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Question 1
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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: 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 2
Correct
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Which of the following statements regarding restless leg syndrome is not true?
Your 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 3
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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: 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 4
Correct
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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: 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 5
Correct
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A 67 year-old attorney presents with a 2 month history of tremors affecting his left arm. He suffers from depressive psychosis for the last 10 years, for which he has been receiving intermittent chlorpromazine and amitriptyline but has not been on any therapy for the last 4 months. He describes that his two brothers also had tremors. Upon examination, he has a resting tremor of his left hand with cogwheel rigidity of that arm and mild generalized bradykinesia. Which of the following is the most likely diagnosis?
Your Answer: Idiopathic Parkinson’s disease
Explanation:The most likely diagnosis is idiopathic Parkinson’s disease because of the unilateral presentation. In addition, cogwheel rigidity is a classic presenting symptom. Neuroleptic-induced parkinsonism is usually bilateral and symmetrical. Essential tremors do not cause rest tremors.
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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 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 tract
Correct 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 7
Correct
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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: 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
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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: 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 9
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:
- Neurology
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Question 10
Incorrect
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Which of the following physical findings is least typical on a patient with multiple sclerosis?
Your Answer: Brisk reflexes
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 11
Incorrect
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Causes of dilated pupils include which of the following?
Your Answer: Argyll Robertson pupil
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 12
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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Question 13
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 + memantine
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 14
Correct
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A 41-year-old gentleman undergoes a temporal lobectomy after the discovery of a brain tumour. Which one of the following consequences would be least likely to develop?
Your Answer: Astereognosis
Explanation:Astereognosis is associated with lesions to the parietal lobe, not the temporal lobe, so this symptom would not arise in this patient.
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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 25-year-old woman with Charcot-Marie-Tooth disease (type 1) asks how likely it is that any future children will have the disease. What is the most accurate answer?
Your Answer: 25%
Correct Answer: 50%
Explanation:Because Charcot-Marie-Tooth disease (type 1) is an autosomal dominant condition; therefore, there is a 50% chance that the children of this patient will be affected.
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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 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 smaller 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 17
Correct
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A 32 year-old man presents with his first generalized tonic-clonic seizure (GTCS). He has been complaining of headaches for the past 2 weeks, although he has been able to continue working at his job. Upon examination, he has mild left hemiparesis and bilateral extensor plantar responses. General examination is otherwise unremarkable. An urgent CT scan of the brain shows a 5cm multicentric mass lesion in the right frontal lobe with surrounding vasogenic oedema and some hemisphere shift. Which of the following is the most likely underlying pathology?
Your Answer: Glioblastoma
Explanation:Glioblastoma multiforme, also considered as grade IV astrocytoma, is the most malignant form of the tumour and accounts for about 20% of all cerebral tumours. These often remain clinically silent until they have reached a large enough size. In adults, glioblastoma multiforme usually occurs in the cerebral hemispheres, especially the frontal and temporal lobes of the brain. About half occupy more than one hemisphere at presentation, and some are multicentric. Biopsy shows high cellularity with mitoses, pleomorphism, and vascular hyperplasia. Prognosis is extremely poor, with only 20% surviving beyond 1 year and 10% beyond 2 years.
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This question is part of the following fields:
- Neurology
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Question 18
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A 22-year-old male has had complex tics since childhood. He repeatedly bends his knees and rubs his nose. He is prone to loud vocalisations, sometimes including swear-words. A diagnosis of Gilles de la Tourette syndrome has been made. Which of the following is the best treatment option?
Your Answer: Risperidone
Explanation:Gilles de la Tourette syndrome is the most severe and rare of the tic syndromes, consisting of multiple tics involving both motor actions and vocalisation. Onset is usually in childhood. Symptoms include utterance of obscenities (coprolalia); echolalia (repetition of another person’s spoken words) and palilalia (involuntary repetition of words, phrases, or sentences).
The underlying cause is unknown, with no particular imaging or standard histopathological abnormalities having been identified. The EEG shows non-specific abnormalities in about half of patients. However, more recent immunocytochemical studies have suggested altered dopamine uptake in the striatal system.
Risperidone is an effective therapeutic option without the effects associated with chlorpromazine and haloperidol. -
This question is part of the following fields:
- Neurology
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Question 19
Incorrect
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A 17-year-old girl presented with fever, headache and photophobia. Cerebrospinal fluid examination reveals: Opening pressure 260 mm H20 (50-180) Total protein 0.8 g/l (0.15-0.45) Glucose 4.2 mmol/l (3.3-4.4) White cell count 60 per ml (<5) Lymphocytes 90% Plasma glucose 6.4 mmol/l (3.0-6.0) Which of the following is the most likely diagnosis?
Your Answer: Viral encephalitis
Correct Answer: Viral meningitis
Explanation:Normal cerebrospinal fluid (CSF) glucose together with lymphocytosis, an increased opening pressure and raised CSF protein are typical of a viral meningitis.
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This question is part of the following fields:
- Neurology
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Question 20
Incorrect
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Three days after being admitted for a myocardial infarction, a male patient complains of sudden change in vision. The medical registrar examines the patient and finds that the patient’s vision in both eyes is significantly reduced although the patient still claims that he can see. The pupils are equal in size, and the pupil responses are normal with normal fundoscopy. Significantly, the patient has now developed atrial fibrillation. A referral is made to the ophthalmologist who confirms bilateral blindness. Despite this, however, the patient fervently believes that he can see and has taken to describing objects that he has never seen previously, in discriminating detail. What is the most likely diagnosis?
Your Answer: Distal basilar artery occlusion
Correct Answer: Bilateral occipital cortex infarction
Explanation:Bilateral occipital cortex infarction will produce varying degrees of cortical blindness, wherein the patient has no vision but fundoscopy findings are normal. When there are extensive lesions, patients my present with denial of their condition, known as Anton’s Syndrome, and begin to describe objects that they have never seen before.
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This question is part of the following fields:
- Neurology
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