Chronic inflammatory demyelinating polyradiculoneuropathy: unusual clinical features and therapeutic responses.

Abstract

We present three patients with atypical chronic inflammatory demyelinating polyradiculoneuropathy and discuss the management of patients who appear treatment resistant or present with unusual manifestations. The clinical features of the patients included massive nerve root hypertrophy causing myelopathy and movement-provoked paresthesia, pupillary dysfunction, visual loss due to increased intracranial pressure, and focal brachial plexus involvement. Each patient ultimately required prolonged courses of immune modulating therapy before benefit was attained, illustrating the importance of intensive and prolonged treatment combined with objective assessment of response to therapy.