Aggressive course in encephalitis with opsoclonus, ataxia, chorea, and seizures the first pediatric case of y-aminobutyric acid type B receptor autoimmunity

Michael C. Kruer, Romana Hoeftberger, Kit Yeng Lim, Jason C. Coryell, Melissa D. Svoboda, Randall (Randy) Woltjer, Josep Dalmau

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

IMPORTANCE Autoantibodies to the ?-aminobutyric acid type B (GABAB) receptor have recently been identified as a cause of autoimmune encephalitis. Most patients with GABAB encephalitis have presented with limbic encephalitis. About half of the cases reported have been paraneoplastic in origin, with the majority of tumors representing small cell lung cancer. OBSERVATIONS We describe a 3-year-old boy who presented with a mixed movement disorder (opsoclonus, ataxia, and chorea) as well as seizures refractory to treatment. His seizures required continuous pentobarbital sodium infusion to be controlled. Despite treatment with intravenous corticosteroids and immunoglobulins, the patient ultimately died of overwhelming sepsis. CONCLUSIONS AND RELEVANCE To our knowledge, this report represents the first pediatric case of GABAB-associated encephalitis. Our patient presented with encephalopathy, refractory seizures, and a mixed movement disorder rather than limbic encephalitis. ?-Aminobutyric acid type B receptor autoimmunity deserves consideration in pediatric patients presenting with encephalitis.

Original languageEnglish (US)
Pages (from-to)620-623
Number of pages4
JournalJAMA Neurology
Volume71
Issue number5
DOIs
StatePublished - 2014

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Aminobutyrates
Ocular Motility Disorders
Chorea
Encephalitis
Ataxia
Autoimmunity
Seizures
Limbic Encephalitis
Pediatrics
Movement Disorders
Intravenous Immunoglobulins
Small Cell Lung Carcinoma
Brain Diseases
Pentobarbital
Autoantibodies
Sepsis
Adrenal Cortex Hormones
Therapeutics
Neoplasms

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Aggressive course in encephalitis with opsoclonus, ataxia, chorea, and seizures the first pediatric case of y-aminobutyric acid type B receptor autoimmunity. / Kruer, Michael C.; Hoeftberger, Romana; Lim, Kit Yeng; Coryell, Jason C.; Svoboda, Melissa D.; Woltjer, Randall (Randy); Dalmau, Josep.

In: JAMA Neurology, Vol. 71, No. 5, 2014, p. 620-623.

Research output: Contribution to journalArticle

Kruer, Michael C. ; Hoeftberger, Romana ; Lim, Kit Yeng ; Coryell, Jason C. ; Svoboda, Melissa D. ; Woltjer, Randall (Randy) ; Dalmau, Josep. / Aggressive course in encephalitis with opsoclonus, ataxia, chorea, and seizures the first pediatric case of y-aminobutyric acid type B receptor autoimmunity. In: JAMA Neurology. 2014 ; Vol. 71, No. 5. pp. 620-623.
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