Severe central nervous system involvement in juvenile dermatomyositis

Elisabeth F. Elst*, Sylvia S.M. Kamphuis, Berent J. Prakken, Nicolas M. Wulffraat, Janjaap Van der Net, A. C.Boudewyn Peters, Wietse Kuis

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

17 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)2059-2063
Number of pages5
JournalJournal of Rheumatology
Issue number9
Publication statusPublished - 1 Sept 2003


  • Central nervous system
  • Juvenile dermatomyositis
  • Vasculitis


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