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Intestinal pseudo-obstruction in adult spinal muscular atrophy

✍ Scribed by Dr. Victor Ionasescu; Dr. James Christensen; Dr. Michael Hart

Publisher
John Wiley and Sons
Year
1994
Tongue
English
Weight
352 KB
Volume
17
Category
Article
ISSN
0148-639X

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✦ Synopsis

A 42-year-old woman with negative family history had the insidious onset of weakness in her lower extremities 8 years before, in 1983. The disorder slowly progressed to include cramps and muscle twitches. The diagnosis of adult spinal muscular atrophy (SMA) was made when electromyography showed large rapidly firing motor unit potentials, positive waves, and fibrillation potentials, and when muscle biopsy of the quadriceps revealed severe alterations consistent with neurogenic atrophy. The patient also had severe chronic constipation for many years. More recently she had developed unremitting diarrhea. Gastrointestinal studies showed no evidence of peristaltic contractions in the rectum, delayed gastric emptying, and abnormal jejunal manometry with altered propagation of the migrating myoelectrical complex.

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