Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy: Clinical features and pathological study of the sural nerves

We investigated clinical and pathological features of the sural nerves of 5 patients with the ataxic form of chronic inflammatory demyelinating polyra diculoneuropathy (CIDP) and compared these features with those of chronic a taxic neuropathies due to other causes. The CIDP patients presented with sl owly progressive ataxia with deep sensory impairment. The durations of the symptoms from onset were relatively short in CIDP (4-8 months) and cancer ( 3 and 10 months), but long in chronic idiopathic ataxic neuropathy (24-260 months). Corticosteroid therapy elicited a good response in all the patient s with CIDP, but a poor response in the patients with other ataxic neuropat hies. Sural nerve biopsy of CIDP patients showed a slight or moderate loss of myelinated fiber. This report suggests that ataxic form of CIDP is a ste roid-responsive ataxic neuropathy, and large myelinated fibers of the sural nerves in ataxic form of CIDP were better preserved than those in nerves w ith other chronic ataxic neuropathies. Copyright (C) 2001 S. Karger AG, Bas el.