Small-fiber neuropathy associated with autoinflammatory syndromes in children and adolescents.
Behcet disease; erythromelalgia
autoinflammatory syndrome
familial Mediterranean fever
small-fiber neuropathy
Journal
Muscle & nerve
ISSN: 1097-4598
Titre abrégé: Muscle Nerve
Pays: United States
ID NLM: 7803146
Informations de publication
Date de publication:
06 2020
06 2020
Historique:
received:
27
03
2019
revised:
28
02
2020
accepted:
01
03
2020
pubmed:
7
3
2020
medline:
30
7
2020
entrez:
6
3
2020
Statut:
ppublish
Résumé
Small-fiber neuropathy is rare in children. It has been associated with several autoimmune disorders, but there are no reports of an autoinflammatory etiology. The data of four children/adolescents presenting with erythromelalgia and neuropathic pain from 2014 to 2019 were collected retrospectively from the electronic database of a pediatric medical center. Results of clinical and/or electrophysiological evaluation excluded large nerve fiber involvement. Skin biopsy results confirmed small-fiber neuropathy. According to genetic analysis, two patients were heterozygous and one was homozygous for mutations in the familial Mediterranean fever (MEFV) gene. Behcet disease was diagnosed in the fourth patient. Treatment with anti-interleukin-1 agents, intravenous immunoglobulin, and glucocorticoids was beneficial. The diagnosis of small-fiber neuropathy should be considered in children/adolescents presenting with erythromelalgia. A thorough investigation is required to reveal the underlying disorder. Clinicians should be alert to the peripheral neurological manifestations of autoinflammatory syndromes because effective treatments are available.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
791-796Informations de copyright
© 2020 Wiley Periodicals, Inc.
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