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  • Question 1 - A 74-year-old retired judge who is known to have Alzheimer’s disease is examined...

    Incorrect

    • A 74-year-old retired judge who is known to have Alzheimer’s disease is examined in clinic. His latest Mini Mental State Examination (MMSE) score is 18 out of 30. Which of the following is the most appropriate treatment option?

      Your Answer: Supportive care + memantine

      Correct Answer: Supportive care + donepezil

      Explanation:

      NICE now recommends the three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer’s disease. Memantine is reserved for patients with moderate to severe Alzheimer’s.

    • This question is part of the following fields:

      • Neurology
      30.7
      Seconds
  • Question 2 - A 22-year-old male has had complex tics since childhood. He repeatedly bends his...

    Correct

    • A 22-year-old male has had complex tics since childhood. He repeatedly bends his knees and rubs his nose. He is prone to loud vocalisations, sometimes including swear-words. A diagnosis of Gilles de la Tourette syndrome has been made. Which of the following is the best treatment option?

      Your Answer: Risperidone

      Explanation:

      Gilles de la Tourette syndrome is the most severe and rare of the tic syndromes, consisting of multiple tics involving both motor actions and vocalisation. Onset is usually in childhood. Symptoms include utterance of obscenities (coprolalia); echolalia (repetition of another person’s spoken words) and palilalia (involuntary repetition of words, phrases, or sentences).
      The underlying cause is unknown, with no particular imaging or standard histopathological abnormalities having been identified. The EEG shows non-specific abnormalities in about half of patients. However, more recent immunocytochemical studies have suggested altered dopamine uptake in the striatal system.
      Risperidone is an effective therapeutic option without the effects associated with chlorpromazine and haloperidol.

    • This question is part of the following fields:

      • Neurology
      97.8
      Seconds
  • Question 3 - A 71 year-old dentist is seen in clinic with a history of worsening...

    Incorrect

    • A 71 year-old dentist is seen in clinic with a history of worsening memory problems and confusion. His wife had noted that his sleep was becoming more disturbed and he complains of vivid nightmares and visual hallucinations. Over the past few weeks, he has had increasing difficulty in dressing himself, and his mobility has deteriorated. On examination, he is bradykinesic with a resting tremor and rigidity affecting his arms and legs. His Mini-Mental-State Examination (MMSE) is 18/30. Which of the following is the most likely diagnosis?

      Your Answer: Vascular dementia

      Correct Answer: Lewy body disease

      Explanation:

      Lewy body dementia is the second most common cause of dementia in the elderly after Alzheimer’s disease. The core feature is a progressive dementia, but other characteristic features include Parkinsonism, visual hallucinations, fluctuating cognitive abilities and executive function, and an increased risk of falls or autonomic failure.

    • This question is part of the following fields:

      • Neurology
      230
      Seconds
  • Question 4 - A 59-year-old scientist is referred to you with a 2-year history of ascending...

    Incorrect

    • A 59-year-old scientist is referred to you with a 2-year history of ascending lower limb numbness and, more recently, foot drop. In the last 6 months he has also developed numbness in his fingers. He has a distal reduction to pinprick and relatively preserved muscle power, except for ankle dorsiflexion and hyporeflexia in his legs. The GP has already organised nerve conduction studies and the report is sent along with the patient. Which of the following would be suggestive of an axonal neuropathy?

      Your Answer:

      Correct Answer: Reduced compound muscle action potential amplitude

      Explanation:

      Reduced conduction velocity is associated with demyelinating neuropathies. An abnormally slow response is associated with very proximal disease, i.e. radiculopathies. Delayed P100 latency is a feature of performing visual evoked potentials in those with optic nerve disease. Conduction block is usually associated with certain types of demyelinating neuropathy.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 5 - A 57-year-old architect presents with weakness of the right hand. You note global...

    Incorrect

    • A 57-year-old architect presents with weakness of the right hand. You note global wasting of the small hand muscles. There is also sensory loss over the medial border of the forearm around the elbow. Which of the following nerve roots is damaged?

      Your Answer:

      Correct Answer: T1

      Explanation:

      This patient has Klumpke’s paralysis due to damage to the T1 nerve root. This root eventually supplies the median and ulnar nerves. The ulnar nerve supplies all of the intrinsic hand muscles except for those of the thenar eminence and the first and second lumbricals, which are innervated by the median nerve.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 6 - A 29 year-old new mother presented with a headache that was first noticed...

    Incorrect

    • A 29 year-old new mother presented with a headache that was first noticed as she was picking up her 5 week-old baby. On admission, she was unable to tolerate the lights and complained of feeling sick. Fundoscopy showed bilateral papilledema, and she was complaining that she was unable to see on her left side. CT head showed a small right occipital bleed. Which of the following treatments is most appropriate?

      Your Answer:

      Correct Answer: Heparin

      Explanation:

      This patient has developed a venous sinus thrombosis peri-partum, resulting in her symptoms. Anticoagulation therapy including Heparin improves outcomes.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 7 - A 46-year-old plumber develops chronic, severe pain after sustaining a brachial plexus injury...

    Incorrect

    • A 46-year-old plumber develops chronic, severe pain after sustaining a brachial plexus injury as a result of a motorbike accident. He has had no benefit from paracetamol or ibuprofen. In addition, he has had an unsuccessful trial of amitriptyline. Following recent NICE guidelines, which of the following is the most appropriate medication to consider?

      Your Answer:

      Correct Answer: Pregabalin

      Explanation:

      Neuropathic pain may be defined as pain which arises following damage or disruption of the nervous system. It is often difficult to treat and responds poorly to standard analgesia.
      The most recent update to the NICE guidelines for management of neuropathic pain occurred in 2013: first-line treatment* includes amitriptyline. If the first-line drug treatment does not work then move on to one of the other 3 drugs: duloxetine, gabapentin or pregabalin. Tramadol may be used as ‘rescue therapy’ for exacerbations of neuropathic pain. Topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia). Pain management clinics may be useful in patients with resistant problems.

      *please note that for some specific conditions the guidance may vary. For example carbamazepine is used first-line for trigeminal neuralgia.

    • This question is part of the following fields:

      • Neurology
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  • Question 8 - A 6-year-old boy is referred by his GP to the neurology clinic with...

    Incorrect

    • A 6-year-old boy is referred by his GP to the neurology clinic with abnormal movements. His mother noticed that for the last year, the boy has been falling over more and more frequently. He has also been having increasingly slurred speech. These have been getting progressively worse. He has had recurrent chest infections in his childhood. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Ataxic telangiectasia

      Explanation:

      Ataxic telangiectasia is an inherited combined immunodeficiency disorder that is characterised by cerebellar ataxia and telangiectasia as seen in this child, as well as frequent infections as noted in this child’s history. The other differentials would not present with this clinical picture:

      Friedreich’s ataxia and Infantile-onset spinocerebellar ataxia do not present with immune problems, whereas Cerebral palsy and Di-George Syndrome do not present with ataxia.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 9 - A 78-year-old retired journalist known to have prostatic carcinoma presents to the ED...

    Incorrect

    • A 78-year-old retired journalist known to have prostatic carcinoma presents to the ED complaining of pain in the spine and the onset of severe lower-leg weakness accompanied by a loss of sensation. On examination, he is found to have percussion tenderness of his spine, loss of sensation up to the umbilicus and a distended bladder. He has markedly reduced power of the lower legs with hyperreflexia. Which of the following should not be part of your management of this patient?

      Your Answer:

      Correct Answer: Spinal X-rays

      Explanation:

      Acute cord compression is a medical emergency. Typically, signs of segmental damage at the level of compression are usually combined with corticospinal tract dysfunction (e.g., hyperreflexia, Babinski’s sign and weakness) and sensory deficits below the level of compression. Symptoms include spinal pain that precedes the development of weak legs and sensory loss. There may be loss of bladder (and anal) sphincter control, manifesting as hesitancy, frequency and, finally, painless retention.
      Spinal X-rays are rarely diagnostic. MRI is usually the investigation of choice and should not be delayed, but if not available consider doing a CT scan and myelography to confirm cord compression and fully define the level and extent of the lesion. If malignancy is the cause, it is important to give dexamethasone (oral or intravenous) while considering therapy more specific to the cause.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 10 - A 72-year-old woman presents with 18 month history of gait ataxia, dysarthria, and...

    Incorrect

    • A 72-year-old woman presents with 18 month history of gait ataxia, dysarthria, and dysphagia. On examination there is down beating nystagmus and slurred speech. There is past pointing in both upper limbs and a wide-based ataxic gait. Reflexes and sensation are normal. There is no wasting or fasciculations. Plantar response is flexor bilaterally. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Arnold-Chiari malformation

      Explanation:

      Downbeat nystagmus (DBN) suggests a lesion in the lower part of the medulla. Arnold-Chiari malformation usually presents with symptoms due to brainstem and lower cranial nerve dysfunction such as DBN.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 11 - A 27-year-old realtor presented with progressive weakness of both legs over the last...

    Incorrect

    • A 27-year-old realtor presented with progressive weakness of both legs over the last 3 years. He complained of being unable to see well at night and having an impaired sense of smell. On examination he had a shortened fourth toe bilaterally with pes cavus. Neurological examination revealed a loss of pinprick sensation to bilateral knees, and weakness of both legs that was more prominent distally. Which of the following would be the best blood test to order to make a diagnosis?

      Your Answer:

      Correct Answer: Phytanic acid

      Explanation:

      The diagnosis is Refsum’s disease. This is an autosomal recessive disorder that causes a sensorimotor peripheral neuropathy. It is caused by defective alpha oxidation of phytanic acid leading to its accumulation in tissues. Cardiac conduction abnormalities and cardiomyopathies may also occur.
      Epiphyseal dysplasia causes a characteristic shortening of the fourth toe. Serum phytanic acid levels are elevated. Treatment is by dietary restriction of foods containing phytanic acid (dairy products, fish, beef and lamb).

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 12 - A 17-year-old girl presented with fever, headache and photophobia. Cerebrospinal fluid examination reveals:...

    Incorrect

    • A 17-year-old girl presented with fever, headache and photophobia. Cerebrospinal fluid examination reveals: Opening pressure 260 mm H20 (50-180) Total protein 0.8 g/l (0.15-0.45) Glucose 4.2 mmol/l (3.3-4.4) White cell count 60 per ml (<5) Lymphocytes 90% Plasma glucose 6.4 mmol/l (3.0-6.0) Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Viral meningitis

      Explanation:

      Normal cerebrospinal fluid (CSF) glucose together with lymphocytosis, an increased opening pressure and raised CSF protein are typical of a viral meningitis.

    • This question is part of the following fields:

      • Neurology
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      Seconds
  • Question 13 - Which of the following features is not associated with an oculomotor nerve palsy?...

    Incorrect

    • Which of the following features is not associated with an oculomotor nerve palsy?

      Your Answer:

      Correct Answer: Miosis

      Explanation:

      Oculomotor nerve palsy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. A complete oculomotor nerve palsy will result in a characteristic down and out position in the affected eye. This is because the lateral rectus (innervated by the sixth cranial nerve) and superior oblique (innervated by the fourth cranial or trochlear nerve), is unantagonized by the paralyzed superior rectus, inferior rectus and inferior oblique. The affected individual will also have a ptosis, or drooping of the eyelid, and mydriasis (pupil dilation), not miosis.

    • This question is part of the following fields:

      • Neurology
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  • Question 14 - A 47-year-old hypertensive man presents with difficulty using his right arm, slow walking...

    Incorrect

    • A 47-year-old hypertensive man presents with difficulty using his right arm, slow walking and occasional loss of balance. He has a broad-based gait with cogwheel rigidity and intention tremor of his right arm. His blood pressure is 140/80 mmHg sitting and 100/60 mmHg standing. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Multiple system atrophy

      Explanation:

      This patient presents with a combination of akinetic rigid syndrome, cerebellar signs and the suggestion of autonomic features. This is most indicative of a diagnosis of multiple system atrophy.

      Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 15 - A 53-year-old cashier with a history of chronic back pain presents for a...

    Incorrect

    • A 53-year-old cashier with a history of chronic back pain presents for a check-up. He is aware of a dragging feeling affecting his left foot when he tries to walk. This has developed since a minor injury to his left knee. On examination, he has weakness of dorsiflexion and eversion of the left foot. The right is unaffected and plantar flexion and inversion are normal on the left. MRI of the spinal cord shows degenerative disc changes at multiple levels, but no evidence of cord or nerve root impingement. Nerve conduction studies and EMG results are pending. Which of the following sensory loss patterns would you expect to find in association with this motor defect?

      Your Answer:

      Correct Answer: Sensory loss over the dorsum of the foot and anterolateral leg

      Explanation:

      This patient presentation is unlikely to be an L5 nerve root lesion given the results of the MRI scan. Therefore, the most likely diagnosis is a mononeuritis affecting the left common peroneal nerve. This would lead to sensory loss over the dorsum of the foot and anterolateral leg on the left.

    • This question is part of the following fields:

      • Neurology
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  • Question 16 - A 44-year-old hairdresser with a history of myasthenia gravis is admitted to the...

    Incorrect

    • A 44-year-old hairdresser with a history of myasthenia gravis is admitted to the Emergency Department. She is currently taking pyridostigmine, but there has been a significant worsening of her symptoms following antibiotic treatment for a chest infection. On examination she is dyspnoeic and cyanotic with quiet breath sounds in both lungs. Other than respiratory support, what are the two other treatments of choice?

      Your Answer:

      Correct Answer: Plasmapheresis or intravenous immunoglobulins

      Explanation:

      This patient is having a myasthenic crisis. Opinions vary as to whether plasmapheresis or intravenous immunoglobulins should be given first-line. Plasmapheresis usually works much faster, but is more costly due to equipment.
      Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 85-90% of cases. Myasthenia gravis is more common in women (2:1).

    • This question is part of the following fields:

      • Neurology
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      Seconds
  • Question 17 - Which of the following is true concerning baclofen? ...

    Incorrect

    • Which of the following is true concerning baclofen?

      Your Answer:

      Correct Answer: Causes hallucinations when withdrawn

      Explanation:

      Baclofen is used to treat spastic movement symptoms such as those seen in cerebral palsy and multiple sclerosis. It is known to be associated with a withdrawal syndrome similar to alcohol withdrawal; thus, gradual withdrawal is necessary to avoid this.

    • This question is part of the following fields:

      • Neurology
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  • Question 18 - A 59-year-old surgeon presents with a progressive paraesthesia and numbness in both feet,...

    Incorrect

    • A 59-year-old surgeon presents with a progressive paraesthesia and numbness in both feet, which have deteriorated over the last six months. He has a 10 year history of type 2 diabetes mellitus and had cervical spondylosis, for which he underwent surgery eight years ago. He also confessed to drinking approximately 40 units of alcohol weekly. On examination he had mild bilateral weakness of foot dorsiflexion and both ankle reflexes were absent. There was absent sensation to light touch to mid-shin level with loss of joint position sensation in the toes and absent vibration sensation below the hips. He had a marked sensory ataxia and pseudoathetosis of the upper limbs. He had no evidence of a retinopathy and urinalysis was normal. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Vitamin B 12 deficiency

      Explanation:

      Diabetic peripheral neuropathy usually goes in parallel with retinopathy and nephropathy. It is also slowly progressive and affects mainly the spinothalamic pathway.
      Alcohol induced peripheral neuropathy is also slowly progressive and affects mainly the spinothalamic pathway.
      Vitamin B 12 deficiency usually causes a more rapidly progressive neuropathy with dorsal column involvement (joint position and vibration involvement with sensory ataxia and pseudoathetosis of upper limbs).

    • This question is part of the following fields:

      • Neurology
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  • Question 19 - A 20-year-old gentleman presents to the A&E department complaining of a sudden-onset occipital...

    Incorrect

    • A 20-year-old gentleman presents to the A&E department complaining of a sudden-onset occipital headache associated with vomiting. His symptoms started 2 hours previously and are continuing. He has a previous history of infrequent migraine without aura, which also causes nausea but not vomiting. He rated his current headache as much more severe than his usual migraine. Examination is unremarkable. In particular, there is no neck stiffness or photophobia. Which of the following management options would be the most appropriate?

      Your Answer:

      Correct Answer: CT brain scan, followed by lumbar puncture if CT normal

      Explanation:

      The patient presented with sudden-onset headache that is more painful than his usual migraine attacks. This gives a high suspicion of subarachnoid haemorrhage; thus, a CT brain scan should be ordered first to rule this out. However, a normal CT scan is apparent in 30% of patients with subarachnoid haemorrhage and should be referred for lumbar puncture to look for red blood cells.

    • This question is part of the following fields:

      • Neurology
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  • Question 20 - A 66 year-old marketing analyst presents to the respiratory clinic with a 2-month...

    Incorrect

    • A 66 year-old marketing analyst presents to the respiratory clinic with a 2-month history of progressive weakness and shortness of breath. He finds it difficult to stand from sitting, and struggles climbing stairs. He is an ex-smoker with chronic obstructive pulmonary disease (COPD). He had a recent exacerbation one month ago for which he was treated by the GP with a course of oral prednisolone, during which time his weakness transiently improved. On examination, you note a left-sided monophonic wheeze and reduced breath sounds at the left lung base. Blood tests and a chest x-ray are requested. Hb 145 g/L WCC10.5 109/L, Na+136 mmol/L, K+ 4.3 mmol/L, Urea 6.8 mmol/L, Creatinine 93 mmol/L, Calcium 2.62 mmol/L, Phosphate 1.44 mmol/L, Chest x-ray shows hyperinflated lungs, left lower lobe collapse and a bulky left hilum What is the most likely cause of this patient's weakness?

      Your Answer:

      Correct Answer: Lambert-Eaton myasthenic syndrome

      Explanation:

      This man has a small-cell lung cancer (SCLC) and associated Lambert-Eaton myasthenic syndrome – a well-recognized paraneoplastic manifestation of SCLC. This classically affects the proximal muscles, especially in the legs, causing difficulty in standing from a seated position and climbing stairs. In contrast to myasthenia gravis, eye involvement is uncommon. Treatment with steroids is often helpful, which explains his transient symptomatic improvement during treatment for his COPD exacerbation. Steroid myopathy does not fit as the symptoms started well before his course of prednisolone. Although the patient is mildly hypercalcaemic, this would not be sufficient to produce his presenting symptoms, although it does reinforce the suspicion of lung malignancy. Motor neurone disease would be unlikely in this context and would not improve with steroids. Myasthenia gravis could produce these symptoms, but in the context of a new lung mass is a less viable diagnosis.

    • This question is part of the following fields:

      • Neurology
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  • Question 21 - Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with...

    Incorrect

    • Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with sensory loss?

      Your Answer:

      Correct Answer: Uraemia

      Explanation:

      Diseases that cause predominantly sensory loss include diabetes, uraemia, leprosy, alcoholism, vitamin B12 deficiency, and amyloidosis.
      Those that cause predominantly motor loss include Guillain-Barre syndrome, porphyria, lead poisoning, hereditary sensorimotor neuropathies, chronic inflammatory demyelinating polyneuropathy, and diphtheria.

    • This question is part of the following fields:

      • Neurology
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  • Question 22 - A 43-year-old police officer is admitted with a history of unsteadiness and slurring...

    Incorrect

    • A 43-year-old police officer is admitted with a history of unsteadiness and slurring of speech. This has worsened over a period of three months. He complains of a tremor affecting his right hand and diplopia on right lateral gaze. He smokes 30 cigarettes a day and takes regular diclofenac for his arthritis. On examination, he is dysarthric and feels nauseated. Fundoscopy is normal, however there is marked horizontal nystagmus and evidence of a right VI nerve palsy. There also appears to be some mild facial weakness on the right side. Upon conducting Weber’s test, a louder tone is heard in the left ear. On conducting the Rinne test, both ears are normal. On examination of the upper limb, there is a right intention tremor and dysdiadochokinesis. Tone, power and reflexes are normal. On examination of the lower limb, tone, power and reflexes are normal, however he appears to walk with a broad-based gait and is leaning to the right. Lumbar puncture: Opening pressure 13 cm H20 (5–18) Protein 0.67 g/l (0.15–0.45) WCC 3 cells/ml (<5) Red cell count (RCC) 2 cells/ml (<5) Glucose 3.2 mmol/l (3.3–4.4) Blood glucose 5.8 mmol/l (3.0–6.0) Oligoclonal bands Present Serum oligoclonal bands Present Magnetic resonance scan shows a calcified lesion broadly attached to the petrous part of the temporal bone. In view of the above history and findings, what is the likely cause of this patient’s symptoms?

      Your Answer:

      Correct Answer: Meningioma of the cerebellar pontine angle

      Explanation:

      This patient has a combination of right cerebellar dysfunction with right-sided cranial nerve palsies (VI, VII, and VIII). The magnetic resonance imaging (MRI) shows a calcified meningioma within the right cerebellar pontine area, which would account for these findings. The cerebrospinal fluid (CSF) analysis shows oligoclonal bands, however, these are matched in the serum, which reflects a systemic inflammatory response from his rheumatoid arthritis.

      The MRI scan and CSF analysis would not be consistent with progressive multiple sclerosis. The progressive nature of her symptoms would be against a diagnosis of brainstem infarct, and one would expect more pyramidal signs in the peripheral nervous system.

    • This question is part of the following fields:

      • Neurology
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  • Question 23 - A 58-year-old psychologist with small cell lung cancer complains of muscle weakness. Each...

    Incorrect

    • A 58-year-old psychologist with small cell lung cancer complains of muscle weakness. Each one of the following are features of Lambert-Eaton syndrome, except:

      Your Answer:

      Correct Answer: Repeated muscle contractions lead to decreased muscle strength

      Explanation:

      In myasthenia gravis, repeated muscle contractions lead to reduced muscle strength. The opposite is however classically seen in the related disorder Lambert-Eaton syndrome. Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer, and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system.

    • This question is part of the following fields:

      • Neurology
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  • Question 24 - A 75-year-old retired tailor is examined for involuntary, jerking movements of his arms....

    Incorrect

    • A 75-year-old retired tailor is examined for involuntary, jerking movements of his arms. His symptoms seem to resolve when he is asleep. Damage to which one of the following structures may lead to hemiballism?

      Your Answer:

      Correct Answer: Subthalamic nucleus

      Explanation:

      Hemiballismus or hemiballism in its unilateral form is a very rare movement disorder. It is a type of chorea caused in most cases by a decrease in activity of the subthalamic nucleus of the basal ganglia, resulting in the appearance of flailing, ballistic, undesired movements of the limbs. Symptoms may decrease while the patient is asleep. Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment.

    • This question is part of the following fields:

      • Neurology
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  • Question 25 - A 22 year-old university graduate presented with progressive unsteadiness during walking over the...

    Incorrect

    • A 22 year-old university graduate presented with progressive unsteadiness during walking over the last year. She had been otherwise healthy apart from recent difficulty hearing her lecturer in classes. She took no prescription medication but had occasionally taken cocaine during her first year of college. She also admits to drinking up to 30 units of alcohol per week and smoked 10 cigarettes per day. Her parents were both well, but her father's sister had problems with walking before she died. Examination reveals normal tone and power throughout all four limbs. Reflexes were normal in the upper limbs but decreased at the knees and absent at the ankles. Coordination was normal in all four limbs but her gait was ataxic. Sensation in the upper limbs was normal but decreased vibratory sensation and proprioception was noted to the ankles bilaterally. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Friedreich’s ataxia

      Explanation:

      Friedreich’s ataxia is an autosomal recessive disorder that usually begins before the end of the teens. It has an estimated prevalence in Europe of 1 in 50,000 and life expectancy is around 40–50 years. Neurological features include a progressive ataxia, cerebellar dysarthria, lower limb areflexia, decreased vibratory sensation and proprioception, and pyramidal weakness. Pes cavus and scoliosis are also both seen. Cardiomyopathy occurs in over 70% of cases. Less common features include optic atrophic, diabetes mellitus, and deafness.

    • This question is part of the following fields:

      • Neurology
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  • Question 26 - A patient presented with acute onset of mild right hemiparesis affecting the body....

    Incorrect

    • A patient presented with acute onset of mild right hemiparesis affecting the body. He also has evidence of sensory loss on the right hand side. There is evidence of Horner’s syndrome and sensory loss on the face on the left hand side. Which of the following structures are involved?

      Your Answer:

      Correct Answer: Brain stem

      Explanation:

      The patient is presenting with symptoms of lateral medullary syndrome also known as Wallenberg’s syndrome or posterior inferior cerebellar artery syndrome, where the symptoms are due to an ischemia in the brainstem. The classical symptoms include contralateral sensory deficits of the trunk region paired with ipsilateral facial sensory deficits.

    • This question is part of the following fields:

      • Neurology
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  • Question 27 - A 60-year-old lady has been admitted to the stroke ward following a sudden...

    Incorrect

    • A 60-year-old lady has been admitted to the stroke ward following a sudden onset of dizziness and visual disturbances, which started yesterday morning. She initially thought she was just dehydrated, however, later realised she was unable to read her own shopping list. On the ward rounds, the consultant examines her and finds she is indeed unable to read. She is, however, able to write. When she writes a sentence it makes perfect sense, although she is again unable to read it out. She has no problems with her speech and is able to converse normally. She has no motor focal neurological deficit. The consultant asks you where the lesion is likely to be?

      Your Answer:

      Correct Answer: Corpus callosum

      Explanation:

      The patient presents with sudden onset of alexia (the inability to read) WITHOUT agraphia (inability to write) which is consistent with lesions of the corpus callosum where there is a disconnect syndrome and the patient’s language and visual centres are actually in tact, but are unable to communicate between hemispheres. In this case, the damage due to the stroke is most likely in the left visual cortex, leaving visual processing to the intact right hemisphere which unfortunately cannot communicate the information to the language centres (Broca and Wernicke’s) in the left hemisphere, hence the alexia. Alternatively, the speech and writing are unaffected as the language centres can still communicate with the primary motor cortex.

    • This question is part of the following fields:

      • Neurology
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  • Question 28 - A 43 year-old female artist with no past medical history presents to the...

    Incorrect

    • A 43 year-old female artist with no past medical history presents to the emergency department with a 2-day history of pins and needles in the lower limbs, and progressive walking difficulties. She states she had diarrhoea 1 week ago. On examination, there is a loss of pinprick sensation noted to the lower limbs from mid-thigh distally and in the upper limbs from MCP joints distally. There is bilateral weakness of ankle dorsiflexion, noted at 3/5, and knee flexion and extension weakness, noted at 4/5 bilaterally. Power in upper and lower limbs is otherwise normal. Knee and ankle deep tendon reflexes are absent. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Guillain-Barre syndrome

      Explanation:

      Guillain-Barre syndrome is an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). Characteristic features include progressive weakness of all four limbs, and it is classically ascending, affecting the lower extremities first. Sensory symptoms tend to be mild.

      Functional neurological syndrome can be discounted due to presence of hard neurological signs. Multiple sclerosis can be excluded because of the presence of lower motor neuron signs and absence of upper motor neuron signs. Chronic inflammatory demyelinating polyneuropathy is the chronic form of Guillain-Barre syndrome.

    • This question is part of the following fields:

      • Neurology
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  • Question 29 - Which of the following may cause a downbeat nystagmus? ...

    Incorrect

    • Which of the following may cause a downbeat nystagmus?

      Your Answer:

      Correct Answer: Chiari type I malformation

      Explanation:

      Downbeat nystagmus (DBN) suggests a lesion in the lower part of the medulla. Chiari Type I malformation usually presents with symptoms due to brain stem and lower cranial nerve dysfunction, which includes DBN.

    • This question is part of the following fields:

      • Neurology
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  • Question 30 - Which one of the following medications is most useful for helping to prevent...

    Incorrect

    • Which one of the following medications is most useful for helping to prevent attacks of Meniere’s disease?

      Your Answer:

      Correct Answer: Betahistine

      Explanation:

      Betahistine is a histamine analogue that has been the mainstay treatment drug for Meniere’s disease.

    • This question is part of the following fields:

      • Neurology
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