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Neuromuscul. Disord. · Jan 2009
Case ReportsChildhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers: a dysimmune neuropathy?
- Zoltan Pfund, Jozsef Stankovics, Tamas Decsi, and Zsolt Illes.
- Department of Neurology, University of Pecs, 2 Ret St, 7623 Pecs, Hungary.
- Neuromuscul. Disord. 2009 Jan 1; 19 (1): 49-52.
AbstractA 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.
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