title: The neuropsychological profile of children with basal ganglia encephalitis: a case series.
abstract: 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.
authors.affiliation: Psychology Department, Macquarie University, Sydney, Australia.
authors.forename: Chiara
authors.initials: C
authors.lastname: Pawela
authors.affiliation: Kids Rehab, The Children's Hospital at Westmead, Sydney, Australia.
authors.forename: Ruth K
authors.initials: RK
authors.lastname: Brunsdon
authors.affiliation: Kids Rehab, The Children's Hospital at Westmead, Sydney, Australia.
authors.forename: Tracey A
authors.initials: TA
authors.lastname: Williams
authors.affiliation: Psychology Department, Macquarie University, Sydney, Australia.
authors.forename: Melanie
authors.initials: M
authors.lastname: Porter
authors.affiliation: Discipline of Paediatrics and Child Health, University of Sydney, Sydney, Australia.
authors.forename: Russell C
authors.initials: RC
authors.lastname: Dale
authors.affiliation: Discipline of Paediatrics and Child Health, University of Sydney, Sydney, Australia.
authors.forename: Shekeeb S
authors.initials: SS
authors.lastname: Mohammad
mesh_terms: D001480:Basal Ganglia Diseases / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D015331:Cohort Studies; D004660:Encephalitis / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D009483:Neuropsychological Tests