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Chronic inflammatory demyelinating polyradiculoneuropathy with autonomic involvement
Author(s) -
Yamamoto Kanji,
Watarai Megumi,
Hashimoto Takao,
Ikeda ShuIchi
Publication year - 2005
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20140
Subject(s) - polyradiculoneuropathy , medicine , axon , autonomic ganglion , autonomic nervous system , myelin , pathology , ganglion , anesthesia , guillain barre syndrome , immunology , central nervous system , anatomy , blood pressure , heart rate
Abstract We report a case of chronic acquired neuropathy predominantly affecting sensory and autonomic nerves. Investigations showed a demyelinating polyradiculoneuropathy with axonal degeneration and depletion of postganglionic noradrenergic fibers in the rectal mucosa. Intravenous immunoglobulin and corticosteroid administration were effective in alleviating symptoms and improving electrophysiological abnormalities. This neuropathy may be a novel variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), in which autoimmunoreactivity is directed not only against myelin but also against axon‐ or ganglion‐composing protein. Autonomic nerve involvement does not exclude a diagnosis of CIDP. Muscle Nerve 2004