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Question 1
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:
- Neurology
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Question 2
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: Becker’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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Question 3
Correct
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Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with sensory loss?
Your 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 4
Incorrect
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A 24-year-old gentleman presents with visual loss in his right eye, and this is diagnosed as optic neuritis. Which one of the following statements would be seen in an afferent pupillary defect?
Your Answer: Pupil of affected eye larger than the unaffected eye
Correct Answer: Accommodation response is unaffected
Explanation:Afferent pupillary defect is simply a delayed pupillary response to light. Accommodation is otherwise unaffected.
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This question is part of the following fields:
- Neurology
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Question 5
Correct
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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 6
Incorrect
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A 41-year-old yoga instructor presents with a 2-month history of left-hand weakness. She has no significant past medical history. On examination, there is mild weakness of the left upper and lower limbs with a right sided facial weakness, which spares the forehead. Which of the following is the most likely location of the lesion?
Your Answer: Right cerebrum
Correct Answer: Right pons
Explanation:The pons is above the level of decussation of the corticospinal tracts so a pontine lesion would cause a contralateral limb weakness.
The facial motor nucleus is located in the pons and supplies the ipsilateral facial muscles.
A right cerebral lesion would give left upper and lower limb weakness. It would also cause a left sided facial weakness.
A left cerebral lesion would give right upper and lower limb weakness with right facial weakness.
Finally, a cervical spinal cord lesion would not cause a facial weakness. -
This question is part of the following fields:
- Neurology
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Question 7
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: ANCA
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 8
Correct
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A 52-year-old chef presents to the ED with acute visual changes. He has a past medical history of hypertension and type 2 diabetes mellitus. On neurological examination, his upper and lower limbs are normal however he has a homonymous hemianopia with central preservation. Where is the most likely cause of his problems within the central nervous system?
Your Answer: Optic radiation
Explanation:Lesions in the optic radiation can cause a homonymous hemianopia with macular sparing, as a result of collateral circulation offered to macular tracts by the middle cerebral artery.
Lesions in the optic tract also cause a homonymous hemianopia, but without macular sparing.
Lesions in the optic chiasm, optic nerve, and temporal lobe cause bitemporal hemianopia, ipsilateral complete blindness, and superior homonymous quadrantanopia respectively. -
This question is part of the following fields:
- Neurology
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Question 9
Correct
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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
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 10
Incorrect
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A 38-year-old musician presented with a two-day history of sudden-onset occipital headache associated with nausea and vomiting. The next day, his right hand became weak for a few hours. On the same day he had an episode of sensory disturbance in his right upper limb consisting of tingling in his hand that spread up the arm, to his shoulder lasting less than two minutes in total. On the day of admission he had a similar episode of sensory disturbance lasting 30 seconds in total. On examination he had bilateral papilledema, no neck stiffness and an otherwise normal neurological examination. Which of the following is the most likely diagnosis?
Your Answer: Embolic transient ischaemic attack
Correct Answer: Venous sinus thrombosis
Explanation:Cerebral venous sinus thrombosis (CVST) is associated with headache (>90% of cases), seizures, focal weakness (40%) and papilledema (40%), all seen in this patient.
Risk factors for CVST include genetic or acquired prothrombotic disorders, pregnancy, the oral contraceptive pill, vasculitis, malignancy, dehydration and infection. However, there are multiple other associated factors.
Diagnosis is normally confirmed with magnetic resonance venography (MRV). Treatment is with anticoagulation, initially with heparin and subsequently with warfarin. -
This question is part of the following fields:
- Neurology
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Question 11
Incorrect
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Which of the following statements regarding restless leg syndrome is not true?
Your Answer: Family history is found in up to 50% of patients
Correct Answer: It is three times as common in females
Explanation:In restless leg syndrome (RLS), males and females are thought to be equally affected. RLS is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population and a family history may be present.
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This question is part of the following fields:
- Neurology
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Question 12
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: paraneoplastic brachioplexy
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 13
Incorrect
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Which of the following is true concerning baclofen?
Your Answer: Acts directly on skeletal muscle
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.
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This question is part of the following fields:
- Neurology
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Question 14
Incorrect
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A 44 year-old electrician is referred to you complaining of increasing problems with concentration. He also complains of irregular jerky movements of his extremities and fingers. He consumes approximately 25 units of alcohol per week. His father was diagnosed with dementia at the age of 40. Apart from generalized choreiform movements, his neurological and systemic examinations were normal. Which of the following is the most likely diagnosis?
Your Answer: Alcoholic cerebral degeneration
Correct Answer: Huntington's disease
Explanation:Huntington’s disease is an autosomal inherited condition characterized by progressive dementia and worsening choreiform movements. Symptoms typically appear between ages 30 and 50. Ultimately the weakened individual succumbs to pneumonia, heart failure, or other complications.
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This question is part of the following fields:
- Neurology
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Question 15
Correct
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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: 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.
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This question is part of the following fields:
- Neurology
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Question 16
Incorrect
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A 61-year old mechanic recently attended A&E, with a 3 month history of bilateral paraesthesia's and twitching affecting the thumb, first finger and lateral forearm. He denied any trauma. An MRI scan of his spine was performed and revealed cervical canal stenosis with mild cord compression. He was discharged and advised to see his GP for follow-up. Which of the following is the most appropriate initial step in management?
Your Answer: Commence neuropathic analgesia in the first instance and consider surgical evaluation if this does not work
Correct Answer: Refer to spinal surgery services
Explanation:Bilateral median nerve dysfunction is suggestive of degenerative cervical myelopathy (DCM) rather than bilateral carpal tunnel syndrome. DCM should be suspected in elderly patients presenting with limb neurology. This patient’s twitches are probably fibrillations, a sign of lower motor neuron dysfunction.
Degenerative cervical myelopathy is associated with a delay in diagnosis. It is most commonly misdiagnosed as carpal tunnel syndrome. In one study, 43% of patients who underwent surgery for degenerative cervical myelopathy had been initially diagnosed with carpal tunnel syndrome.
Management of these patients should be by specialist spinal services (neurosurgery or orthopaedic spinal surgery). Decompressive surgery is the mainstay of treatment and has been shown to stop disease progression. Physiotherapy and analgesia do not replace surgical opinion, though they may be used alongside. Nerve root injections do not have a role in management. -
This question is part of the following fields:
- Neurology
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Question 17
Correct
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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 18
Incorrect
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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: Sarcoidosis
Correct 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 19
Correct
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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: 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.
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This question is part of the following fields:
- Neurology
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Question 20
Incorrect
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Which one of the following medications is most useful for helping to prevent attacks of Meniere’s disease?
Your Answer: Prochlorperazine
Correct Answer: Betahistine
Explanation:Betahistine is a histamine analogue that has been the mainstay treatment drug for Meniere’s disease.
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This question is part of the following fields:
- Neurology
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Question 21
Incorrect
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Which of the following may cause a downbeat nystagmus?
Your Answer: Central cerebellar lesion
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.
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This question is part of the following fields:
- Neurology
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Question 22
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: Drug-induced parkinsonism
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 23
Incorrect
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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: Conduction block
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.
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This question is part of the following fields:
- Neurology
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Question 24
Incorrect
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A 11-year-old is referred to neurology due to episodes her GP feels are epileptiform. Her mother reports that she appears to just 'stop', sometimes even in mid conversation, for several seconds at random times during the day. During these episodes, she can be unresponsive to questioning and has no recollection of them. Which of these drugs is contraindicated in this condition?
Your Answer: Clonazepam
Correct Answer: Carbamazepine
Explanation:The patient’s history points to absence seizures. Carbamazepine has been shown to aggravate generalized seizure types, especially absence seizures, because it acts directly on the ventrobasal complex of the thalamus which is critical to the neurophysiology of absence seizures.
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This question is part of the following fields:
- Neurology
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Question 25
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 26
Correct
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A 71-year old gentleman presents with a subacute history of intermittent difficulty in walking, dry mouth, and variable slurring of speech. When the latter is severe he also has difficulty in swallowing. He has lost 3 kg in weight in the last 2 months. On examination he has bilateral mild ptosis, dysarthria, and proximal weakness of the upper and lower limbs, and he is areflexic. The degree of weakness is variable. Nerve conduction studies confirm the clinical suspicion of a neuromuscular junction disorder. Which of the following autoantibodies is likely to be the underlying cause of his neurological symptoms?
Your Answer: Anti-voltage-gated, calcium-channel antibody
Explanation:The clinical picture points to Lambert– Eaton myasthenic syndrome (LEMS) which often presents with weakness of the arms and legs. In LEMS, antibodies against voltage-gated calcium channels (VGCC) decrease the amount of calcium that can enter the nerve ending, causing autonomic symptoms like dry mouth and slurring of speech, as seen in this patient.
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This question is part of the following fields:
- Neurology
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Question 27
Incorrect
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Causes of dilated pupils include which of the following?
Your Answer: Pontine haemorrhage
Correct Answer: Ethylene glycol poisoning
Explanation:Ethylene glycol poisoning is the only poison listed that will cause dilation of the pupils whereas all the other listed conditions and poisons will present with small pupils other than in the case of Myotonic dystrophy wherein the patient will present with a cortical cataract.
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This question is part of the following fields:
- Neurology
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Question 28
Correct
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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 29
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:
- Neurology
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Question 30
Incorrect
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A 27 year-old ballet instructor presents with 1-day history of left-sided facial weakness and an increased sensitivity to noise in her left ear. She is very anxious because 2 years ago she had some problems with her vision and was told that multiple sclerosis was a possibility. Her medical history is significant only for type 1 diabetes mellitus managed with insulin, and she is also taking a combined oral contraceptive pill. Upon examination, she has a lower motor neuron lesion of the left VII (facial) nerve with Bell's phenomenon present and difficulty closing her left eye. There is no objective hearing loss and no sensory signs. Examination of the auditory meatus and canal is unremarkable. The remainder of the neurological examination appears normal. The next management step in her care should be:
Your Answer: Eighty mg oral prednisolone for five days
Correct Answer: Eye patch and artificial tears
Explanation:From the given history and physical examination findings, this patient has Bell’s palsy. There is no evidence to suggest involvement of any other cranial nerves, which might raise suspicion of a cerebello-pontine angle space-occupying lesion. With her history of possible optic neuritis, there is a possibility that the lesion is in fact a manifestation of multiple sclerosis, although this should be differentiated by examination of an upper motor neuron lesion (with sparing of the forehead facial muscles because of bilateral innervation). In light of her diabetes and the limited evidence of benefit from corticosteroid use, the most sensible first management step for her would be meticulous eye care to avoid corneal ulceration, as a result of the difficulty she is having closing her left eye.
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This question is part of the following fields:
- Neurology
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SESSION STATS - PERFORMANCE PER SPECIALTY
