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

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.

• Authors: Zoltan Pfund, Jozsef Stankovics, Tamas Decsi, Zsolt Illes
• Journal: Neuromuscular disorders : NMD (Neuromuscul Disord) Vol. 19 Issue 1 Pg. 49-52 (Jan 2009) ISSN: 0960-8966 [Print] England
• PMID: 19056270 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Steroids

Topics

• Acute Disease
• Age of Onset
• Autoimmune Diseases of the Nervous System (pathology, physiopathology)
• Axons (pathology)
• Child
• Erythromelalgia (drug therapy, immunology, pathology)
• Extremities (innervation, physiopathology)
• Female
• Humans
• Motor Neurons (immunology, pathology)
• Muscle Weakness (immunology, pathology, physiopathology)
• Nerve Fibers, Myelinated (pathology)
• Neuralgia (immunology, pathology, physiopathology)
• Peripheral Nerves (pathology, physiopathology)
• Peripheral Nervous System Diseases (immunology, pathology, physiopathology)
• Skin (innervation, pathology, physiopathology)
• Steroids (pharmacology, therapeutic use)
• Treatment Outcome
• Wallerian Degeneration (immunology, pathology, physiopathology)