title: Central nervous system complications in two cases of juvenile onset dermatomyositis.
abstract: Central nervous system (CNS) complications are rarely reported in either juvenile or adult onset inflammatory myositides, such as dermatomyositis and polymyositis. We report two children, aged 4 and 10 yr respectively, with a diagnosis of juvenile dermatomyositis, both of whom subsequently developed clinical features of severe CNS involvement, possibly consistent with cerebral vasculopathy. One child died from apparent brainstem involvement; the other developed seizures, pseudoseizures and clinical depression which responded to aggressive immunosuppression. Although the vasculopathy or vasculitis underlying this disorder is known to have a systemic distribution, CNS involvement has rarely been reported and may be under-recognized.
authors.affiliation: Juvenile Dermatomyositis Research Centre, Rheumatology Unit, Great Ormond Street Hospital and the Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK.
authors.forename: A V
authors.initials: AV
authors.lastname: Ramanan
authors.forename: S
authors.initials: S
authors.lastname: Sawhney
authors.forename: K J
authors.initials: KJ
authors.lastname: Murray
mesh_terms: D002561:Cerebrovascular Disorders / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D003882:Dermatomyositis / Q000150:complications*; D004827:Epilepsy / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans