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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?
GuillainBarr syndrome
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
Tuberculous 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
Motor neurone disease
GuillainBarr syndrome
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
Motor neurone disease
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
Tuberculous meningitis
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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