Distal Chronic Inflammatory Demyelinating Polyradiculoneuropathy: 
A Variant with Predominant Distal Weakness

J Korean Neurol Assoc > Volume 22(3); 2004 > Article

Journal of the Korean Neurological Association 2004;22(3): 219-225.

원위부 만성염증성탈수초성다발성신경병증: 원위부 근력 약화가 우세한 변형

배종석, 김병준

지방공사 강남병원 신경과, 성균관대학교 의과대학 신경과학교실

Distal Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Variant with Predominant Distal Weakness

Jong-Seok Bae

Department of Neurology, Kangnam General Hospital Public Corporation, Seoul; Department of Neurology, Sungkyunkwan University School of Medicine*, Seoul, Korea

Abstract

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a heterogenous group of acquired peripheral neuropathies. A subset of CIDP involves predominantly distal parts of the limbs, which is similar to axonal polyneuropathy. The clinical course or response to treatment may be different in this group. We investigated the clinical course and electrodiagnostic findings of the distal CIDP.
Methods: Twenty five CIDP cases were reviewed retrospectively. Patients with proximal as well as distal involvement were grouped as typical CIDP, and patients with predominantly distal involvement as distal CIDP. We compared the clinical, laboratory and electrophysiological findings of these two groups.
Results: Sixteen patients had typical CIDP and nine had distal CIDP. Distal CIDP differed significantly from typical CIDP; later age of onset (p=0.049), less frequent relapses (p=0.041), more rapidly progressive to maximal disability (p =0.01), low disability score at the diagnosis (p=0.02) and after treatment (p=0.01), poor response to immunomodulating therapy (p=0.02), and infrequent conduction blocks or abnormal temporal dispersions (p<0.01).
Conclusions: Distal CIDP is a distinctive variant of CIDP different from typical CIDP in clinical and electrophysiological features. Identification of the pathogenesis underlying this new entity may lead to better understanding of the heterogeneous acquired demyelinating neuropathies.Key Words: Polyradiculoneuropathy, Chronic inflammatory demyelinating, Distal, Variant