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Abstract Details

Pure Motor Chronic Inflammatory Demyelinating Polyneuropathy
Peripheral Nerve
P01 - (-)
151
BACKGROUND: The autoimmune disease CIDP is clinically heterogeneous. Subtypes of CIDP differ in clinical presentation, electrophysiologic and laboratory features, and response to treatment. Patients with pure motor CIDP have selective motor involvement on clinical and electrophysiologic examination, but the clinical, pathologic, electrophysiologic, and prognostic features of pure motor CIDP are poorly understood.
DESIGN/METHODS: In 6 patients with pure motor CIDP, we reviewed the clinical features, electrophysiologic and laboratory findings, treatment, and prognosis.
RESULTS: The characteristic features of the 6 patients with pure motor CIDP included: (1) symmetric weakness and generalized areflexia within several months of disease onset; (2) predominantly upper limb weakness, which was the initial symptom; (3) absence of sensory deficits except for mild distal paresthesia; (4) absence of muscle atrophy or cranial nerve involvement; (5) marked improvement after treatment with intravenous immunoglobulin infusion (IVIG); (6) increased muscle strength after treatment with IVIG, beginning 1 day after infusion, reaching a maximum within a few weeks, and lasting only a few months; and (7) a relapsing-remitting clinical course, with normal daily life because of repeated IVIG.
CONCLUSIONS: The characteristic clinical features, response to treatment, and prognosis suggest that pure motor CIDP is a distinct subtype of CIDP with an immunologic basis. Repeated IVIG were necessary to maintain the motor function of patients with pure motor CIDP.
Authors/Disclosures
Anthony Polazzo
PRESENTER
No disclosure on file
No disclosure on file
No disclosure on file
Yuichi Hayashi (Department of Neurology, Gifu University Grad) Yuichi Hayashi has nothing to disclose.
Takashi Inuzuka, MD, FAAN (Gifu Municipal Hospital) No disclosure on file
No disclosure on file