Childhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers: a dysimmune neuropathy?

Autor(es): Pfund Zoltan,Stankovics Jozsef,Decsi Tamas,Illes Zsolt

Resumo: A 12-year-old girl developed acute erythromelalgia of distal extremities. Physical, imaging and laboratory examinations failed to find an infective, systemic autoimmune, metabolic, endocrine, and vascular origin. The severe pain and allodynia indicated small-fiber neuropathy but muscle weakness suggested an involvement of large myelinated nerve fibers. This was confirmed by electrophysiological testing. High-dose then slowly tapered methylprednisolone resulted in rapid remission of painful erythromelalgia and complete electrophysiological recovery. Our case may suggest an additional variant to recently described steroid-responsive erythromelalgia with small-fiber axonopathy and may denote a transitory variant to Guillain-Barré syndrome or chronic dysimmune neuropathies.

Palavras-Chave: Erythromelalgia; Dysimmune; Neuropathy; Neuropathic pain; Allodynia

Imprenta: Neuromuscular Disorders : NMD, v. 19, n. 1, p. 49-52, 2009

Identificador do objeto digital: 10.1016/j.nmd.2008.10.005

Descritores: Guillain-Barre Syndrome - Cell ; Guillain-Barre Syndrome - Cytopathology ; Guillain-Barre Syndrome - Pathogenesis ; Guillain-Barre Syndrome - Immunology ; Guillain-Barre Syndrome - Public health

Data de publicação: 2009