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Question 1
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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: 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.
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This question is part of the following fields:
- Nervous System
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Question 2
Correct
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A 25-year-old athlete presented with a 7-month history of difficulty gripping things. He complained of finding it particularly difficult in cold weather. He remembered his father having similar problems. Upon examination, he had a bilateral ptosis with weakness of the facial muscles. He also had difficulty opening his eyes quickly. Limb examination revealed distal weakness in both hands with difficulty opening and closing both hands quickly. Which of the following is the most likely diagnosis?
Your Answer: Myotonic dystrophy
Explanation:Myotonic dystrophy is the most likely diagnosis here.
It is a multisystem disorder causing cognitive impairment, cataracts, cardiac problems and testicular atrophy, as well as affecting the muscles. Patients have muscle weakness, normally worse distally, and/or myotonia (which is worse in cold weather).
On examination, patients may also have frontal balding, a myopathic facies, bilateral ptosis, an ophthalmoplegia and wasting of facial muscles and other limb muscles. Myotonic dystrophy is associated with diabetes mellitus and pituitary dysfunction.
Diagnosis is normally based on clinical features with a characteristic electromyogram (EMG) of myotonic discharges. Creatine kinase is generally normal and muscle biopsy is non-specific. -
This question is part of the following fields:
- Nervous System
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Question 3
Correct
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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:
- Nervous System
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Question 4
Correct
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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: 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.
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This question is part of the following fields:
- Nervous System
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Question 5
Incorrect
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Which is the most appropriate agent for the prophylaxis of cluster headaches?
Your Answer: Aspirin
Correct Answer: Verapamil
Explanation:Verapamil, can effectively reduce the number of attacks during a cluster headache period of exacerbation. Oral sumatriptan has not been shown to be effective prophylactically. Verapamil in dosages of 360 to 480 mg daily is one of the few treatments for episodic cluster headache tested in a randomized controlled trial (RCT) and found effective in reducing attack frequency.
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This question is part of the following fields:
- Nervous System
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Question 6
Correct
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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:
- Nervous System
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Question 7
Incorrect
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A 55-year-old male visited his diabetic clinic with a complaint of inability to walk properly due to weakness of his lower limbs. On examination, dorsiflexion was found to be weak bilaterally and there was diminished sensation on the dorsum of his feet as well as on the lower lateral portions of his legs. Which of the following could have led to this condition?
Your Answer: Compression of the S1 nerve root
Correct Answer: Compression of the common peroneal nerve
Explanation:The branches of the common peroneal nerve innervate the skin of the dorsum of the foot as well as the muscles which help to carry out dorsiflexion of the foot. Compression of the common peroneal nerve cause foot drop as well as the loss in sensation of the skin on the dorsum of the foot.
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This question is part of the following fields:
- Nervous System
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Question 8
Correct
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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: 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.
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This question is part of the following fields:
- Nervous System
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Question 9
Correct
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A 55-year-old male, known hypertensive on antihypertensive medications, presented with complaints of dizziness and lethargy, especially when rising from the chair. The most appropriate test would be?
Your Answer: Ambulatory blood pressure
Explanation:Ambulatory blood pressure recording is used to monitor BP for 24 hours whilst continuing the daily routine activities.
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This question is part of the following fields:
- Nervous System
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Question 10
Correct
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A 55-year-old female school teacher complains of double vision when she writes on the school blackboard. She has no history of trauma. Her most recent visit to her GP was to seek attention for a rash which developed after she was bitten when walking through the forest on a school trip. Choose the cranial nerve most likely to be affected here.
Your Answer: Abducens
Explanation:This nerve is responsible for the side to side movement of the eye, hence why damage can cause double vision.
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This question is part of the following fields:
- Nervous System
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SESSION STATS - PERFORMANCE PER SPECIALTY
