Familial steroid-responsive neuropathy

A hereditary sensory neuropathy different from any of the four classic types was diagnosed in three members of one family. Biopsy in one of the patients revealed histologic inflammatory cell infiltration and the presence of perivascular cuffing in muscle, similar to that seen in nerves affected by idiopathic polyneuritis. The response to steroids was dramatic, unlike that noted with nonvasculitic neuropathies. Signs of vasculitis may suggest reversibility.