Neurology Multiple Choice Questions With Explanations: Volume I
By Dr P A Shah
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Neurology Multiple Choice Questions With Explanations - Dr P A Shah
Cord
CHAPTER 1 - APPROACH
1.
A 16-year old girl complains of progressive difficulty in climbing stairs and pain in the legs for last two months. On examination, she has rash over her knuckles, weakness of neck muscles and proximal muscles of upper and lower limbs. There is mild tenderness of muscles. Deep tendon reflexes are normal. The most likely diagnosis is: (AIIMS)
1. Polymyositis
2. Muscular dystrophy
3. Poliomyelitis
4. Guillain-Barre syndrome
DISCUSSION:
Proximal, symmetrical weakness with normal reflexes suggests the diagnosis of myopathy (polymyositis or muscular dystrophy), and rules out poliomyelitis or Guillain-Barre syndrome.
A two months history suggests an acquired myopathic syndrome, so polymyositis is the most likely diagnosis. Muscular dystrophy is likely to present with long standing history, positive family history and selective involvement of certain group of muscles e.g. calf hypertrophy, facial weakness, ocular weakness etc.
ANSWER:
‘Polymyositis.’
REFERENCES:
Dalakas MC. Chapter 388. Polymyositis, Dermatomyositis, and Inclusion Body Myositis. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18th edition; 2012.
Stone J H. Chapter 19.11.7. Polymyositis and dermatomyositis. In: Warrell DA, Cox TM, Firth JD, eds. Oxford Textbook of Medicine. 5th edition; 2010.
2.
A 28-year-old laborer develops bilateral foot drop, progressing over one week to paralysis of both legs and trunk. There are no constitutional symptoms. Cerebrospinal fluid (CSF) shows albumin-leucocytic dissociation. The diagnosis is: (AIIMS)
1. Lead toxicity
2. Cyanide poisoning
3. Guillain-Barre syndrome
4. Alcoholic neuropathy
DISCUSSION:
Guillain-Barre syndrome (GBS) is an acute polyradiculoneuropathy, which often presents with ascending paralysis with weakness beginning in the feet and hands and migrating towards the trunk. CSF showing albumin-leucocytic dissociation (high proteins but normal cell count in CSF) is characteristic of GBS.
Lead toxicity can produce encephalopathy or motor weakness of extensors of arm (resembling radial nerve palsy). Cyanide poisoning affects respiratory muscles and is rapidly fatal. Alcoholic neuropathy is usually a chronic in presentation, and often complicated with nutritional neuropathies.
ANSWER:
‘Guillain-Barre syndrome.’
REFERENCES:
Katirji B, Koontz D. Chapter 76. Disorders of Peripheral Nerves. In: Daroff RB, Fenichel GM, Jankovic J, John C. Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6th edition; 2012.
Ropper AH, Samuels MA, Klein JP. Chapter 46. Diseases of the Peripheral Nerves. In: Ropper AH, Samuels MA, Klein JP. eds. Adams & Victor’s Principles of Neurology, 10th edition; 2014.
3.
A 19-year-old woman, who is a dancer, presents with progressive weakness of the toes and ankles. On examination, she has only mild paresis in those areas, loss of only ankle reflex, unresponsive plantar reflex and decreased sensation in the toes and feet. She has: (AIIMS)
1. Myasthenia gravis
2. Toxic polyneuropathy
3. Polymyositis
4. Thoracic spinal cord tumor
DISCUSSION:
Both myasthenia and polymyositis are pure motor disorders, therefore unlikely to have abnormal sensations. Thoracic spinal cord tumor may have both motor and sensory signs, but motor involvement will be of upper motor neuron type, and there should have been extensor plantar response with brisk reflexes in the lower limbs. Myelopathies typically show sensory level over the trunk.
This patient is showing lower motor neuron signs with abnormal sensations, starting in distal areas. Neuropathies typically show signs and symptoms in stocking and glove distribution. Of the options provided, toxic neuropathy is the most likely diagnosis.
ANSWER:
‘Toxic polyneuropathy.’
REFERENCES:
Amato AA, Barohn RJ. Chapter 384. Peripheral Neuropathy. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18edition
Hughes R A C, Thomas P K. Chapter 24.16. Diseases of the peripheral nerves. In: Warrell DA, Cox TM, Firth JD, eds. Oxford Textbook of Medicine. 5th edition.
4.
A patient had unilateral lower motor neuron type of facial nerve paralysis with loss of taste sensation. The duration of illness had been one month. After taking treatment, he noticed that during food intake, he was having tears from the affected eye. No other neurological deficit was found on examination. The most likely diagnosis is: (AIIMS)
1. Ramsay-Hunt syndrome
2. Crocodile tears syndrome
3. Millard-Gubler’s syndrome
4. Weber’s syndrome
DISCUSSION:
Gusto-lacrimation, or crocodile tear syndrome,
is a rare complication of Bell’s palsy. The mechanism appears to be a misdirection of regenerating gustatory fibers destined for the salivary glands, so that they become secretory fibers to the lacrimal gland and cause ipsilateral tearing while the patient is eating.
ANSWER:
‘Crocodile tear syndrome.’
DISCUSSION:
McCoy FJ, Goodman RC. The crocodile tear syndrome. Plast Reconstr Surg. 1979 Jan;63(1):58–62.
Rucker J C. Chapter 70. Cranial Neuropathies. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC. eds. Bradley’s Neurology in Clinical Practice, 6th edition; 2012.
5.
An acute peripheral facial neuropathy associated with erythematous vesicular rash of the skin of the ear canal, auricle, and mucous membrane of the oropharynx is also known as: (AIIMS)
1. Bell’s palsy
2. Ramsay Hunt syndrome
3. Melkersson-Rosenthal syndrome
4. Gradenigo syndrome
DISCUSSION:
This description suggests Ramsay Hunt syndrome. It is due to the infection of the geniculate ganglion by varicella-zoster virus.
Gradenigo syndrome results from inflammation of the petrous apex and causes facial nerve palsy in combination with trigeminal and abducens nerve impairment. Melkersson–Rosenthal syndrome is characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip), and plication of tongue.
ANSWER:
‘Ramsay Hunt syndrome.’
REFERENCES:
Bhupal HK. Ramsay Hunt syndrome presenting in primary care. Practitioner. Mar 2010;254(1727):33–5, 3.
Beal M, Hauser SL. Chapter 376. Trigeminal Neuralgia, Bell’s Palsy, and Other Cranial Nerve Disorders. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 18th edition; 2012.
6.
A middle-aged man present with progressive weakness and atrophy of muscles of hands and forearms. On examination he is found to have slight spasticity of the legs, generalized hyperreflexia and increased signal in the corticospinal tracts on T2 weighted MRI. The most likely diagnosis is: (AIIMS)
1. Multiple sclerosis
2. Amyotrophic lateral sclerosis
3. Subacute combined degeneration
4. Progressive spinal muscular atrophy
DISCUSSION:
Spinal muscular atrophies are examples of motor neuron diseases, affecting only lower motor neurons. Subacute combined degeneration affects posterior column (hence sensory symptoms) and corticospinal tracts. Multiple sclerosis is a demyelinating disorder of central nervous system mainly affecting eyes and cerebellum.
This patient has a combination of upper motor neuron (spasticity, hyperreflexia) and lower motor neuron (atrophy) signs. In addition, there is no evidence of sensory involvement. Therefore, the most