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Question 1
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A 73-year-old gentleman with type 2 diabetes mellitus, complains of difficulty walking and trouble with his hands. It began with a tingling sensation in his soles, which later extended up to his ankles. He now feels unsteady when walking, and more recently, has noticed numbness and tingling in the fingers of both hands. On examination, he has absent ankle reflexes, a high steppage gait, and altered sensation to his mid-calves. What is the underlying pathological process?
Your Answer: Axonal degeneration
Explanation:This case presents with sensorimotor neuropathy secondary to his DM. The progression of the neuropathy, known dying-back neuropathy, is a distal axonopathy or axonal degeneration as where the sensorimotor loss begins distally and travels proximally.
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This question is part of the following fields:
- Neurology
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Question 2
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A 29-year-old physiotherapist with a history of bilateral vitreous haemorrhage is referred due to progressive ataxia. Which of the following is the most likely diagnosis?
Your Answer: Von Hippel-Lindau syndrome
Explanation:Retinal and cerebellar haemangiomas are key features of Von Hippel-Lindau syndrome. Retinal haemangiomas are bilateral in 25% of patients and may lead to vitreous haemorrhage. Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3.
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This question is part of the following fields:
- Neurology
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Question 3
Incorrect
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A 37-year-old teacher with multiple sclerosis complains that her vision becomes blurred during a hot bath. Which of the following explain this?
Your Answer: Lhermitte's sign
Correct Answer: Uhthoff's phenomenon
Explanation:Uhthoff’s phenomenon is worsening of vision following a rise in body temperature.
Lhermitte’s sign describes paraesthesia in the limbs on neck flexion.
Oppenheim’s sign is seen when scratching of the inner side of leg leads to extension of the toes. It is a sign of cerebral irritation and is not related to multiple sclerosis.
Werdnig-Hoffman’s disease is also known as spinal muscular atrophy. -
This question is part of the following fields:
- Neurology
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Question 4
Incorrect
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A 35-year-old woman was on a camping holiday in Spain. She awoke at three o’clock one morning with severe neck pain radiating down into her left shoulder and down to her forearm. The next day it spread to the dorsal aspect of the forearm. She was otherwise well. Her symptoms resolved after 24 hours. She noticed that after a week she was unable to wind down the car window with her left arm. On examination of the left arm there was wasting of brachioradialis, shoulder, biceps and winging of left scapula. What is the diagnosis?
Your Answer: C5 root lesion
Correct Answer: Amyotrophic neuralgia
Explanation:This patient present with the classical symptoms of Amyotrophic neuralgia, characterised by sudden onset of pain in the shoulders that radiate down to the forearms and later resolve spontaneously but is followed by muscle wasting.
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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 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: Chronic inflammatory demyelinating polyneuropathy
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.
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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 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: Corticobasal degeneration
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.
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This question is part of the following fields:
- Neurology
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Question 7
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A 21 year-old software developer undergoes a planned lumbar puncture (LP) as part of a neurological investigation for possible multiple sclerosis. During the consent process, she expresses concern about a post-LP headache. What is the mechanism of post-LP headaches?
Your Answer: Leaking cerebrospinal fluid from the dura
Explanation:Leaking of cerebrospinal fluid from the dura is the most likely explanation for post-lumbar puncture headaches. It is thought that ongoing leak of cerebrospinal fluid (CSF) through the puncture site causes ongoing CSF loss, leading to low pressure. A post-LP headache is typically frontal or occipital and occurs within three days. It is normally associated with worsening on standing and improvement when lying down. Treatment in severe cases includes an epidural blood patch, but most resolve on their own.
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This question is part of the following fields:
- Neurology
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Question 8
Correct
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A 69 year-old librarian with motor neuron disease is seen in clinic. Which of the following interventions will have the greatest effect on survival?
Your Answer: Non-invasive ventilation
Explanation:Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before age 40 and various patterns of disease are recognised, including amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy.
Non-invasive ventilation (usually BIPAP) is used at night, with studies having shown a survival benefit of around 7 months. Riluzole prevents stimulation of glutamate receptors, used mainly in amyotrophic lateral sclerosis and has been shown to prolong life by about 3 months. -
This question is part of the following fields:
- Neurology
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Question 9
Correct
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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 10
Incorrect
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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: Duchenne’s muscular dystrophy
Correct 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:
- Neurology
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