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Childhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers: a dysimmune neuropathy?
Neuromuscul Disord. 2009 Jan; 19(1):49-52.ND

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+Show Affiliations

Department of Neurology, University of Pecs, 2 Ret St, 7623 Pecs, Hungary.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19056270

Citation

Pfund, Zoltan, et al. "Childhood Steroid-responsive Acute Erythromelalgia With Axonal Neuropathy of Large Myelinated Fibers: a Dysimmune Neuropathy?" Neuromuscular Disorders : NMD, vol. 19, no. 1, 2009, pp. 49-52.
Pfund Z, Stankovics J, Decsi T, et al. Childhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers: a dysimmune neuropathy? Neuromuscul Disord. 2009;19(1):49-52.
Pfund, Z., Stankovics, J., Decsi, T., & Illes, Z. (2009). Childhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers: a dysimmune neuropathy? Neuromuscular Disorders : NMD, 19(1), 49-52. https://doi.org/10.1016/j.nmd.2008.10.005
Pfund Z, et al. Childhood Steroid-responsive Acute Erythromelalgia With Axonal Neuropathy of Large Myelinated Fibers: a Dysimmune Neuropathy. Neuromuscul Disord. 2009;19(1):49-52. PubMed PMID: 19056270.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Childhood steroid-responsive acute erythromelalgia with axonal neuropathy of large myelinated fibers: a dysimmune neuropathy? AU - Pfund,Zoltan, AU - Stankovics,Jozsef, AU - Decsi,Tamas, AU - Illes,Zsolt, Y1 - 2008/12/03/ PY - 2008/08/13/received PY - 2008/09/29/revised PY - 2008/10/08/accepted PY - 2008/12/6/pubmed PY - 2009/5/21/medline PY - 2008/12/6/entrez SP - 49 EP - 52 JF - Neuromuscular disorders : NMD JO - Neuromuscul Disord VL - 19 IS - 1 N2 - 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. SN - 0960-8966 UR - https://www.unboundmedicine.com/medline/citation/19056270/Childhood_steroid_responsive_acute_erythromelalgia_with_axonal_neuropathy_of_large_myelinated_fibers:_a_dysimmune_neuropathy L2 - https://linkinghub.elsevier.com/retrieve/pii/S0960-8966(08)00671-8 DB - PRIME DP - Unbound Medicine ER -