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Question 1
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A 26-year-old technician with no past medical history presents to the neurology clinic complaining of muscle pain. He describes severe bilateral cramping calf pain on minimal exercise. This has been present since his late teens and as such he has avoided exercise. Recently, he has attended the local gym to try and lose weight, but attempts at exercise have been dampened by the pain. He does note that if he perseveres with exercise, the pain settles. He has noticed passing dark urine in the evenings following a prolonged bout of exercise. Which of the following clinical tests would be most useful in aiding the diagnosis in this patient?
Your Answer: Muscle biopsy
Explanation:The differential diagnosis of bilateral exercise-induced pain would include metabolic muscle disease, lumbar canal stenosis and intermittent claudication. The patient’s age, history and lack of other risk factors make the latter two options unlikely.
The syndrome described is in fact McArdle’s disease (myophosphorylase deficiency). This is a disorder of carbohydrate metabolism. Clinical features of pain and fatigue are precipitated in early exercise, as carbohydrates cannot be mobilized to provide an energy substrate to the muscle. With prolonged exercise, fatty acid metabolism provides energy, and symptoms lessen. The dark urine described is likely to represent myoglobinuria following rhabdomyolysis. Definitive diagnosis of most metabolic muscle diseases relies on muscle biopsy and enzyme analysis.
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This question is part of the following fields:
- Neurology
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Question 2
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A 42 year old male undergoes a routine cranial nerve examination, which reveals the following findings: Rinne's test: Air conduction > bone conduction in both ears Weber's test: Localizes to the right side What do these test results imply?
Your Answer: Left sensorineural deafness
Explanation:Weber’s test – if there is a sensorineural problem, the sound is localized to the unaffected side (right), indicating a problem on the left side.
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This question is part of the following fields:
- Neurology
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Question 3
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A 48-year-old patient with diabetes is referred from the Emergency department complaining of dizziness and vomiting. On examination he is alert and orientated, his pulse is 80 irregularly irregular and BP 160/90 mmHg. There is nystagmus on left lateral gaze and his speech is slurred. On examination of the limbs, you note intention tremor and past pointing. He is ataxic when mobilised. What is the likely diagnosis?
Your Answer: Cerebellar CVA
Explanation:The patient’s presentation with slurred speech, intention tremor and past pointing, as well as ataxia and nystagmus, paired with a history of vertigo suggest the cerebellum as the site of cerebrovascular accident (CVA) or stroke.
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This question is part of the following fields:
- Neurology
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Question 4
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A 27-year-old gentleman presents with a 2 year history of involuntary neck movements. There is no history of trauma. He is on no drugs. There is no relevant family history. On examination he has an episodic right torticollis. The rest of his neurological examination is normal. MRI of his cervical spine is normal. The neurologist in the Movement Disorders Clinic has diagnosed cervical dystonia. Which of the following treatments will be most beneficial?
Your Answer: Botulinum toxin
Explanation:Botulinum toxin is a neurotoxic protein from Clostridium botulinum that causes flaccid paralysis as it acts by preventing the release of Ach at the neuromuscular joint. It is the first-line treatment for cervical dystonia (torticollis) because the condition is a neurological disorder characterised by unusual muscle contractions of the neck. With the use of Botulinum toxin, the contractions would be released.
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This question is part of the following fields:
- Neurology
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Question 5
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A 23-year-old designer is requesting the combined oral contraceptive pill. During the history taking, she states that in the past she has had migraines with aura. She asks why the combined oral contraceptive pill is contraindicated. Which of the following is the most appropriate response?
Your Answer: Significantly increased risk of ischaemic stroke
Explanation:SIGN produced guidelines in 2008 on the management of migraines. Key points include that if patients have migraines with aura then the combined oral contraceptive (COC) is absolutely contraindicated due to an increased risk of stroke (relative risk 8.72).
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This question is part of the following fields:
- Neurology
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Question 6
Incorrect
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A 63-year-old gentleman presents with left-sided eye pain and diplopia for the past 2 days. Examination of his eyes shows his pupils equal and reactive to light with no proptosis. There is however an apparent palsy of the 6th cranial nerve associated with a partial 3rd nerve palsy on the left side. Examining the remaining cranial demonstrates hyperaesthesia of the upper face on the left side. Where is the likely lesion?
Your Answer: Pons
Correct Answer: Cavernous sinus
Explanation:A lesion on the cavernous sinus would explain the palsy observed on the III and VI cranial nerves because the cranial nerves III, IV, V, and VI pass through the cavernous sinus. Pain in the eye is due to the nearby ophthalmic veins that feeds the cavernous sinus. Additionally, the lesions in the other structures would have presented with pupil abnormalities and less localized pain and symptoms.
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This question is part of the following fields:
- Neurology
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Question 7
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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: 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.
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This question is part of the following fields:
- Neurology
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Question 8
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A 39 year-old engineer presents with progressive weakness of his hands. Upon examination, you notice wasting of the small muscles of the hand. A diagnosis of syringomyelia is suspected. Which one of the following features would most support this diagnosis?
Your Answer: Loss of temperature sensation in the hands
Explanation:Syringomyelia is a development of a cavity (syrinx) within the spinal cord. Signs and symptoms include loss of feeling, paralysis, weakness, and stiffness in the back, shoulders, and extremities. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands. Symptoms typically vary depending on the extent and, often more critically, on the location of the syrinx within the spinal cord.
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This question is part of the following fields:
- Neurology
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Question 9
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A 26-year-old graduate student with a history of migraines presents for examination. His headaches are now occurring about once a week. He describes unilateral, throbbing headaches that may last over 24 hours. Neurological examination is unremarkable. Other than a history of asthma, he is fit and well. What is the most suitable therapy to reduce the frequency of migraine attacks?
Your Answer: Topiramate
Explanation:It should be noted that as a general rule 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis. NICE produced guidelines in 2012 on the management of headache, including migraines. Prophylaxis should be given if patients are experiencing 2 or more attacks per month. Modern treatment is effective in about 60% of patients. NICE advises either topiramate or propranolol ‘according to the person’s preference, comorbidities and risk of adverse events’. Propranolol should be used in preference to topiramate in women of child bearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives.
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This question is part of the following fields:
- Neurology
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Question 10
Incorrect
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A 51 year-old teacher presents complaining of numerous falls. He states he has difficulty walking up stairs, and he thinks it is because of his weak legs rather than blackouts. He is hypertensive and has suffered chronic back pain for many years. He has smoked for many years as well and has a chronic smokers cough. Upon examination, he has weakness of hip flexion and particularly knee extension. He is unable to keep his fingers flexed against force, with the right being weaker than the left. There are no sensory abnormalities and reflexes are preserved bilaterally. Which of the following is the most likely diagnosis?
Your Answer: Motor neurone disease
Correct Answer: Inclusion body myositis
Explanation:The pattern of muscle involvement seen with quadriceps and long-finger flexors is characteristic of inclusion body myositis, an inflammatory myopathy. Polymyositis is likely to cause a predominantly proximal weakness, associated with muscle pain. The signs and symptoms are not consistent with upper cord compression, as there would likely be sensory signs, reflex changes, and possible urinary symptoms. Motor neuron disease cannot be ruled out, but there are no findings of upper motor neuron or bulbar features.
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This question is part of the following fields:
- Neurology
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SESSION STATS - PERFORMANCE PER SPECIALTY
