Distal Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Variant with Predominant Distal Weakness.
- Author:
Jong Seok BAE
1
;
Byoung Joon KIM
Author Information
1. Department of Neurology, Kangnam General Hospital Public Corporation, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Polyradiculoneuropathy;
Chronic inflammatory demyelinating;
Distal;
Variant
- MeSH:
Age of Onset;
Axons;
Diagnosis;
Extremities;
Humans;
Peripheral Nervous System Diseases;
Polyneuropathies;
Polyradiculoneuropathy;
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating*;
Recurrence;
Retrospective Studies
- From:Journal of the Korean Neurological Association
2004;22(3):219-225
- CountryRepublic of Korea
- Language:Korean
-
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.
