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Question 1
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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 2
Correct
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Which of the following physical findings is least typical on a patient with multiple sclerosis?
Your 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 3
Correct
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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: 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 4
Correct
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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: 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 5
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 6
Incorrect
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A 10-year-old gentleman is referred with a six month history of daily headache, which is mostly frontal in location and occasionally associated with nausea. He has been taking paracetamol 3 g daily, aspirin 300 mg thrice daily, and codeine 40 mg thrice daily, all of which have had only a temporary effect. He has a two year history of depression treated with paroxetine. No abnormalities were found on examination. What is the most likely diagnosis?
Your Answer:
Correct Answer: Analgesic misuse headache
Explanation:Because of the patient’s history of chronic analgesic use of daily paracetamol intake, the most likely diagnosis of this case is Analgesic misuse headache. In these cases, the headache is only temporarily relieved by analgesics. Treatment involves gradual withdrawal of analgesics.
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This question is part of the following fields:
- Neurology
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Question 7
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 8
Incorrect
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A 13-year-old boy complains of several episodes of collapse. He describes the majority of these episodes occurring when he is laughing. He states that he loses power in his legs and falls to the ground. He is alert throughout and recovers quickly. He also describes excessive daytime sleepiness with episodes in the morning of being awake but being unable to move his body. Examination is unremarkable. A diagnosis of narcolepsy is made. Which of the following is the first line treatment for excessive daytime sleepiness?
Your Answer:
Correct Answer: Modafinil
Explanation:Narcolepsy is a rare condition characterised by excessive daytime sleepiness, sleep paralysis, hypnagogic hallucinations, and cataplexy (sudden collapse triggered by emotion such as laughing or crying). There is no cure for narcolepsy. Treatment options include stimulants, such as methylphenidate (Ritalin) or modafinil (Provigil), antidepressants, such as fluoxetine (Prozac), citalopram (Celexa), paroxetine (Paxil), sertraline (Zoloft) and sodium oxybate (Xyrem). Modafinil has replaced methylphenidate and amphetamine as the first-line treatment of excessive daytime sleepiness (EDS).
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This question is part of the following fields:
- Neurology
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Question 9
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 10
Incorrect
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A 53 year-old dancer presents to the ED with increasing weakness. She has no pertinent past medical history aside from a recent diarrhoeal illness, which she attributes to an undercooked chicken meal. Her husband says that she has been unable to get up out of a chair for the past day. Upon examination, there is bilateral limb weakness and areflexia noted, but it is more severe in the lower limbs. You notice that if she lies flat in the bed, her oxygen saturations fall by around 2% on the pulse oximeter and she is unable to perform spirometry. Which of the following represents the most appropriate immediate management of choice in this patient?
Your Answer:
Correct Answer: ITU review for consideration of ventilation
Explanation:This woman has a history that is suggestive of Guillain– Barré syndrome. This may be precipitated by Campylobacter, and her history of recent diarrhoeal illness is pointing towards that. Certain features point to a poor prognosis, including rapidity of onset, reduced vital capacity or respiratory failure, age >40 and reduced amplitude of compound muscle action potential. Her inability to perform spirometry and desaturating whilst lying flat are suggestive of impending respiratory muscle weakness. Review for consideration of ventilation is recommended. Further management of choice for Guillain-Barre syndrome is IV immunoglobulins. Steroids have no value in the treatment of the condition.
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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 65-year-old real estate broker presents with a tremor. Which one of the following features would suggest a diagnosis of essential tremor rather than Parkinson's disease?
Your Answer:
Correct Answer: Tremor is worse when the arms are outstretched
Explanation:Difficulty in initiating movement (bradykinesia), postural instability and unilateral symptoms (initially) are typical of Parkinson’s. Essential tremor symptoms are usually worse if arms are outstretched and eased by rest and alcohol.
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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 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:
Correct 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 13
Incorrect
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A 44-year-old hairdresser with a history of myasthenia gravis is admitted to the Emergency Department. She is currently taking pyridostigmine, but there has been a significant worsening of her symptoms following antibiotic treatment for a chest infection. On examination she is dyspnoeic and cyanotic with quiet breath sounds in both lungs. Other than respiratory support, what are the two other treatments of choice?
Your Answer:
Correct Answer: Plasmapheresis or intravenous immunoglobulins
Explanation:This patient is having a myasthenic crisis. Opinions vary as to whether plasmapheresis or intravenous immunoglobulins should be given first-line. Plasmapheresis usually works much faster, but is more costly due to equipment.
Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 85-90% of cases. Myasthenia gravis is more common in women (2:1). -
This question is part of the following fields:
- Neurology
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Question 14
Incorrect
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A patient presented with acute onset of mild right hemiparesis affecting the body. He also has evidence of sensory loss on the right hand side. There is evidence of Horner’s syndrome and sensory loss on the face on the left hand side. Which of the following structures are involved?
Your Answer:
Correct Answer: Brain stem
Explanation:The patient is presenting with symptoms of lateral medullary syndrome also known as Wallenberg’s syndrome or posterior inferior cerebellar artery syndrome, where the symptoms are due to an ischemia in the brainstem. The classical symptoms include contralateral sensory deficits of the trunk region paired with ipsilateral facial sensory deficits.
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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 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:
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 16
Incorrect
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A 48-year-old hairdresser presents to the GP with loss of sensation over the lateral three and a half fingers of her right hand, tenderness over her right forearm, and inability to make a tight fist. She complains of pain in her right arm when twisting door handles anticlockwise. Phalen’s and Tinel’s tests are negative. She is otherwise neurologically intact. Which of the following is the most likely diagnosis?
Your Answer:
Correct Answer: Pronator teres syndrome
Explanation:Entrapment of the median nerve by pronator teres causes a median nerve neuropathy, which is worse during pronation of the forearm. Examination should involve excluding carpal tunnel syndrome and pronation of the affected forearm against resistance, which brings on the pain. Unlike carpal tunnel syndrome, the median nerve proximal to the wrist may be tender to palpation.
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This question is part of the following fields:
- Neurology
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Question 17
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 18
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:
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 19
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:
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 20
Incorrect
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A 14 year-old is brought to the ED after being hit on the head with a cricket ball during a match. His teacher describes that he initially collapsed on the ground and complained of a sore head. Two minutes later, he got up and said he felt OK and continued playing. However, 30 minutes later he suddenly collapsed and lost consciousness. What injury is he most likely to have sustained?
Your Answer:
Correct Answer: Extradural haematoma
Explanation:A lucid interval, in which the patient portrays a temporary improvement in condition after a traumatic brain injury, is especially indicative of an epidural haematoma.
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This question is part of the following fields:
- Neurology
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Question 21
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:
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 22
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:
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 23
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:
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 24
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 25
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:
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 26
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 27
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:
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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Question 28
Incorrect
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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:
Correct 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 29
Incorrect
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A 59-year-old surgeon presents with a progressive paraesthesia and numbness in both feet, which have deteriorated over the last six months. He has a 10 year history of type 2 diabetes mellitus and had cervical spondylosis, for which he underwent surgery eight years ago. He also confessed to drinking approximately 40 units of alcohol weekly. On examination he had mild bilateral weakness of foot dorsiflexion and both ankle reflexes were absent. There was absent sensation to light touch to mid-shin level with loss of joint position sensation in the toes and absent vibration sensation below the hips. He had a marked sensory ataxia and pseudoathetosis of the upper limbs. He had no evidence of a retinopathy and urinalysis was normal. Which of the following is the most likely diagnosis?
Your Answer:
Correct Answer: Vitamin B 12 deficiency
Explanation:Diabetic peripheral neuropathy usually goes in parallel with retinopathy and nephropathy. It is also slowly progressive and affects mainly the spinothalamic pathway.
Alcohol induced peripheral neuropathy is also slowly progressive and affects mainly the spinothalamic pathway.
Vitamin B 12 deficiency usually causes a more rapidly progressive neuropathy with dorsal column involvement (joint position and vibration involvement with sensory ataxia and pseudoathetosis of upper limbs). -
This question is part of the following fields:
- Neurology
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Question 30
Incorrect
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Which of the following statements regarding restless leg syndrome is not true?
Your Answer:
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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