The steroid treatment of hereditary motor and sensory neuropathy.
A placebo or methylprednisolone (45-60 mg/M2) was administered in a crossover study as a single morning dose on alternate days to fourteen patients who had a familial progressive polyneuropathy that either began or was maximum in the distribution of the peroneal nerves. Neither the patients nor the examining physician were told whether the patient was taking a placebo or steroid but the steroid side-effects made a double-blind trial impossible. Patients were evaluated at the initiation of the study, the time of crossover, and at the conclusion of the study. Five patients with proven HMSN-I completed the study; eight patients with HMSN-I were placed on a placebo or on steroids for a six-month-period. In neither group was there any evidence of a significant increase in nerve conduction times, a decrease in terminal latencies, an increase in strength or a decrease in sensory loss when evaluated by quantitative methods. These patients were subject to a high incidence of complications from steroids possibly because of root hypertrophy, relative inactivity, and high CSF proteins. These included excessive weight gain, a compression fracture of T-12; cord compression from enlarged nerve roots; myopathy; pseudotumor; and psychiatric disturbances. Considering the lack of benefit of steroids and the high incidence of complications due to the medication further trials of adrenal-corticosteroids in patients who definitely have familial HMSN-I do not seem to be justified.
D000293:Adolescent; D000328:Adult; D002986:Clinical Trials as Topic; D004311:Double-Blind Method; D006801:Humans; D008475:Median Nerve / Q000187:drug effects; D008775:Methylprednisolone / Q000627:therapeutic use*; D009046:Motor Neurons / Q000187:drug effects; D009132:Muscles / Q000294:innervation; D009133:Muscular Atrophy / Q000188:drug therapy / Q000235:genetics*; D009431:Neural Conduction / Q000187:drug effects; D010543:Peroneal Nerve / Q000187:drug effects; D011115:Polyneuropathies / Q000188:drug therapy / Q000235:genetics*; D011930:Reaction Time / Q000187:drug effects; D012677:Sensation / Q000187:drug effects; D009435:Synaptic Transmission / Q000187:drug effects; D014459:Ulnar Nerve / Q000187:drug effects
D008775:Methylprednisolone