October 27, 2010

PLAB Question 17


Match the following mechanism or pattern with the appropriate disease:

Progressive degeneration of anterior horn cells resulting in progressive muscle weakness, wasting and fasciculations

a) Rhabdomyolysis
b) Guillain-Barré syndrome
c) Charcot-Marie-Tooth
d) Duchenne muscular dystrophy
e) Becker muscular dystrophy
f) Myasthenia gravis (MG)
g) Amyotrophic lateral sclerosis
h) Dermatomyositis
i) Myotonic dystrophy
j) Amyloid

The correct answer is G

Amyotrophic lateral sclerosis is a progressive neuromuscular condition characterized by weakness, muscle wasting, fasciculations and increased reflexes. The disease is most commonly diagnosed in middle age and affects more men than women. It usually presents with problems in dexterity or gait resulting from muscle weakness. Difficulty in speaking or swallowing is the initial symptom in the bulbar form of the disease.

Over a period of months or years, patients with ALS develop severe, progressive muscular weakness and other symptoms caused by loss of function in both upper and lower motor neurons.

Sphincter control, sensory function, intellectual abilities and skin integrity are preserved. Patients become completely disabled, often requiring ventilatory support and gastrostomy. Death usually occurs within five years of diagnosis and is attributed to respiratory failure or cachexia.

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