is typically preserved and the neurological pattern of suggestive. In patients with true seizure activity and focal weakness, differentiating seizure plus Todd's paresis from stroke with seizure at onset is difficult clinically and often requires imaging.

Question 64

Suspect a spastic hemiparesis if the following signs are present:

Answer 64

unilateral spastic hypertonia, hyper-reflexia, ankle clonus, and upgoing plantar.

Question 65

Central Causes of Spastic hemiparesis

Answer 65

auses of lesions in the cerebral hemisphere include: multiple sclerosis, a space-occupying lesion (such as a tumour or a cerebral abscess), a stroke, or hemiplegic cerebral palsy.

Question 66

Peripheral Causes of Spastic hemiparesis

Answer 66

Causes of lesions in the hemicord include multiple sclerosis, cord compression (although this is more likely to present with bilateral upper motor neurone signs in the lower limbs).

Question 67

Clinical Features of Spastic paraparesis

Answer 67

Spastic paraparesis presents with: lower limb spastic hypertonia, ankle clonus, pyramidal weakness (extensors stronger than flexors in the lower limbs), hyper-reflexia and upgoing plantars (upper motor neurone signs). There is scissoring gait.

Question 68

Spinal Stenosis

Answer 68

Lumbar spinal stenosis is an important differential diagnosis for a patient with leg pain brought on by exercise, which is often misattributed to the intermittent claudication of peripheral vascular disease. Neurogenic claudication more commonly manifests as proximal thigh or buttock pain, which is cramping in character and may be associated with other neurological symptoms such as burning or tingling. It is worse with exercise, but is often not immediately relieved when standing still, and may be worse in extension (descending stairs) and improved in flexion (some patients describe being able to cycle far in excess of their exercise tolerance on foot).

Question 69

Acute management of status epilepticus

Answer 69

Premonitory stage (0-10 minutes) - Diazepam 10−20 mg given rectally, repeated once 15 minutes later if status continues to threaten, or midazolam 10 mg given buccally. If seizures continue, treat as below. Early status (0-30 minutes) - Lorazepam (intravenous) 0.1 mg/kg (usually a 4 mg bolus, repeated once after 10−20 minutes; rate not critical). Give usual AED medication if already on treatment. For sustained control or if seizures continue, treat as below. Established status (0-60 minutes) - Phenytoin infusion at a dose of 15–18 mg/kg at a rate of 50 mg/minute or phosphenytoin infusion at a dose of 15−20 mg phenytoin equivalents (PE)/kg at a rate of 50–100 mg PE/minute and/or phenobarbital bolus of 10–15 mg/kg at a rate of 100 mg/minute. Refractory status (30-90 minutes) - General anaesthesia, with one of: Propofol (1–2 mg/kg bolus, then 2–10 mg/kg/hour) titrated to effect Midazolam (0.1–0.2 mg/kg bolus, then 0.05–0.5 mg/kg/hour) titrated to effect Thiopental sodium (3–5 mg/kg bolus, then 3–5 mg/kg/hour) titrated to effect; after 2–3 days infusion rate needs reduction as fat stores are saturated Anaesthetic continued for 12−24 hours after the last clinical or electrographic seizure, then dose tapered.

Question 70

Surgical third nerve palsy

Answer 70

In a 'surgical' third nerve palsy there is pupil involvement. This is because the parasympathetic (constrictive) fibres run on the outside of the nerve. External compression will impair function of these fibres, causing pupil dilation.

Question 71

Benign positional paroxysmal vertigo (BPPV) features

Answer 71

The presence of debris in the semicircular canals of the ears causes vertigo upon head movement. The Hallpike manoeuvre is diagnostic, where certain movements of the head causes fatiguable nystagmus. Epley manoeuvres treat BPPV by clearing the debris.

Question 72

Acute labyrinthiti

Answer 72

cute severe vertigo, which may be associated with vomiting. | plus hearing loss and tinnitus,

Question 73

Ménière's disease feature

Answer 73

Endolymphatic system pressure increases causes recurrent episodes of vertigo, sensorineural hearing loss, tinnitus, and a feeling of aural fullness. Treatment involves antihistamines and bed rest.

Question 74

Acoustic neuroma features

Answer 74

Actually a vestibular schwannoma. They usually present with unilateral hearing loss and progress to involve cranial nerves 5, 6, 9, 10, and the ipsilateral cerebellum.

Question 75

Visual field loss at Optic nerve:

Answer 75

Monocular visual loss

Question 76

Visual field loss at Optic chiasm

Answer 76

Bitemporal hemianopia

Question 77

Visual field loss at Optic tract

Answer 77

Contralateral homonymous hemianopia

Question 78

Visual field loss at Optic radiation

Answer 78

Optic radiation (backward extension of the optic tract - from the lateral geniculate body of the thalamus to the ipsilateral primary visual cortex): Contralateral homonymous hemianopia

Question 79

Visual field field loss at temporal lobe , parietal lobe, occipital lobe

Answer 79

A lesion in the temporal lobe would cause contralateral homonymous superior (upper) quadrantanopia A lesion in the parietal lobe would cause contralateral homonymous inferior (lower) quadrantanopia A lesion in the occipital lobe would cause contralateral homonymous hemianopia

Question 80

pellagra

Answer 80

B3 def | 3 d's - dementia, dermatitis, diarrhoea

Question 81

Causes of autonomic neuropathy includes:

Answer 81

Diabetes mellitus Infections (including HIV, lyme and chagas) Autoimmune disease (including SLE) Amyloidosis

Question 82

Myotonic dystrophy

Answer 82

myotonic dystrophy is a trinucleotide repeat disorder affecting muscle-specific chloride channels. It is inherited in an autosomal dominant pattern. Clinical features: patients typically present in their 20s. It is useful to think of the clinical features from head to toe: Features in the face: frontal balding, myopathic facies (a long and thin face), bilateral ptosis, cataracts. Features in speech: dysarthria (caused by a myotonic tongue and pharyngeal muscles). Features in the neck: wasted sternocleidomastoid muscles. Features in the hands: distal wasting and weakness, slow relaxing grip, percussion myotonia (thumb flexion on percussion of the thenar eminence). Internal features: insulin resistance/metabolic syndrome, cardiomyopathy/arrhythmia, testicular atrophy.

Question 83

one treatment to extend life in MND

Answer 83

Riluzole