Neuronal voltage-gated potassium channel complex autoimmunity in children

Radhika Dhamija, Deborah L. Renaud, Sean J Pittock, Andrew B McKeon, Daniel H Lachance, Katherine C Nickels, Elaine C Wirrell, Nancy L. Kuntz, Mary D. King, Vanda A Lennon

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

Autoimmunity targeting voltage-gated potassium channel complexes have not been systematically documented in children. Identified in the Neuroimmunology Laboratory records of Mayo Clinic were 12 seropositive children, 7 among 252 Mayo Clinic pediatric patients tested on a service basis for serologic evidence of neurologic autoimmunity (June 2008-April 2010), 4 during the assay's preimplementation validation (before June 2008) and 1 non-Mayo patient with available clinical information. Neurologic manifestations were subacute and multifocal. Three had global developmental regression, 6 movement disorders, 4 dysarthria, 3 seizures, 1 Satoyoshi syndrome, 1 painful red feet, 2 insomnia, 2 gastrointestinal dysmotility, and 2 small fiber neuropathy. Neoplasia was found in 1 child. Treating physicians recorded improvement in all 7 children who received immunotherapy. Neurologic symptom relapse occurred in 3 of 6 children after ceasing immunotherapy. These findings highlight a diverse clinical spectrum of neuronal potassium channel complex autoimmunity in children, and they illustrate benefit from early initiated immunotherapy, with a tendency to relapse when therapy ceases. Diagnosis is generally delayed in the process of eliminating neurodegenerative causes. Currently 2.7% of pediatric sera evaluated for neurologic autoimmunity are positive for neuronal potassium channel complex-reactive immunoglobulin G. The frequency and full spectrum of neurologic accompaniments remains to be determined.

Original languageEnglish (US)
Pages (from-to)275-281
Number of pages7
JournalPediatric Neurology
Volume44
Issue number4
DOIs
StatePublished - Apr 2011

Fingerprint

Voltage-Gated Potassium Channels
Autoimmunity
Immunotherapy
Nervous System
Potassium Channels
Neurologic Manifestations
Pediatrics
Recurrence
Dysarthria
Movement Disorders
Sleep Initiation and Maintenance Disorders
Foot
Seizures
Immunoglobulin G
Physicians
Serum
Neoplasms

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Neuronal voltage-gated potassium channel complex autoimmunity in children. / Dhamija, Radhika; Renaud, Deborah L.; Pittock, Sean J; McKeon, Andrew B; Lachance, Daniel H; Nickels, Katherine C; Wirrell, Elaine C; Kuntz, Nancy L.; King, Mary D.; Lennon, Vanda A.

In: Pediatric Neurology, Vol. 44, No. 4, 04.2011, p. 275-281.

Research output: Contribution to journalArticle

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