A 62-year-old Caucasian man comes to the physician because of progressive weight loss and right lower extremity weakness for the last two months. On further questioning, he tells you that he has been having frequent leg cramps in the right leg. These cramps are most prominent in the morning, after he wakes up. He also has difficulty with swallowing and chewing. He denies any problems with bowel or bladder function. His other medical problems include hypertension and hypercholesterolemia. He does not use tobacco. His family history is not significant. His vital signs are within normal limits. The physical examination reveals weakness in his right lower leg, atrophy and fasciculation of the thigh and calf muscles, a hyperactive knee jerk, and tongue fasciculations. The rest of his neurological examination, including the sensory examination, is normal.
Which of the following treatment has been shown to be beneficial in these patients?

Question

A 62-year-old Caucasian man comes to the physician because of progressive weight loss and right lower extremity weakness for the last two months.  On further questioning, he tells you that he has been having frequent leg cramps in the right leg.  These cramps are most prominent in the morning, after he wakes up.  He also has difficulty with swallowing and chewing.  He denies any problems with bowel or bladder function.  His other medical problems include hypertension and hypercholesterolemia.  He does not use tobacco.  His family history is not significant.  His vital signs are within normal limits.  The physical examination reveals weakness in his right lower leg, atrophy and fasciculation of the thigh and calf muscles, a hyperactive knee jerk, and tongue fasciculations.  The rest of his neurological examination, including the sensory examination, is normal.
Which of the following treatment has been shown to be beneficial in these patients?

Quizplus · Accepted Answer

Explanation: Riluzole is a glutamate inhibitor that is currently approved for the management of amyotrophic lateral sclerosis. Although it cannot arrest the underlying pathological process, it may prolong survival and delay the need for a tracheostomy. (Choices B, C, and D)Amyotrophic lateral sclerosis is a neurodegenerative disease. Agents such as corticosteroids, intravenous immunoglobulins, and cyclophosphamide, have no role in its management. These agents may be useful in immunologically-mediated neurological diseases such as multiple sclerosis and Guillain-Barre syndrome. (Corticosteroids are used to treat acute exacerbations of multiple sclerosis, while plasmapheresis and intravenous immunoglobulins are the main treatment modalities of Guillain-Barre syndrome.) Educational Objective: Riluzole is a glutamate inhibitor that is currently approved for the management of amyotrophic lateral sclerosis.

A 62-Year-Old Caucasian Man Comes to the Physician Because of Progressive