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Question 1
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A 77 year old mail carrier presents to the emergency department with severe flinging movements of his left arm. Where would the causative lesion be located?
Your Answer: Right subthalamic nucleus
Explanation:Hemiballismus is a hyperkinetic involuntary movement disorder characterized by intermittent, sudden, violent, involuntary, flinging, or ballistic high amplitude movements involving the ipsilateral arm and leg caused by dysfunction in the central nervous system of the contralateral side.Global incidence and prevalence are largely unknown, given the wide variety of etiologies but estimated to be 1-2/1,000,000, classifying it as a rare disorder. -
This question is part of the following fields:
- Neurology
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Question 2
Incorrect
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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: Lumbar puncture
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
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Question 3
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A 32 year-old active male presents with a three month history of pain in his feet and lower legs. He was previously diagnosed with diabetes at age 14 and treated with insulin. He admits to drinking 30 units of alcohol per week and is a current cannabis smoker. On examination, pain and temperature sensation in his feet are diminished, but joint position and vibratory sensation appear normal. What is the most likely diagnosis?
Your Answer: Diabetic polyneuropathy
Explanation:The given history suggests a small fibre painful peripheral sensory neuropathy, the most common cause of which is diabetes. Joint position sense and vibratory sensation are carried through large fibres, and therefore are not currently affected. Sensory nerves are affected more often than motor, so reflexes usually remain in tact.
Vitamin B12 deficiency causes impairment of joint position and vibratory sensation.
Chronic inflammatory demyelinating polyneuropathy (CIPD) causes a large fibre peripheral neuropathy with areflexia.
In syringomyelia there is impaired pain and temperature noted in the upper limbs.
Finally, with alcoholic polyneuropathy, all fibre types are affected (sensory and motor loss). It is usually gradual with long term alcohol abuse and may be accompanied by a nutritional deficiency. In addition, pain is a more dominant feature.
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This question is part of the following fields:
- Neurology
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Question 4
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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:
- Neurology
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Question 5
Incorrect
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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 + donepezil + low-dose aspirin
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.
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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 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: Genetic testing
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
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Question 7
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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: Heparin
Explanation:This patient has developed a venous sinus thrombosis peri-partum, resulting in her symptoms. Anticoagulation therapy including Heparin improves outcomes.
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This question is part of the following fields:
- Neurology
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Question 8
Incorrect
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The chorda tympani of the facial nerve (CN VII) carries:
Your Answer: parasympathetic fibres to the submandibular and sublingual glands and the nerve to stapedius
Correct Answer: parasympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue
Explanation:In the petrous temporal bone the facial nerve produces three branches:
1. The greater petrosal nerve, which transmits preganglionic parasympathetic fibres to the sphenopalatine ganglion. These postganglionic fibres supply the lacrimal gland and the glands in the nasal cavity;
2. The nerve to stapedius;
3. Parasympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue. -
This question is part of the following fields:
- Neurology
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Question 9
Correct
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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: 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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Question 10
Correct
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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: 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.
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This question is part of the following fields:
- Neurology
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