J Korean Neurol Assoc.  2004 Jun;22(3):219-225.

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

• Bae JS
• Kim BJ

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.

Keyword

Polyradiculoneuropathy; Chronic inflammatory demyelinating; Distal; Variant

MeSH Terms

Age of Onset
Axons
Diagnosis
Extremities
Humans
Peripheral Nervous System Diseases
Polyneuropathies
Polyradiculoneuropathy
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating*
Recurrence
Retrospective Studies