The neuropsychological profile of children with basal ganglia encephalitis: a case series

Inflammatory basal ganglia encephalitis (BGE) is a rare but distinct entity of putative autoimmune aetiology, with specific basal ganglia inflammation and acute movement disorders. Unlike most brain injuries, BGE is a radiologically pure basal ganglia syndrome. The current study systematically descr...

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Bibliographic Details
Published in:Developmental medicine and child neurology 2017-04, Vol.59 (4), p.445-448
Main Authors: Pawela, Chiara, Brunsdon, Ruth K, Williams, Tracey A, Porter, Melanie, Dale, Russell C, Mohammad, Shekeeb S
Format: Article
Language:English
Subjects:
Basal Ganglia Diseases - complications
Child
Child, Preschool
Cognition Disorders - diagnosis
Cognition Disorders - etiology
Cohort Studies
Encephalitis - complications
Humans
Infant
Male
Neuropsychological Tests
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Summary:Inflammatory basal ganglia encephalitis (BGE) is a rare but distinct entity of putative autoimmune aetiology, with specific basal ganglia inflammation and acute movement disorders. Unlike most brain injuries, BGE is a radiologically pure basal ganglia syndrome. The current study systematically describes the neuropsychological outcomes of four paediatric cases of BGE, and thus the neuropsychological outcomes of focal basal ganglia insult in childhood. Although all patients made significant motor recoveries, all four cases displayed executive dysfunction, fine motor difficulties, and anxiety. Three out of four cases displayed attention deficits. The case who received intravenous immunoglobulin (IVIg) treatment and steroids during the acute phase of the disease had the best cognitive outcome. These findings highlight the need for detailed neuropsychological assessment and long‐term follow‐up. What this paper adds Paediatric basal ganglia encephalitis presents with a distinctive neuropsychological phenotype, consistent with focal basal ganglia dysfunction. The pattern of results suggests better outcomes for those patients treated earlier with IVIg. This article is commented on by Peall on pages 353–354 of this issue.
ISSN:0012-1622
1469-8749
DOI:10.1111/dmcn.13351