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PURE MOTOR CIDP. A DISTINCT ENTITY?
Author(s) -
Sabatelli M.,
Madia F.,
De Armas L.,
Tonali P.
Publication year - 2000
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2000.00513-52.x
Subject(s) - medicine , pathological , electrophysiology , compound muscle action potential , sural nerve , weakness , polyradiculoneuropathy , pathology , pediatrics , surgery , guillain barre syndrome
The spectrum of clinical, pathological, and electrophysiological features of CIDP is very heterogeneous. The onset may be acute or slowly progressive and clinical course monophasic or relapsing‐remitting. Motor‐sensory, ataxic sensory and pure motor variants have been described. The importance of the classification of CIDP is not an academic one, as each subtype might respond to specific treatments. We describe clinical features and long term follow up of 4 patients affected by pure motor CIDP. 4 out of our 30 CIDP patients showed no clinical, electrophysiological, or (in three patients) sural nerve biopsy features of sensory fiber involvement. All four patients disclosed similar clinical, electrophysiological features: the age of onset was before 30 years, clinical course was relapsing‐remitting, and electrophysiological examination showed features of pure demyelinating neuropathy with normal distal CMAP amplitude and clear evidence of conduction blocks. None of them showed significant response to corticosteroids, while high‐dose intravenous immunoglobulins were very effective in all patients. Three patients showed marked improvement with interferon alpha therapy. Two patients showed marked myastenic‐like fluctuation of weakness Our findings suggest that pure motor CIDP represents a distinct entity. Supported by Telethon Italy.