Corticosteroid therapy for acute attacks in hereditary neuropathy with liability to pressure palsies―A case report

Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by repeated acute palsies caused by pressure neuropathy ; however, only conservative management, such as preventing trauma, has been used for treating symptoms. Here, we describe the beneficial use of corticosteroid therapy in a 41–year–old patient with HNPP, who had been experiencing repetitive episodes of acute weakness in his right shoulder, over a period of 27 years. During one episode, we administered corticosteroid half–pulse therapy (methylprednisolone sodium succinate at 500mg/day, for two days), and he became symptom–free within a few days. The corticosteroid effects on acute HNPP are possibly mediated by edema and inflammation improvement, although some inherited structural alterations in the myelin sheaths may also induce immune–mediated responses.