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Neurology

51. A 25-year-old woman is brought to the accident and emergency department aftersuffering a blackout. She was waiting for the bus when she felt nauseated, dizzy and

sweaty. She lost consciousness for 1 minute. A friend says she was pale, had some jerkingof her limbs for 10 s, but did not bite her tongue and there was no history of urinary

incontinence. She recovered immediately with no confusion or disorientation. Examination

is normal. ECG is normal. What further investigations are required to made a diagnosis?

CT brain

Doppler of carotids

EEG

MRI brain

No investigations Your answer

The most likely diagnosis is vasovagal syncope. It is usually preceded by symptoms of

dizziness, feeling hot, nausea and darkening of vision. The syncope only lasts for a short

period (up to a minute). Commonly there is some jerking of the limbs due to brainhypoperfusion. Tongue biting does not occur. Patients recover quickly after the syncope.

Common predisposing factors are pain, prolonged standing, a hot environment and a heavy

meal. No investigations are needed apart, perhaps, from an ECG in typical cases.

52. A 69-year-old man with recently diagnosed coeliac disease presents withparaesthesias in the feet and gait disturbance. On lower limb examination he hasincreased tone and weakness predominantly affecting the flexor muscle groups.Knee jerks are brisk and ankle jerks are absent. Both plantar responses areabsent. He has loss of vibration sense to the costal margins and impaired joint-position sense at the toes, although pinprick and temperature sensations arenormal. He has a macrocytic anaemia and his vitamin B12 level is confirmed to bevery low.

What imaging abnormalities are likely to be seen on spinal MRI?

Diffuse patchy white-matter lesions on T2-weighted imaging

Evidence of microhaemorrhages in the pyramidal tracts

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Evidence of microhaemorrhages in the posterior columns

Increased T1-weighted signal in the pyramidal tracts

Increased T2-weighted signal in the posterior columns

Your answer

He has subacute combined degeneration of the cord due to vitamin B12(cobalamin) deficiency. This causes the classic combination of a slowly evolvingspastic paraparesis (long tract signs), peripheral neuropathy (absent ankle jerks)and a posterior column pattern of sensory loss (vibration and joint-positionsense). The MRI typically shows increased signal on T2-weighted imaging,predominantly in the dorsal columns. This probably represents the knownpathological changes of cord oedema and demyelination in the posterior andlateral cord. (These MRI features are, however, non-specific and can be seen in

other conditions, eg multiple sclerosis.) Axonal loss and cord atrophy maydevelop in long-standing cases. However, the changes are partly or fullyreversible in most cases with vitamin B12 replacement therapy.

53. A 34-year-old woman attends your clinic and gives a description of recurrentearly morning attacks of severe throbbing unilateral orbital pain over the last 2months. Each attack lasts 6090 minutes, but leaves a dull ache around the eye

lasting for hours. These have not occurred previously. She feels that her lifestyleis severely compromised and is becoming depressed. There are no abnormalitieson examination. Blood pressure is 140/80 mmHg. What is the most likelydiagnosis?

Migraine without aura

Parotid tumour

Cluster headaches

Your answer

Paroxysmal hemicrania

Analgesic overuse headaches

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The symptoms are characteristic of cluster headache. Migraine is possible, but

the length of attack, the frequency and the occurrence first thing in the morningare all suggestive of cluster headache. Paroxysmal hemicrania causes pain ofmuch shorter duration. There is no history of analgesic overuse. The possibilityof structural abnormalities in the head or neck with this condition should alwaysbe considered, but the non-progressive nature makes it less likely in this case.

54. A 32-year-old intravenous drug abuser is admitted with a one-day history of double

vision, droopy eyelids and a dry throat. Over the next 24 hours, her symptoms worsen andshe begins to notice limb weakness and breathing difficulties. Examination reveals poorly

reactive pupils, decreased deep-tendon reflexes and symmetrical upper and lower limbweakness, but sensation is normal. Cerebrospinal fluid examination is normal. The

edrophonium test is positive. Electromyography shows no postactivation exhaustion.

What is the most likely diagnosis?

Botulism Your answer

LambertEaton syndrome

MillerFisher variant of the GuillainBarr syndrome

Myasthenia gravisPoliomyelitis

Botulism is caused by a toxin produced by the anaerobic bacterium Clostridium botulinum,

which blocks the release of acetylcholine from the motor nerve terminal. Botulism can bespread by the ingestion of foods contaminated by inadequate sterilisation, or, as in this

case, through wound contamination in drug abusers after subcutaneous injection of heroin

(the poor venous access of chronic addicts precludes intravenous heroin administration).Early symptoms are blurred vision, dysphagia and dysarthria. Pupillary responses to light

are impaired, tendon reflexes reduced and typically there is progressive symmetrical limb

weakness. Most patients have evidence of autonomic dysfunction such as dry mouth,

constipation or urinary retention.

The edrophonium test is falsely positive in about one-third of cases and does not

distinguish botulism from other causes of neuromuscular blockade. Lambert Eaton

Myasthenic syndrome(LEMS) could conceivably present with similar signs (reducedreflexes, autonomic signs), but the clinical context is less suggestive of this diagnosis, as

LEMS is most often a paraneoplastic disorder; in addition the rapid decline would be very

atypical of LEMS. Cerebrospinal fluid examination is associated with a high protein

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content in GuillainBarr syndrome and with pleocytosis in poliomyelitis. In myasthenia

gravis, there is postactivation exhaustion on electromyography.

55. A 28-year-old woman began to stumble when walking. Her right leg wasslightly stiff and weak, especially after exercise and hot showers. Thesesymptoms developed over 3 days and gradually disappeared over 4 weeks. Shewas on the college swimming team before these symptoms arose. There, shedeveloped a unique and extreme type of fatigue that was different from theusual fatigue she experienced when swimming. This disappeared after severalweeks, but had reappeared again 6 months before her current presentation.Which of the following diagnoses is most likely?

Myalgic encephalomyelitis (chronic fatigue syndrome)

Acute disseminated encephalomyelitis

Multiple sclerosisYour answer

GuillainBarr syndrome

Diabetic sensorimotor neuropathy

Patients with multiple sclerosis commonly complain that their symptoms areworse with exercise and heat. They also experience profound fatigue. The focalneurological syndrome described in the initial paragraph is inconsistent withchronic fatigue syndrome. Acute disseminated encephalomyelitis tends to causea more acute demyelinating syndrome with impaired consciousness. GuillainBarr syndrome and diabetic neuropathy are symmetrical.

56. In a patient with diplopia which one of the following findings is MOSTsuggestive of myasthenia gravis?

Loss of pin prick sensation around the chin area

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Preserved pupillary light reflex with absent accommodationreflex

Thymoma on computed tomography scan (CT scan) of the chest

Your answer

Elevated creatinine phosphokinase (CPK)

Proptosis

Myasthenia gravis is an acquired autoimmune disorder associated withacetylcholine receptor deficiency at the motor endplate. The ocular muscle

involvement is usually, bilateral, asymmetrical and typically associated withptosis and diplopia. Pupillary and accommodation reflexes are characteristicallynormal. Two-thirds of patients with myasthenia gravis have thymic hyperplasiaand 10%15% will have thymoma. The creatinine phosphokinase (CPK) is typicallynormal. Exophthalmos and diplopia are suggestive of Graves disease. There isrestriction in upward and/or outward gaze; this is due not to weakness of thesuperior eye muscles but to swelling and fibrosis of the inferior rectus andinferior oblique muscles beneath the globe.

57. A 25-year-old man presents with a 3-day history of diplopia and unsteadiness. He had

had an upper respiratory infection 3 weeks ago. On examination there is bilateral partial

ptosis, complete ophthalmoplegia, areflexia and gait ataxia. Sensory examination is normal,and plantar response flexor bilaterally.

Given the likeliest clinical diagnosis, what is the most appropriate treatment?

Azathioprine

Immunoglobulins Your answer

Prednisolone

Pyridostigmine

Thiamine

The most likely diagnosis is Miller, Fisher syndrome, a GuillainBarr syndrome variant. It

presents with a triad of ophthalmoplegia, areflexia and ataxia. It usually followsgastroenteritis or an upper respiratory tract infection. Positive anti-GQ1b ganglioside

antibodies (serum and CSF) are diagnostic. The treatment of choice is either

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immunoglobulins or plasma exchange. The majority of patients improve, but recovery may

be slow (weeks to months).

58. A 48-year-old man, with a history of chronic intravenous drug abuse, presents with a

severe subacute neuropathy. Nerve conduction studies show evidence of a multifocalaxonal neuropathy. He is also noted to have nailfold infarcts in his hands and feet and

hepatomegaly.

Which of the following diagnoses is the most likely?

Cytomegalovirus polyradiculoneuropathy

Systemic toxoplasmosis

Hepatitis B-related GuillainBarr syndromeHepatitis C-related GuillainBarr syndrome

Hepatitis C-related cryoglobulinaemia Your answer

The presence of nailfold infarcts and the multifocal nature of the neuropathy indicate that avasculitic cause is most likely. Hepatitis C infection may be associated with

cryoglobulinaemia, which causes a vasculitic syndrome including neuropathy. Treatment

of the vasculitis may include one or several of the following: high-dose intravenous

steroids, plasma exchange, intravenous immunoglobulins. In addition, interferon therapymay be indicated for clearance of the hepatitis viral load. The latter is usually decided in

conjunction with gastroenterology colleagues. Other conditions associated with vasculitic

neuropathy include polyarteritis nodosa, ChurgStrauss syndrome, rheumatoid arthritis,systemic lupus erythematosus, systemic sclerosis and Wegeners granulomatosis.

59. A 42-year-old man has been in the intensive care unit for 6 weeks following a roadtraffic accident in which he had crashed his car after drinking heavily. You are called to see

him as the staff has noticed that he appears to have developed some distal limb weakness

over the past 710 days. On examination a symmetrical distal weakness is confirmed and

he appears to have absent ankle and knee reflexes. There is some wasting of the intrinsic

hand muscles bilaterally, worse on the right. He has possible patchy sensory loss in aglove and stocking distribution, although this is inconsistent on testing.

Which of the following is the most likely diagnosis?


Critical illness polyneuropathy Your answer

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Critical illness myopathy

Nutritional polyneuropathy

Alcoholic polyneuropathy

Prolonged periods in intensive care, irrespective of the underlying pathology, are

associated with a risk of developing critical illness polyneuropathy. This is an axonalneuropathy and thus muscle wasting may occur. It may be predominantly sensory,

predominantly motor or mixed. Nerve conduction studies and electromyography may beindicated to confirm the diagnosis and exclude any other condition that may require

specific therapy (eg GuillainBarr syndrome). A patient with a previous history of chronic

alcohol misuse, diabetes or another condition predisposing to neuropathy may be moreprone to develop significant critical illness polyneuropathy.

60. A 30-year-old man is brought to the emergency department with severe confusion andataxia. On examination he has bilateral VIth nerve palsies and gait ataxia. He is disorientedin time and place. A full blood count shows an MCV of 110 fl. CT brain and EEG are both

normal. What immediate treatment should be administered?

Intravenous aciclovir

Intravenous cefuroxime

Intravenous dexamethasone

Intravenous diazepam

Intravenous thiamine Your answer

The combination of confusion, ataxia and ophthalmoplegia are the typical features ofWernickes encephalopathy. Other less common features include autonomic dysfunction

and hypothermia. The high MCV is most likely to be consistent with chronic alcohol abuse.

Other causes of Wernickes encephalopathy include vomiting and chemotherapy.

Immediate intravenous thiamine is the treatment of choice.

61. A 69-year-old woman presents with a sudden onset of weakness of her right arm and

leg. She is known to be hypertensive. There has been no headache, loss of consciousness,visual, speech or sensory symptoms. On examination, her blood pressure is 180/90 mmHg,

pulse 100 and regular. Heart sounds are normal. There is no carotid bruit. Higher mental

function tests are normal. There is no apraxia or neglect. Speech, swallowing and sensationare all normal. There is no visual field defect. There is a mild facial weakness sparing the

forehead. The right arm and leg are flaccid and weak. Reflexes and tone are normal. There

is a right extensor plantar response. What is the most likely cause of this patients

symptoms?

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Cardioembolic stroke

Lacunar stroke Your answer

Right internal carotid artery atheroembolic stroke

Right internal carotid artery dissection

Right vertebral artery atheroembolic stroke

A sudden onset of focal symptoms and signs strongly suggests stroke. The presence of

right-sided weakness involving the face, arm and leg, in the absence of sensory loss,language disturbance, hemineglect or visual field defect favours a small deep (lacunar)

infarct affecting the corticospinal tract at the level of either the internal capsule or the upper

pons. The sparing of the forehead, indicating upper motor-neurone facial weakness, favours

the former diagnosis. The term lacune refers to a small deep infarct resulting fromocclusion of a penetrating branch. They occur predominantly in the basal ganglia, internal

capsule, thalamus and pons. Hypertension and diabetes are the main risk factors. Common

lacunar strokes include a pure motor hemiparesis, pure hemisensory loss, ataxichemiparesis and dysarthria clumsy-hand syndrome.

62. A 55-year-old woman presents with a four year history of memory loss and difficultyspeaking. She has also been noted to be abulic and apathetic. On examination she has

difficulty in naming some common objects and is also noted to have some positive grasp

and palmomental reflexes. What is the most likely diagnosis?

Picks disease Your answer

Corticobasal degeneration

Alzheimers disease

Wilsons disease

CreutzfeldtJakob disease

Pick's disease is a type of dementia characterised by a slowly progressive deterioration ofsocial skills and changes in personality, along with impairment of intellect, memory, and

language. It tends to affect individuals at a younger age than Alzheimer's: peak incidence

occurs at 55-65 years. Symptoms may include loss of memory, lack of spontaneity,difficulty in thinking or concentrating, and disturbances of speech, gradual emotional

dullness, loss of moral judgement and progressive dementia. Speech and language

dysfunction may occur early and progress rapdily. These neuropsychological symptomsreflect the pathology of the disease, which typically affects frontal and temporal lobescausing severe atrophy. Microscopically some nerve cells have characteristic abnormalities

(Pick bodies). Although the disease usually affects individuals between the ages of 40 and

60, the age of onset may range from 20 to 80.

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63. An 18-year-old girl presented at the age of 3 years with progressive ataxia.She is now wheelchair-bound. On examination, she is now dysarthric, withbilateral optic atrophy. There is ataxia in both upper limbs. Reflexes in her lowerlimbs are absent with bilateral extensor plantar response. She has absentvibration and impaired joint position in both feet. Bilateral pes cavus is

apparent. ECG shows inverted T waves. Echocardiogram reveals left ventricularhypertrophy. What is the most likely diagnosis?

CharcotMarieTooth disease

Friedreichs ataxia

Your answer

Huntingtons disease

Multiple sclerosis

Vitamin B12 deficiency

Friedreichs ataxia is considered the most common hereditary ataxia. It is anautosomal-recessive disorder with variable phenotype. Onset is usually in earlylife and life expectancy is generally about 35 years. Associated features includeataxia, dysarthria, optic atrophy, hearing impairment, learning difficulties,sensory neuropathy, extensor plantar responses, diabetes and cardiomyopathy.Pes cavus and kyphoscoliosis are also common features. Friedreichs ataxia is oneof the causes of absent reflexes in the lower limbs and extensor plantarresponses.

64. A 27-year-old man presents with a 2-day history of drooling when he tries toeat, difficulty closing his eyes and inability to smile. On examination he hasweakness of eye closure, which is worse on the left, and weakness of othermuscles of facial expression. Facial sensation is normal, as are eye movements,hearing and bulbar function. The remainder of both the neurological and generalexamination is likewise normal. There is a family history of stroke. His previoushealth has been good with no recent illness. What is the most likely diagnosis?

Basilar artery territory stroke

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Myasthenia gravis

Sarcoidosis

Lyme disease

Bilateral Bells palsy

Your answer

The clinical signs are those of bilateral lower motor neurone seventh (facial)nerve palsy lower motor neurone as eye closure is impaired. There are a fewcauses of this including sarcoidosis, Lyme disease and bilateral parotidinfiltration (eg by a lymphoma). However, in this case, with no preceding illnessand no associated signs, the cause is most likely to be Bells palsy, which canoccasionally present bilaterally. The aetiology of this is unclear but some casesmay have a viral origin. The value of steroid therapy and/or antiviral therapy forthis condition is unclear and trials are underway in the UK. This patient will needcareful eye care to avoid corneal abrasion and infection.

65. An elderly man presented with progressive lower limb weakness, particularly with hip

flexion, arreflexia of bilateral knees and ankles with bilateral extensor plantars. He had ahistory of prostate carcinoma. Investigations : raised alkaline phosphatase (ALP),

hypercalcaemia, normal parathyroid hormone (PTH). What is the next most appropriate

investigation?

Magnetic resonance imaging (MRI) of the spine Your answer

Computerised tomography (CT) of spine

Bone density scanMyelogram

Plain films of the spine

This patient has clear evidence of progressive spinal cord compression. Extradural tumours

principally involved in causing spinal cord compression include carcinomas of the

bronchus, breast, prostate and thyroid, lymphoma and malignant melanoma. Plain films

may show degeneration of vertebrae and destruction, but MRI is the best investigation for

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identifying the size and nature of any destructive lesion. Acute management involves use of

corticosteroids to reduce oedema, followed by referral for local radiotherapy.

66. A 62-year-old retired postman awoke with darkened, impaired vision in theupper half of the visual field of his left eye 3 days prior to evaluation. Hedescribed the onset of symptoms as like a shade being pulled down over thevisual picture. He did not complain of eye pain. He had noted headache for 6weeks beforehand, and had consulted his GP on three occasions about this. Hewas told that he was suffering from tension headache, and was prescribed simpleanalgesics. When questioned specifically he reports having pain at each side ofhis jaw when he eats, especially towards the end of the meal. He is hypertensiveand on treatment with a -blocker. He has never smoked. Which of the followingdiagnoses is likely?

Central retinal artery occlusion

Non-arteritic ischaemic optic neuropathy

Migraine

Arteritic ischaemic optic neuropathy

Your answer

Optic neuritis

Altitudinal defects (from the bottom up, or the top down) are common invascular disorders of the optic nerve head. Vascular problems are common onwaking. The preceding headache raises the suspicion of temporal arteritis, thefeared complication of which is arteritic anterior ischaemic optic neuropathy.Jaw claudication as described above is pathognomonic of temporal arteritis.Non-arteritic ischaemic optic neuropathy is common in patients with multiplevascular risk factors, does not cause headache and is not associated with raisedinflammatory markers. Neurological signs precede headache in migraine, whichwould be uncommon in this age group, as would optic neuritis.

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67. A 56-year-old man is referred to you with a 2-year history of ascending lowerlimb numbness and, more recently, foot drop. In the last 6 months he has alsodeveloped numbness in his fingers. He has a distal reduction to pinprick,relatively preserved muscle power, except for ankle dorsiflexion andhyporeflexia in his legs. The GP has already organised nerve conduction studies

and the report is sent along with the patient. Which of the following issuggestive of an axonal neuropathy?

Reduced conduction velocity

Abnormally slow F-response

Delayed P100 latency

Reduced compound muscle action-potential amplitude

Your answer

Conduction block

Reduced conduction velocity is associated with demyelinating neuropathies. Anabnormally slow F-response is associated with very proximal disease, ieradiculopathies. Delayed P100 latency is a feature of performing visual evokedpotentials in those with optic nerve disease. Conduction block is usuallyassociated with certain types of demyelinating neuropathy.

68. A 40-year-old banker came to the emergency room complaining of intenseheadache of two hours duration. The pain is localised around the right eye and isassociated with tearing and redness of the eye. The patient reported he has hadsimilar episodes over the last year. He also admits that these episodes occurredevery day for a few weeks with one to three attacks a day which last for one totwo hours, frequently at night. After six weeks, the attacks stopped. He lost hisjob six months ago and has noticed an increase in the intensity of the pain since.Examination reveals drooping of the eyelid and small pupil on the right side.Which one of the following is the MOST probable diagnosis?

Migraine

Tension-type headache

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Iritis associated headache

Cerebral tumour

Cluster headache

Your answer

The features are classical for cluster headache. Cluster headache afflicts lessthan one in a thousand in the general population. The majority of sufferers aremen. The syndrome is characterised by frequent attacks of intense pain localised

in and around the eye on one side, characteristically accompanied byconjunctival injection and lacrimation in this eye, along with nasal stuffiness onthe same side and sometimes Horners syndrome. All signs and symptoms arestrictly unilateral and occur during attacks lasting between 15 minutes and threehours. The attacks occur from one to eight daily during a period lasting fromsome weeks to months. After a remission of varying duration, the same patternrecurs. In contrast to migraine, during an attack the cluster patient prefers topace about. Attacks frequently occur at night. Recent findings suggest a pivotalrole of the hypothalamus in relation to the pathophysiology. Sumatriptaninjection or oxygen inhalation aborts pain attacks in most patients. The mostfrequently used prophylactic agents are verapamil, lithium and steroids.

69. A 28-year-old woman was diagnosed 3 months ago with schizophrenia. Her

psychiatrist started her on haloperidol. She has now presented to A&E with confusion and

drowsiness. On examination she is pyrexial (39.5 C), disoriented and her blood pressure is200/100 mmHg. She has severe bilateral rigidity. What treatment should be administered?

Aciclovir

Cefuroxime

Dantrolene Your answer

Nifedipine

Propranolol

The most likely diagnosis is neuroleptic malignant syndrome. It is a known complication of

neuroleptic treatment. It presents with an altered mental state, pyrexial, rigidity andautonomic dysfunction. Management consists of muscle relaxants, such as dantrolene, and

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intravenous fluids. Dopamine agonists need to be considered but may exacerbate the

psychiatric condition.

70. A 50-year-old woman presents following a fall. She reports pain and weakness in her

hands for several months, stiff legs and swallowing difficulties and has bilateral wasting of

the small muscles of her hands. Reflexes in the upper limbs are absent. Tonguefasciculations are present and both legs show increased tone, pyramidal weakness and

hyperreflexia with extensor plantars. Pain and temperature sensation is impaired in the

upper limbs. What is the most likely diagnosis?

Multiple sclerosis

Motor neurone disease

Syringobulbia Your answer

SyringomyeliaCervical spondylosis

This patient presents with lower motor neurone (LMN) signs in her arms and upper motorneurone (UMN) signs in her legs, indicating a lesion in the spinal cord. There is also a

dissociated sensory loss of thermal and pain sensation sparing the posterior columns. This

is typical of a syrinx. The LMN lesion of the XIIth nerve proves that the lesion extends intothe brainstem, ie syringobulbia.

71. A 75-year-old woman had a left total hip replacement yesterday. Today she has

developed weakness and numbness of her left foot. On examination there is weakness of all

ankle movements (dorsiflexion, plantarflexion, eversion and inversion) and numbness overthe dorsum of her foot and lateral aspect of her leg. The left ankle jerk is absent. Where is

the most likely site of the lesion?

Left common peroneal nerve

Left femoral nerve

Left obturator nerve

Left sciatic nerve Your answer

Left tibial nerve

Sciatic nerve damage is a common complication of total hip replacement. It causes globalweakness of the ankle due to the involvement of both its branches (common peroneal and

tibial nerve). The ankle jerk is absent due to the involvement of the tibial nerve. Sensory

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loss is variable but most commonly occurs around the dorsum of the foot and lateral aspect

of the leg. Common peroneal nerve palsy causes weakness of ankle dorsiflexion and

eversion. Tibial nerve palsy causes weakness of ankle plantarflexion and inversion.Femoral nerve palsy causes weakness of hip flexion and knee extension. Obturator nerve

palsy causes weakness of hip adduction.

72. A 30-year-old pregnant woman presents with malaise, headache, diarrhoea and neck

stiffness. On examination she has a temperature of 38.2 C and mild meningism. Her

general practitioner had prescribed oral antibiotics earlier that day. After a CT brain scan,which was normal, CSF examination shows a slightly elevated protein of 0.6 g/dl, a white

cell count of 200/mm3 (mostly lymphocytes), CSF glucose level of 3.3 mmol/l and blood

glucose of 4 mmol/l. Which of the following is the most likely diagnosis?

Viral meningitis due to echovirus Your answerPartially treated streptococcal meningitis

Listeria meningitis

Cryptococcal meningitis

Tuberculous meningitis

The pattern of slightly elevated protein, lymphocytosis and normal CSF/blood glucose ratiois most suggestive of viral meningitis. The other aetiologies typically cause a low

(bacterial) or very low (tuberculous, fungal) CSF/blood glucose ratio. Partial treatment of

bacterial meningitis may give a lymphocytic rather than a polymorph infiltrate, but theglucose ratio still tends to be low. Listeria meningitis is more common in pregnant women.

73. A 30-year-old pregnant woman presents with malaise, headache, diarrhoea and neck

stiffness. On examination she has a temperature of 38.2 C and mild meningism. Hergeneral practitioner had prescribed oral antibiotics earlier that day. After a CT brain scan,

which was normal, CSF examination shows a slightly elevated protein of 0.6 g/dl, a white

cell count of 200/mm3 (mostly lymphocytes), CSF glucose level of 3.3 mmol/l and bloodglucose of 4 mmol/l. Which of the following is the most likely diagnosis?

Viral meningitis due to echovirus Your answerPartially treated streptococcal meningitis

Listeria meningitis

Cryptococcal meningitis


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The pattern of slightly elevated protein, lymphocytosis and normal CSF/blood glucose ratio

is most suggestive of viral meningitis. The other aetiologies typically cause a low

(bacterial) or very low (tuberculous, fungal) CSF/blood glucose ratio. Partial treatment ofbacterial meningitis may give a lymphocytic rather than a polymorph infiltrate, but the

glucose ratio still tends to be low. Listeria meningitis is more common in pregnant women.

74. Which one of the following is MOST suggestive of a lesion of the sciatic nerve?Absent knee tendon jerk

Foot drop

Your answer

Inability to flex the hip

Decreased sensation on anterior thigh and medial leg

Intervertebral disc prolapse at L2/L3 levels

The sciatic nerve originates in the sacral plexus mainly from the spinal segmentL5 to S2. It supplies muscles that cause extension of the thigh and flexion of the

leg. It divides into two major branches, the tibial nerve and the commonperoneal nerve, which are responsible for all foot movements. Anterior thigh andmedial leg sensory loss is typical of femoral nerve lesion. The femoral nervemediates flexion of the hip. Sciatica is pain in the distribution of the sciaticnerve regardless of the aetiology.

75. A 37-year-old woman is referred to your clinic with two episodes of sudden-onset limb paralysis at night-time over the last 4 months. She describes waking

shortly after falling asleep and being aware of an inability to move her limbs orto speak, associated with a feeling of suffocation lasting for about a minute. Herfather recently died from an intracerebral haemorrhage during sleep. She has noother symptoms other than daytime sleepiness, which she puts down to lookingafter her two young children. There are no abnormalities on examination. Whatis the most likely diagnosis?

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Depression and anxiety

Nocturnal seizures

Brainstem transient ischaemic attack

Narcolepsy

Your answer

Cervical disc prolapse

This woman is describing sleep paralysis. It reflects the atonia of REM sleep.

Awareness is preserved during the attack, which heightens the sense of terrorand anxiety, especially if a feeling of suffocation is present (although therespiratory muscles are only ever mildly affected in comparison to the limbs).Sleep paralysis may be isolated or occur in the context of narcolepsy, in which itaffects 1545% of patients.

76. Interferon is a licensed treatment in the United Kingdom for which of the followingneurological conditions?

First episode of optic neuritis

Relapsing remitting multiple sclerosis Your answer

Primary progressive multiple sclerosis



In the UK, -interferon is currently a licensed disease-modifying therapy for relapsing

remitting multiple sclerosis (MS) and secondary progressive MS in adults. Clinical trialshave shown a modest reduction in relapse frequency and a reduction in MRI lesion load,

but no impact on the final level of disability. Relapsing remitting disease, for the purposes

of interferon eligibility, is defined as two or more clinically significant relapses in the 2years preceding prescription. The patient must be able to walk, with or without a walking

aid, a distance of at least 10 metres. The treatment is given as either subcutaneous injection

three times per week or intramuscular injection once weekly. Adverse effects include local

inflammatory reactions at injection sites, flu-like symptoms and depression. Some patientsdevelop autoantibodies to interferon that limit its efficacy. At present, treatment with

interferon is not recommended for clinically isolated syndromes (such as optic neuritis) or

primary progressive MS, due to lack of evidence.

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77. Which of the following is a good first-choice oral treatment for primarygeneralised epilepsy in a 20-year-old man?

Topiramate

Valproate

Your answer

Carbamazepine

Phenytoin

Phenobarbital

There is insufficient evidence to support the use of first-line treatment withtopiramate for any seizure type. Nowadays, phenobarbital should not beinitiated for epilepsy treatment due to its unacceptable side-effects whencompared with more modern alternatives. Phenytoin is a useful drug acutely, butagain has troublesome problems associated with longer term use, and would

generally be avoided in this age group. The first-choice treatments for primarygeneralised epilepsy are lamotrigine and valproate. Carbamazepine, valproate,lamotrigine and oxcarbazepine are all reasonable first choices for partial(localisation-related) epilepsy.

78. A 54-year-old man presents with slowly progressive facial weakness and loss of taste.

He has also noticed that he is more sensitive to loud sounds than usual. On examination

there is weakness of the facial muscles on the right (including the forehead), a small patch

of altered sensation on the right cheek, and decreased taste sensation. Where is the mostlikely location of this lesion?

Cerebellopontine angle Your answer

Parotid gland

Internal auditory canal

Petrous temporal bone

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Stylomastoid foramen

The facial nerve emerges from the brainstem with the nervus intermedius, which gains its

name from its position as it courses across the cerebellopontine angle (CPA) between thefacial nerve and the vestibulocochlear nerves (ie cranial nerves VII and VIII). Cranial nerve

V (trigeminal) also lies in the cerebellopontine angle. The close anatomical associationbetween these structures at the level of the cerebellopontine angle may result in

disturbances in tearing, taste, salivary gland flow, hearing, balance and facial sensation andfacial weakness as the result of lesions at this level.

79. A 65-year-old man presents with sudden-onset visual disturbance. He denies any othersymptoms. He is known to be a heavy smoker and hypertensive. On examination he has a

right incongruous homonymous hemianopia. Where is the most likely site of the lesion?

Left occipital lobe

Left optic nerve

Left optic tract Your answer

Left temporal lobe

Optic chiasma

Incongruous homonymous hemianopia is a feature of an optic tract lesion. An optic chiasm

lesion causes bitemporal hemianopia. A temporal lobe lesion causes a superiorhomonymous quadrantanopia. An occipital lobe lesion causes a congruous homonymous

hemianopia. An optic nerve lesion causes a scotoma.

80. A 40-year-old man describes intermittent unilateral pain above and behindhis left eye, which has woken him from sleep every night for the previous 7 days.The pain is described as severe and stabbing, lasting about 30 minutes andmaking him restless and agitated. It is associated with tearing from his left eyeand nasal stuffiness. When he looked in the mirror during attacks he had notedhis left eyelid drooping. He remembers he had experienced similar symptoms forabout a month last year, but that they had resolved spontaneously and he had

not sought medical attention. Physical examination is normal. He is a lifelongsmoker. What is the diagnosis?

Migraine

Cluster headache Your answer

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Temporal arteritis

Paroxysmal hemicrania

Trigeminal neuralgia

The symptoms are very suggestive of cluster headache, the most common of agroup of headache disorders known as the trigeminal autonomic cephalgias.This commonly occurs in middle-aged men, and occurs classically in clusterslasting weeks to months. Paroxysmal hemicrania is another type of trigeminal

autonomic cephalgia, but is much rarer, tends to occur multiple timesthroughout the day and is commoner in women. Trigeminal neuralgia issometimes mistaken for cluster headache, but the attacks are much shorter andautonomic activation is rare. Sometimes cluster headache can have migrainousfeatures, but migraine lacks the specific features stated in the question.Temporal arteritis would be unheard of in this age group. Verapamil was foundto be more effective than placebo in a randomised controlled trial of treatmentfor cluster headache. Prednisolone is also sometimes used for treatment, but itsuse is not supported by randomised evidence.

81. A 30-year-old pregnant woman presents with a weak grip and tingling of herright hand. She complains of a dull aching pain in her forearm, which is madeworse by carrying a shopping bag. On examination you find weakness of the rightabductor pollicis brevis and mild weakness of thumb flexion. Finger abductionand adduction appear to be within normal limits. There is sensory loss to pinprickmainly affecting the right thumb and index finger. Phalens sign is positive. Theleft-hand sensorimotor examination is normal. The deep tendon reflexes aresymmetrical. What is the most likely cause of her symptoms?

Compression of the right ulnar nerve at the elbow

Right C8 nerve root irritation

Right brachial plexopathy

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Compression of the right median nerve in the forearm

Compression of the right median nerve in the carpal tunnel

Your answer

Her symptoms suggest carpal tunnel syndrome. Pregnancy is a risk factor for this,as are other conditions that promote fluid retention or thickening of thesubcutaneous tissues (eg hypothyroidism), factors that alter the configuration ofthe wrist structures (eg osteoarthritis or rheumatoid arthritis) and conditionsthat predispose to neuropathy (eg diabetes, hereditary tendency to pressurepalsy). Although the median nerve supplies the lateral two lumbricals, opponenspollicis, abductor pollicis brevis and flexor pollicis longus (mnemonic LOAF), the

pattern of weakness may be incomplete. This also applies to the pattern ofsensory loss. Phalens sign involves placing pressure over the carpal tunnel withthe wrist flexed it is considered positive if this reproduces the patientssymptoms (although, of course, it is neither 100% specific nor sensitive). Nerveconduction studies would confirm the diagnosis.

82. A 19-year-old college student is noted to seem confused by her flatmate. Shehas been complaining of a diffuse headache and general malaise for the past 24

hours. On examination she has a temperature of 38 C. She is restless and mildlydysphasic. The remainder of the general and neurological examination is normal.CT brain scan shows hypodensity in both temporal lobes. Cerebrospinal fluid(CSF) examination shows a white cell count of 16/mm3 (lymphocytes), a slightlyraised protein concentration of 0.75 g/l and a normal CSF/blood glucose ratio.Which would be the most appropriate early management pending furtherdiagnostic information?

Intravenous fluids, broad-spectrum antibiotics and prophylacticanticonvulsants pending further CSF analysis

Intravenous fluids, aciclovir and prophylactic anticonvulsants

Intravenous fluids, aciclovir and broad-spectrum antibiotics

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Intravenous fluids, aciclovir and corticosteroids

Intravenous fluids and iv aciclovir

Youranswer

The short history and presence of dysphasia are both characteristic of herpessimplex encephalitis (HSE), which is caused by herpes simplex virus (HSV): HSV-1in 90% of cases, and in the remainder HSV-2, mainly neonates andimmunocompromised patients. The presence of temporal lobe changes (focaloedema) on neuroimaging is typical of HSV (though note that CT may be normalin the early stages MRI is much more sensitive). Diagnosis may be confirmed byCSF polymerase chain reaction (PCR) for HSV (specificity > 95%; sensitivity shouldbe 95% in an experienced laboratory; though there may be false-negatives veryearly or very late). Infective encephalitis must be distinguished from other

causes of encephalopathy (eg metabolic, ischaemic, toxic, etc). Many otherviruses cause encephalitis (varicella, EpsteinBarr virus (EBV), influenza, etc).However, HSE is the most important to consider because of the existence of aneffective antiviral agent; aciclovir treatment should always be given in adequatedoses for any possible case, and continued for a full course of at least 10 daysunless an alternative diagnosis is found. Steroids may be given if there are signsof significant cerebral swelling, although the evidence base is limited.Anticonvulsants are used to treat symptomatic focal and generalised seizures,though are not routinely given prophylactically. This patient has a good prognosisbased on her young age, short duration of illness and absence of coma.

83. A patient with a history of left optic neuritis 1 year ago, and transient spasticparaplegia (lasting 6 weeks) 6 months ago is seen in clinic. His T2 -weighted MRIscan shows multiple periventricular hyperintensities and three similar lesions inthe posterior fossa, with one lesion in the corpus callosum. Lumbar punctureshows oligoclonal bands. Only a relative afferent pupillary defect on the left isevident on examination. So far, he has not been treated for his problems.

What treatment should he be offered?

Plasmapheresis

Intravenous methylprednisolone

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Oral prednisolone

-Interferon

Your answer

Azathioprine

This patient has multiple sclerosis as evidenced by 2 spearted episodes ofneurological dysfunction, a typical MRI appearance and characteristicimmunological changes in the CSF. This patient fulfils the currently accepted UKcriteria for the prescription of -interferon. Randomised evidence now existsthat interferon given after the first attack reduces the likelihood of a secondattack within 2 years, and numerous randomised trials have shown a reduction ofthe relapse rate (by a one-third to one-half) in patients with definite multiple

sclerosis (like this patient). Evidence on the use of azathioprine in this situationis intriguing, but it is not accepted as standard therapy. Oral or ivmethylprednisolone shortens the relapse duration, but is not used to preventrelapse. Oral prednisolone has no role in MS treatment. Plasmapheresis is used asa rescue therapy if steroids fail to help in cases of severe relapse.

84. A 28-year-old man presents to the A&E department complaining of a sudden-onset occipital headache associated with vomiting. His symptoms started 2 hourspreviously and are continuing. He has a previous history of infrequent migraine

without aura, which also causes nausea but not vomiting. His current headache ismuch more severe than his usual migraine, which has not previously startedsuddenly. Examination is unremarkable. In particular there is no neck stiffness orphotophobia. Which of the following management options would be the mostappropriate?

A diagnosis of migraine and suggestion that he be referred to aneurologist for a further opinion

Immediate lumbar puncture, to exclude meningitis andsubarachnoid haemorrhage

CT brain scan, followed by discharge if normal

CT brain scan, followed by lumbar puncture if CT normal

Your

answer

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CT brain scan, followed by angiography

It is certainly likely that this represents recurrent migraine, but there are

concerning features that make this a diagnosis of exclusion. The sudden onsetand greater severity than his usual migraine requires that subarachnoidhaemorrhage be excluded. CT brain scan is normal in up to 30% of patients withsubarachnoid haemorrhage. However, a positive CT brain scan is diagnostic. Ifthe CT is negative for blood, a lumbar puncture is needed to look for red bloodcells. The absence of red blood cells or of xanthochromia excludes subarachnoidhaemorrhage. Because bilirubin and oxyhaemoglobin take 12 hours to appear inthe cerebrospinal fluid following a bleed, lumbar puncture should usually bedelayed until more than 12 hours from the onset of symptoms.

85. A 60-year-old man with chronic atrial fibrillation is on warfarin. He has had two

unprovoked tonicclonic seizures that were witnessed by his wife. CT brain shows only

some diffuse ischaemic changes in the white matter. The GP wants to start him on anantiepileptic drug. Which of the following antiepileptic drugs does not have important drug

interactions with warfarin?

Carbamazepine

Lamotrigine Your answer

Phenytoin

PhenobarbitalPrimidone

Drug interactions should always be considered before introducing antiepileptic drugs.Phenytoin, carbamazepine, primidone and phenobarbital are liver enzyme inducers.

Sodium valproate is a liver enzyme inhibitor. Lamotrigine has no effect on liver enzymes

and is the treatment of choice for the above patient.

86. A 26-year-old woman presents to her GP with mood swings and depression. She is

prescribed Prozac, but returns a few months later with pains and numbness in both legs.

Her antidepressant is changed but her mood worsens and she starts to have problems withher memory and motivation. She is referred to your clinic where, in addition to mild

cognitive impairment, you notice slight increased tone in her limbs and occasional

myoclonic jerks. Her MRI scan reveals thalamic hyperintensity on T2-weighted images, buther EEG is normal. What is the most likely diagnosis?

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Corticobasal degeneration

Progressive severe depression

Juvenile myoclonic epilepsy

New-variant CreutzfeldtJakob disease Your answer

Wilsons disease

This patient is too young to develop corticobasal degeneration. Continuing depression is

also unlikely in view of the abnormal neurological signs. She does have myoclonus but notseizures. Wilsons disease may present with psychiatric symptoms, as well as disorders of

movement, particularly tremor, but new-variant CreutzfeldtJakob disease is more likely.

New-variant CJD commonly presents with psychiatric symptoms in young adults, followed

by non-specific painful sensory symptoms, most often in the lower limbs. Cognitiveimpairment, pyramidal signs, myoclonus and primitive reflexes may then develop. Mean

disease duration is approximately 14 months. MRI commonly shows a high signal on T2-

weighted images in the pulvinar (posterior aspect of thalamus). EEG is often normal,compared to sporadic CJD in which triphasic waves are observed. Diagnosis is made

antemortem by brain biopsy or, more commonly, by tonsillar biopsy.

87. A 30-year-old patient presents with sudden monocular visual loss on the rightassociated with pain behind the eye and alteration of colour vision. Examinationthe following day reveals a relative afferent pupillary defect in the right eye.Which of the following diagnoses is most likely?

Cerebral infarction

Optic neuritis

Your answer

Optic nerve glioma

Migraine

Temporal arteritis

The history is suggestive of an acute optic nerve lesion, the most likely diagnosisin this age group being optic neuritis. Hemianopic visual disturbance is thecommon type seen in cerebral infarction. Optic nerve glioma usually presentsover a longer period, often with proptosis. Migraine can cause monocular

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disturbances, but these are usually in the context of headache, and aretransient. Temporal arteritis does not occur in patients < 50 years of age.

88. You are referred a 57-year-old woman with a marked change in personalityover the last 5 years. She has become increasingly sexually flirtatious withinappropriate behaviour in social situations. Impairment of abstract thinking,memory and planning has become increasingly obvious. However, the ability toperform arithmetic tasks is relatively preserved. Speech output is diminished.There is no motor impairment. Physical examination is unremarkable except forthe presence of grasp reflexes. What is the most likely diagnosis?

Normal-pressure hydrocephalus

Frontotemporal dementia

Your answer

Alzheimers disease

Frontal lobe meningioma

Multiple sclerosis

The prominent early feature of Alzheimers disease is short-term memoryimpairment, which is not really a feature of this womans presentation.Frontotemporal dementia, however, is associated with early personality change,although the exact nature of this change varies. Patients may present withdisinhibition, or alternatively with apathy, loss of initiative and drive withimpairment of frontal executive tasks. Speech gradually deteriorates and manypatients develop aphasia. Motor skills are well preserved. Neuroimaging might

reveal isolated frontotemporal lobar atrophy. Normal-pressure hydrocephaluswould be likely to have caused gait disturbance by now. Multiple sclerosis cancause frontal signs, but is likely to have presented with visual or spinalsymptoms. A frontal lobe meningioma might be associated with features ofraised intracranial pressure, and possibly anosmia.

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89. A 69-year-old woman has become progressively weaker over a 3-month period. Her

symptoms fluctuate diurnally, being worse when she awakes. Although her greatest

difficulty is climbing stairs, she also has difficulty raising her arms. The patient denies thepresence of pain, numbness, diplopia, difficulty swallowing and bladder abnormalities, but

has noted dryness of her mouth. She has been a heavy smoker for years. On examination

there are no abnormalities of the cranial nerve, cerebellar or sensory function. Herweakness is diffuse and mild, with strength increasing with exertion. Reflexes are absent

but are obtainable after exertion. Plantar responses are flexor. What is the most likely

diagnosis?

LambertEaton myasthenic syndrome Your answer


Multiple sclerosis

Myasthenia gravis

Polymyositis

LambertEaton myasthenic syndrome is an antibody-mediated syndrome (anti-voltage-gated calcium antibodies) often associated with small-cell lung carcinoma or with

autoimmune diseases. It starts insidiously, often several years before a tumour becomes

evident. Patients present with proximal weakness, which is typically worse in the morningand decreases with exertion. There are accompanying autonomic features, especially dry

mouth. It is a presynaptic neuromuscular disease with inhibition of acetylcholine release

from the presynaptic junction.

90. A 13-year-old boy presents to A&E one morning after an episode ofunconsciousness, a convincing history of which was obtained from his mother.She had witnessed his eyes rolling upwards, a fall to the floor, jerking of all four

limbs and his lips going blue. He had been incontinent. He had been assessed in ageneral medical clinic 3 years earlier complaining of sudden brief bilateraljerks occurring largely in the morning after waking. Neurological examination isnormal. What is the most likely diagnosis?

Complex partial epilepsy

Juvenile myoclonic epilepsyYour answer

Hypoxic myoclonus

Wilsons disease

Pseudoseizures

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This is a classic history for juvenile myoclonic epilepsy, one of the most common

primary generalised epilepsy syndromes. Juvenile myoclonic epilepsy typicallyappears in the second decade. It is characterised by myoclonic seizures,associated at times with generalised tonicclonic seizures (as in this case) orabsence seizures. The cardinal seizure type is that of myoclonic jerkscharacterised by sudden, brief, bilaterally symmetrical and synchronous musclecontractions. Consciousness remains unimpaired during myoclonic seizures, evenif they occur in series or in myoclonic status epilepticus. Generalised tonicclonicseizures appear after the onset of myoclonic seizures in the majority of cases.Both the myoclonic seizures and generalised tonicclonic seizures have a specialcircadian pattern, in that they occur almost exclusively on or soon afterawakening, either from all-night sleep or from a nap. The condition is treated

with valproate or lamotrigine. Complex partial (localisation related) epilepsy isuncommon in this age group, and not usually associated with myoclonus. There isno history to suggest hypoxia, or the types of movement disorder commonlyassociated with Wilsons disease. This history does not sound non-organic.

91. Which of the following findings best supports a diagnosis of multiple sclerosis?

CSF protein of > 1.2 g/dl

Intrathecal synthesis of IgG Your answerLow CSF : serum glucose ratio

Multiple grey matter lesions on MRI

Normal visual evoked potentials

Multiple sclerosis (MS) is the most common demyelinating disease of the central nervoussystem. It most often affects young adults between the ages of 20 and 50, with a 2 : 1

female/male ratio. Cerebrospinal fluid is typically examined for the presence of increased

intrathecal synthesis of IgG and also for the presence of a discrete set of proteins, called

oligoclonal bands, that appear on gel electrophoresis. Note that oligoclonal bands andincreased rates of IgG synthesis are found in about 90% of patients with definite multiple

sclerosis, but may also be present in other diseases.

92. A 59-year-old man has shown a change in his mood and personality over a 9-month

period. He has subsequently developed difficulty with memory and concentration, and then

progressive fidgety movements of his limbs and facial musculature. By the time of

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medical assessment he has frank choreiform movements and a Mini-Mental State

Examination of 21/30. Other examination is normal. He was adopted and therefore no

information on his family history is available. He has three adult children (aged 27, 30 and33 years) who are asymptomatic.

Based on the likely clinical diagnosis, which of the following genetic patterns of thiscondition is most likely?

Autosomal-dominant inheritance with anticipation Your answer

Autosomal-dominant with variable penetrance

Autosomal-recessive inheritance

X-linked inheritance

Mitochondrial disorder

The clinical scenario is that of Huntingtons disease (also called Huntingtons chorea). Thisis characterised by slowly progressive subcortical dementia with early personality changes

(irritability, disinhibition) followed by the onset of a progressive choreiform movementdisorder. It has an autosomal-dominant mode of inheritance and is one of the trinucleotide-repeat disorders (CAG repeat, on the huntingtin gene, chromosome 4p16.3). Increasing

repeat length in offspring gives rise to the phenomenon of anticipation (clinical onset at an

earlier age in subsequent generations). Referral for genetic counselling prior to genetictesting is therefore an important step if the disease is suspected. Haloperidol and

tetrabenazine can be used for symptomatic treatment of the chorea but the condition itself

inexorably progresses, with terminal stages being reached after 1015 years in most

patients.

93. A 78-year-old man presents with transient left arm weakness and collapse. He recovers

within a period of 24 h and has a carotid ultrasound scan. This reveals a 55% stenosisaffecting the right internal carotid artery and a 90% stenosis affecting the left internal

carotid artery. Other risk factors of note include smoking of 30 cigarettes per day, which he

refuses to stop and hypertension. Blood pressure in the clinic is 145/80 mmHg, he is insinus rhythm and cholesterol is 5.1 mmol/l. Which of the following is the most appropriate

management to reduce risk of a further stroke affecting the same territory?

Aspirin therapy

Aspirin and dipyridamole MR therapy Your answer

Right carotid endarterectomy

Left carotid endarterectomy

Start statin therapy

The right carotid stenosis is not severe enough to justify surgical intervention and the

symptoms are not suggestive of left internal carotid artery stroke; carotid endarterectomy is

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therefore excluded as an option. While aspirin alone reduces stroke risk, the combination of

aspirin and dipyridamole MR has shown to be most effective in reducing the risk of

secondary cerebral infarction. Lowering total cholesterol further from this level is unlikelyto impact on stroke risk to the same extent as combined antiplatelet therapy, although it

will impact on future risk of events connected with ischaemic heart disease.

94. A 53-year-old hospital porter is referred to you with aching and fatigue in hislegs. His symptoms started 2 months ago with difficulty walking up stairs. Ondirect questioning, he says it takes him increased effort to get dressed. He alsocomplains of a dry mouth, but there are no speech or swallowing difficulties. Onsystematic questioning, he has a long history of mild breathlessness on exertionand a chronic cough that he attributes to his heavy smoking. However, over thelast 2 weeks he has noticed blood streaks in his sputum. On examination he has apredominantly proximal weakness, more so on the left. Deep tendon reflexes are

depressed but improve with repeated muscle contraction. There are no sensorychanges or extraocular muscle abnormalities. What is the most likely diagnosis?

Paraneoplastic neuropathy

Vasculitic neuropathy

Metabolic myopathy

Tuberculous myeloradiculopathy

LambertEaton syndrome

Your answer

The LambertEaton syndrome is a myasthenic syndrome with a well-recognisedassociation with malignancy (5060% of cases), most commonly small-cell lungcarcinoma. It is characterised by proximal muscle weakness, particularly in thelegs, which improves for a short while after exercise (postexercise facilitation).Extraocular and bulbar muscles are usually spared. In addition, tendon reflexesare usually depressed or absent and are characteristically enhanced withrepeated muscle contraction. Autonomic disturbances (eg dry mouth andimpotence, as in the above case) are common. High-frequency repetitive nervestimulation results in an incremental response of the compound muscle actionpotential because accumulation of presynaptic calcium facilitates acetylcholinerelease. The haemoptysis could also have been due to tuberculosis or a systemic

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vasculitis, either of which could be associated with neurological features. Hissigns could be consistent with a patchy mononeuritis multiplex, but LambertEaton syndrome is the most likely.

95. A 73-year-old man presents to his GP complaining of a headache and blurringof vision in his right eye lasting for 5 minutes. Two days later he developedsudden-onset, left-sided weakness affecting both upper and lower limbs andlasting for 30 minutes. His past medical history includes mild hypertension. He isa non-smoker. There are no neurological abnormalities. His blood pressure is160/90 mmHg, heart rate 88 bpm. There are no audible carotid bruits. Which ofthe following is the most likely diagnosis?

Migraine equivalent

Transient ischaemic attack secondary to carotid artery disease

Your answer

Transient ischaemic attack secondary to cardioembolism

Transient ischaemic attack secondary to small-vessel disease

Transient ischaemic attack secondary to giant-cell arteritis

The transient monocular visual disturbance is consistent with the description ofamaurosis fugax. Characteristically, patients describe a curtain coming downover their vision, but are just as likely to describe blurring or greying. Anysuggestion that this disturbance is altitudinal (ie has a horizontal cut-off) makesthe possibility that this is a vascular disturbance of the retinal vessels much

greater. The subsequent left-sided weakness is suggestive of a right cerebralhemisphere transient ischaemic attack. The combination of these two stronglypoints towards a right internal carotid artery stenosis as the source of plateletemboli. Other sources of emboli should be considered. However, cardioembolismis unlikely in the absence of either atrial fibrillation or previous cardiacsymptoms (of either cardiac ischaemia or cardiac failure). Transient occlusion ofa small penetrating vessel could cause the hemiparesis, but not the amaurosisfugax. Giant-cell arteritis should always be considered in patients over 60 years

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of age, but there are no specific features to support the diagnosis, although it isalways worthwhile checking the ESR. Lastly, migraine equivalents (aura-likesymptoms without headache) can provide diagnostic difficulties, but there is nosuggestion of the characteristic slow spread of symptoms in this patient.

96. A 58-year-old farmer from Northern Ireland presents with a progressiveneurological illness over 3 weeks. He complains of double vision and has someslurring of speech and unsteadiness on his feet, as well as malaise and tiredness.

He also admits to having lost 6.25 kg (1 stone) in weight over a 6-week period.On examination he has a bilateral VIth nerve palsy, facial asymmetry and markeddysarthria. The deep tendon reflexes are sluggish throughout and the plantarresponses equivocal. He has lymphadenopathy of the axilla and groins, and atemperature of 37.6 C. Chest X-ray is normal. MRI of the brain shows diffusemeningeal enhancement. Lumbar puncture shows an elevated protein of 1.2 g/l,an elevated white cell count of 87/mm3 (mostly lymphocytes) and a lowCSF/blood glucose ratio (CSF glucose 0.2 mmol/l, blood glucose 6.7 mmol/l).Which cause for his symptoms should be considered most likely pending furtherinvestigations?

Brucellosis

Sarcoidosis

Malignant meningitis

Tuberculous (TB) meningitis

Your answer

Lyme disease

Meningeal infection with Mycobacterium tuberculosis is notoriously difficult todiagnose and treat. The clues in this case are a typical clinical presentation and

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CSF picture, with a very low CSF glucose to blood glucose ratio. Repeated CSFsamples (often in largish volumes) are usually required for prolonged culture. Itis not uncommon to need to treat a patient for TB meningitis based onreasonable clinical suspicion, without laboratory confirmation. However, such adecision is clearly a difficult one given the length of the treatment required and

the potential toxicity of the drugs involved. Brucellosis is a reasonableconsideration given his occupation but the CSF glucose would be expected to benormal or only very slightly reduced, as it would in sarcoid and Lyme disease.Malignant meningitis would also be unlikely to produce a glucose level as low asthis; in any case treatment for TB would be given until definite evidence of analternative diagnosis was produced.

97. A 75-year-old man is noted by his family to be increasingly confused. His mental statefluctuates considerably and he appears to be hallucinating at times. His daughter

commented that his gait has become abnormal over the preceding months, and that his GP

had tried a tablet for the nocturnal confusion that apparently made him much worse. On

examination he has a Mini-Mental score of 20/30, has generally increased tone anddifficulty in performing rapidly alternating movements. His tendon reflexes are slightly

brisk but the plantar responses are normal and no primitive reflexes are detected. Which ofthe following is the most likely explanation of this clinical scenario?

Vascular dementia

Senile dementia of Alzheimers type

CreutzfeldtJakob disease

Temporal variant of frontotemporal dementia

Dementia with Lewy bodies Your answer

The associated extrapyramidal features and variable psychiatric features with complexhallucinations are typical of dementia with Lewy bodies (DLB). This condition is now

recognised to be the second most common cause of neurodegenerative disease in olderpeople (after senile dementia of Alzheimers type, SDAT). Typically, standard

antipsychotic drugs such as haloperidol worsen DLB, and indeed potentially fatal

neuroleptic sensitivity reactions can occur. It is one of a group of conditions caused byabnormal aggregation of the synaptic protein a-synuclein. The cholinesterase inhibitor

drugs, initially developed for SDAT, look promising for the treatment of DLB.

98. A 45-year old man complains of numbness of his left thigh. He feels hisbalance may be poorer than usual but is not sure of this. He has put on 6.25 kg (1stone) in weight over the past 6 months. He has mild, chronic, low back pain.There is a family history of a neurological condition, in that his fathers brotheris said to have multiple sclerosis. On examination he is overweight. He has somereduction of pinprick sensation over the anterior part of his thigh on the left,

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with no motor signs. Tendon reflexes are normal. There are no cerebellar signs.What is the most likely diagnosis?

Sporadic multiple sclerosis (MS)

Familial multiple sclerosis

Lumbar canal stenosis with nerve root entrapment

Meralgia paraesthetica

Your answer

Inflammatory disease of the lumbosacral plexus

Meralgia paraesthetica is a symptom complex that includes numbness,paraesthesias and pain in the anterolateral thigh, which may result from eitheran entrapment neuropathy or a neuroma of the lateral femoral cutaneous nerve(LFCN). Obesity or weight gain is a risk factor for this as it predisposes to nervecompression; individual variation in anatomy around the region of the anteriorsuperior iliac spine may also contribute. The diagnosis is mainly clinical(including the absence of other signs) and electrophysiological studies can helpto exclude other more generalised neuroradiculopathy. The family history of MSin this case is a red herring; it should be remembered that even if there are noother clinical features to suggest a particular neurological condition, the patientis likely to be anxious about the possibility and clinicians should be aware of this,paying special attention to answering a patients spoken and unspoken concerns.

99. A 76-year-old man presents with a 1-month history of lower back pain. Morerecently he has developed weakness of and paraesthesias in his legs. On the dayof admission, he was unable to stand, and developed difficulty in initiatingmicturition. He is a long-standing, insulin-dependent diabetic and his diabeticcontrol poor. His most recent Hb A1c is 8.6%. On examination he is mildly febrile.He has increased tone and pyramidal distribution weakness in his lower limbs.Lower limb reflexes are brisk with extensor plantars. Sensory examination isvariable. Straight-leg raising produces pain. What is the most likely diagnosis?

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Spinal epidural abscess

Your answer

Anterior spinal artery thrombosis

Spinal arteriovenous malformation


Prolapsed L3/4 intervertebral disc

This man clearly has an upper motor neurone lesion, most likely in the spinal

cord. Vascular events in the cord (thrombosis or rupture of AVM) are usuallysudden and catastrophic. A prolapsed disc at L3/4 is possible, but the history offever in the context of diabetes should always raise the possibility of infection,eg spinal epidural abscess. He will require an MRI of the spine, blood culturesand probably surgical decompression and intravenous antibiotics.

100. A 49-year-old woman was placed on warfarin therapy because of pulmonary

embolism. While she has been on therapy, she has developed pain, numbness andparaesthesias from her left anterior thigh extending to her medial leg, plus weakness of

knee extension and hip flexion. The left knee jerk is absent. What is the most likely site of

the lesion?

Left common peroneal nerve

Left femoral nerve Your answer

Left obturator nerve

Left sciatic nerve

Left superior gluteal nerve

All abnormalities are indicative of a left femoral neuropathy. The patient has weakness of

knee extension (quadriceps) and hip flexion (iliopsoas). She has decreased sensation in her

anterior thigh (anterior femoral cutaneous nerve) and medial distal leg (saphenous nerve).

A patient on anticoagulation therapy can suffer a retroperitoneal haemorrhage, which candamage the femoral nerve.

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