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Chronic inflammatory demyelinating polyneuropathy of infancy: A corticosteroid‐responsive disorder
Author(s) -
Sladky John T.,
Brown Mark J.,
Berman Peter H.
Publication year - 1986
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410200113
Subject(s) - medicine , chronic inflammatory demyelinating polyneuropathy , polyneuropathy , corticosteroid , sural nerve , pathology , demyelinating disorder , biopsy , nerve biopsy , edema , peripheral neuropathy , immunology , endocrinology , disease , antibody , diabetes mellitus
We present the clinical, electrophysiological, and histopathological findings in 6 children with early‐onset chronic inflammatory demyelinating neuropathy. The clinical features initially suggested a genetically determined disorder in each patient. Sural nerve biopsy showed changes of chronic demyelination with multifocal endoneurial edema and mononuclear cellular infiltrates. All children improved with corticosteroid therapy.