The Steroid Treatment of Hereditary Motor and Sensory Neuropathy

Abstract

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 adrenalcorticosteroids in patients who definitely have familial HMSN-I do not seem to be justified.

Key words

Methylprednisolone - Familial polyneuropathy