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Question 1
Incorrect
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A 6-year-old boy is referred by his GP to the neurology clinic with abnormal movements. His mother noticed that for the last year, the boy has been falling over more and more frequently. He has also been having increasingly slurred speech. These have been getting progressively worse. He has had recurrent chest infections in his childhood. What is the most likely diagnosis?
Your Answer: Infantile-onset spinocerebellar ataxia
Correct Answer: Ataxic telangiectasia
Explanation:Ataxic telangiectasia is an inherited combined immunodeficiency disorder that is characterised by cerebellar ataxia and telangiectasia as seen in this child, as well as frequent infections as noted in this child’s history. The other differentials would not present with this clinical picture:
Friedreich’s ataxia and Infantile-onset spinocerebellar ataxia do not present with immune problems, whereas Cerebral palsy and Di-George Syndrome do not present with ataxia.
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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 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 3
Correct
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A 18-year-old gentleman is referred to dermatology. He has around 10 hyperpigmented macules on his torso which vary in size from 1.5-5 cm in size. His GP also noted some freckles in the groin region. He is also currently under orthopaedic review due to a worsening scoliosis of the spine. His father suffered from similar problems before having a fatal myocardial infarction two years ago. Which chromosome is most likely to have a gene defect?
Your Answer: Chromosome 17
Explanation:The patient’s history and presentation and familial history, meets the diagnostic criteria for Neurofibromatosis type I, presenting with neurofibromas noted in this patient as hyperpigmented macules and freckles, musculoskeletal disorders like the scoliosis in this case, and a familial history. Neurofibromatosis type I is caused by a mutation on Chromosome 17.
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This question is part of the following fields:
- Neurology
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Question 4
Incorrect
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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: Creatine kinase
Correct 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 5
Incorrect
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Which of the following features is not associated with an oculomotor nerve palsy?
Your Answer: Pain if due to a posterior communicating artery aneurysm
Correct Answer: Miosis
Explanation:Oculomotor nerve palsy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. A complete oculomotor nerve palsy will result in a characteristic down and out position in the affected eye. This is because the lateral rectus (innervated by the sixth cranial nerve) and superior oblique (innervated by the fourth cranial or trochlear nerve), is unantagonized by the paralyzed superior rectus, inferior rectus and inferior oblique. The affected individual will also have a ptosis, or drooping of the eyelid, and mydriasis (pupil dilation), not miosis.
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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 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 7
Incorrect
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Which of the following types of motor neuron diseases carries the worst prognosis?
Your Answer: Spinocerebellar ataxia
Correct Answer: Progressive bulbar palsy
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 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy. In some patients however, there is a combination of clinical patterns. In progressive bulbar palsy there is palsy of the tongue and muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei. This carries the worst prognosis.
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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 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 9
Correct
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A 48-year-old hairdresser presented to her GP complaining of tingling in the right palm and right wrist pain. She had intermittently experienced these symptoms; however, recently they had been keeping her awake all night. She had noticed a reduction in grip and was struggling to work at the salon. Otherwise, she felt well in herself and had not noticed any weakness in the other hand or lower limb. Her weight was stable, and she denied any neck problems or swallowing difficulties. She had a past medical history of hypothyroidism and hypertension and took regular thyroxine, Bendroflumethiazide and ibuprofen. She was a non-smoker and rarely drank alcohol. On examination, she appeared alert and orientated. Fundoscopy and cranial nerve examination were all normal and neck movements were full. On examination of the upper limb, there was wasting over the right thenar eminence and fasciculations with a small burn over the right index finger. There was weakness of thumb abduction and opposition, with loss of pinprick and light touch sensation over the thumb, index and middle finger in the right hand. Nerve conduction studies showed absent sensory action potential in right median palmar branches and denervation of the right abductor pollicis brevis. What is the most likely diagnosis?
Your Answer: Median nerve palsy
Explanation:The history is consistent with carpal tunnel syndrome (CTS) arising as a result of pressure on the median nerve in the carpal tunnel. The median nerve supplies the muscles of the thenar eminence: the abductor pollicis (C7, C8), flexor pollicis brevis and opponens pollicis, and the lateral two lumbricals. The nerve conduction studies confirm marked denervation and absent sensory potentials within the median nerve territory.
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This question is part of the following fields:
- Neurology
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Question 10
Correct
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A 38-year-old female patient is brought into the emergency department with a 5 day history of altered personality, and visual and auditory hallucinations. On palpation of the abdomen, a mass is felt in the left iliac fossa. Ultrasound of the abdomen suggests a left ovarian tumour. Her basic observations are as follows: Oxygen saturation 99% on air Heart rate 98 beats/minute Respiratory rate 28 breaths/minute Temperature 37.9 °C What is the most likely diagnosis?
Your Answer: Anti-NMDA receptor encephalitis
Explanation:The case presents with an underlying ovarian tumour, associated with psychiatric symptoms; thus, an organic illness must first be ruled out before considering the other conditions listed which often present with psychiatric features without an underlying organic disease. Among the listed conditions Anti-NMDA receptor encephalitis is the only condition that presents with psychiatric features including agitation, hallucinations, delusions and disordered thinking that is associated with tumours 50% of the time.
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This question is part of the following fields:
- Neurology
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Question 11
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: Magnetic resonance imaging (MRI) brain and cervical spine
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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Question 12
Incorrect
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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: C6
Correct 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 13
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 14
Incorrect
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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: Sub-acute combined degeneration of the cord
Correct 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 15
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:
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 16
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:
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 17
Incorrect
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A 40 year-old lawyer suffered a road traffic accident. MRI reveals that he may have hemisection of the spinal cord. Which of the following findings is most likely to occur?
Your Answer:
Correct Answer: Ipsilateral hyperreflexia
Explanation:Spinal cord hemisection, also known as Brown-Sequard syndrome, is associated with symptoms affecting one spinothalamic and one corticospinal tract. Symptoms include ipsilateral paralysis, loss of vibration and position sense, and hyperreflexia below the level of the lesion. Contralateral loss of pain and temperature sensation is also seen, usually beginning 2-3 segments below the level of the lesion.
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This question is part of the following fields:
- Neurology
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Question 18
Incorrect
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An iv-drug abuser sustains an open head injury and is admitted overnight for observation. The next morning she is ok and is discharged. A week later she is re-admitted with fluctuating hard neurological signs. Blood results show neutrophilia and raised C-reactive protein (CRP). Which of the following is the best initial treatment step?
Your Answer:
Correct Answer: Computed tomography (CT) scan with contrast
Explanation:Increased WBC count and CRP suggest infection. But with the fluctuating hard neurological signs, there is suspicion of the presence of a cerebral mass, which is an indication for requesting for a CT scan with contrast, to rule out an abscess or haematoma. The lumbar puncture can be considered after the CT scan.
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This question is part of the following fields:
- Neurology
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Question 19
Incorrect
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Which of the following is MOST suggestive of a lesion of the sciatic nerve?
Your Answer:
Correct Answer: Foot drop
Explanation:The sciatic neve originates in the sacral plexus, mainly from spinal segments L5-S2. It supplies muscles that cause extension of the thigh and flexion of the leg. It divides into two main branches, the tibial nerve and common peroneal nerve, which are responsible for all foot movements. Anterior thigh and medial leg sensory loss is typical of a femoral nerve lesion. The femoral nerve also mediates flexion of the hip.
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This question is part of the following fields:
- Neurology
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Question 20
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:
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 21
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:
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 22
Incorrect
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Which one of the following is a contraindication to the use of a triptan in the management of migraine?
Your Answer:
Correct Answer: A history of ischaemic heart disease
Explanation:A history of ischaemic heart disease in a contraindication for prescribing triptans because they act by constricting cerebral and also coronary vessels, increasing the risk of stroke.
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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 22 year-old university graduate presented with progressive unsteadiness during walking over the last year. She had been otherwise healthy apart from recent difficulty hearing her lecturer in classes. She took no prescription medication but had occasionally taken cocaine during her first year of college. She also admits to drinking up to 30 units of alcohol per week and smoked 10 cigarettes per day. Her parents were both well, but her father's sister had problems with walking before she died. Examination reveals normal tone and power throughout all four limbs. Reflexes were normal in the upper limbs but decreased at the knees and absent at the ankles. Coordination was normal in all four limbs but her gait was ataxic. Sensation in the upper limbs was normal but decreased vibratory sensation and proprioception was noted to the ankles bilaterally. What is the most likely diagnosis?
Your Answer:
Correct Answer: Friedreich’s ataxia
Explanation:Friedreich’s ataxia is an autosomal recessive disorder that usually begins before the end of the teens. It has an estimated prevalence in Europe of 1 in 50,000 and life expectancy is around 40–50 years. Neurological features include a progressive ataxia, cerebellar dysarthria, lower limb areflexia, decreased vibratory sensation and proprioception, and pyramidal weakness. Pes cavus and scoliosis are also both seen. Cardiomyopathy occurs in over 70% of cases. Less common features include optic atrophic, diabetes mellitus, and deafness.
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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 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:
Correct 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 25
Incorrect
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A 30-year-old painter presents with a burning pain in both feet, which has deteriorated over the last six months. He drinks 60 units of alcohol weekly and has a family history of pernicious anaemia. On examination he has impairment of all modalities of sensation in both feet but particularly pain, temperature and absent ankle jerks. What is the most likely diagnosis?
Your Answer:
Correct Answer: Alcoholic peripheral neuropathy
Explanation:Because of the patient’s history of excessive alcohol consumption, there is a strong suspicion of alcoholic peripheral neuropathy. In the UK, alcohol abuse and diabetes are the most common causes of peripheral neuropathy.
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This question is part of the following fields:
- Neurology
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Question 26
Incorrect
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Which of the following physical findings is least typical on a patient with multiple sclerosis?
Your Answer:
Correct Answer: Decreased tone
Explanation:Attacks or exacerbations of multiple sclerosis (MS) are characterized by symptoms that reflect central nervous system (CNS) involvement, hence upper motor neuron symptoms are seen.
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This question is part of the following fields:
- Neurology
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Question 27
Incorrect
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Which of the following is true concerning baclofen?
Your Answer:
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 28
Incorrect
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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:
Correct 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 29
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:
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 30
Incorrect
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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:
Correct 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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SESSION STATS - PERFORMANCE PER SPECIALTY
