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

Zoltan Pfund, Jozsef Stankovics, T. Decsi, Zsolt Illes

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)49-52
Number of pages4
JournalNeuromuscular Disorders
Volume19
Issue number1
DOIs
Publication statusPublished - Jan 2009

Fingerprint

Erythromelalgia
Steroids
Myelinated Nerve Fibers
Hyperalgesia
Muscle Weakness
Methylprednisolone
Blood Vessels
Extremities
Pain

Keywords

  • Allodynia
  • Dysimmune
  • Erythromelalgia
  • Neuropathic pain
  • Neuropathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Genetics(clinical)
  • Neurology

Cite this

Childhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers : A dysimmune neuropathy? / Pfund, Zoltan; Stankovics, Jozsef; Decsi, T.; Illes, Zsolt.

In: Neuromuscular Disorders, Vol. 19, No. 1, 01.2009, p. 49-52.

Research output: Contribution to journalArticle

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