RT Journal Article
SR Electronic
T1 Case report: severe central nervous system involvement in juvenile dermatomyositis.
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 2059
OP 2063
VO 30
IS 9
A1 Elst, Elisabeth F
A1 Kamphuis, Sylvia S M
A1 Prakken, Berent J
A1 Wulffraat, Nicolas M
A1 van der Net, Janjaap
A1 Peters, A C Boudewyn
A1 Kuis, Wietse
YR 2003
UL http://www.jrheum.org/content/30/9/2059.abstract
AB 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.