title: Case report: severe central nervous system involvement in juvenile dermatomyositis.
abstract: We present 3 patients with juvenile dermatomyositis (JDM) and severe central nervous system (CNS) complications. All patients had at least 4 positive criteria of Bohan and Peter, which confirmed a definite diagnosis of JDM. They were all male, and had a relatively high creatinine kinase value at admission (1532-4260 U/l). Besides, progressive proximal muscle weakness and rash, one patient presented with rapid irreversible decline of vision. Ophthalmologic examination showed active vasculitis of the retina. After 2 weeks of treatment with immunosuppressive drugs and being in improved, relatively stable clinical condition, all 3 patients developed generalized tonic-clonic convulsions. Other causes of the neurological symptoms could be excluded. In all 3 patients, the course of JDM was fatal. The clinical symptoms and further investigations in our patients show CNS involvement in JDM. Although rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JDM.
authors.affiliation: Department of Pediatric Immunology, University Medical Center of Utrecht, Wilhelmina Children's Hospital, mailbox KC.03.063.0, 3508 AB Utrecht, The Netherlands. e.elst@wkz.azu.nl
authors.forename: Elisabeth F
authors.initials: EF
authors.lastname: Elst
authors.forename: Sylvia S M
authors.initials: SS
authors.lastname: Kamphuis
authors.forename: Berent J
authors.initials: BJ
authors.lastname: Prakken
authors.forename: Nicolas M
authors.initials: NM
authors.lastname: Wulffraat
authors.forename: Janjaap
authors.initials: J
authors.lastname: van der Net
authors.forename: A C Boudewyn
authors.initials: AC
authors.lastname: Peters
authors.forename: Wietse
authors.initials: W
authors.lastname: Kuis
mesh_terms: D000794:Angiokeratoma / Q000150:complications / Q000175:diagnosis*; D002493:Central Nervous System Diseases / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D018570:Risk Assessment; D012640:Seizures / Q000150:complications / Q000175:diagnosis*; D012720:Severity of Illness Index; D014657:Vasculitis / Q000150:complications / Q000175:diagnosis*