TY - JOUR
T1 - Severe central nervous system involvement in juvenile dermatomyositis
AU - Elst, Elisabeth F.
AU - Kamphuis, Sylvia S.M.
AU - Prakken, Berent J.
AU - Wulffraat, Nicolas M.
AU - Van der Net, Janjaap
AU - Peters, A. C.Boudewyn
AU - Kuis, Wietse
PY - 2003/9/1
Y1 - 2003/9/1
N2 - 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.
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.
KW - Central nervous system
KW - Juvenile dermatomyositis
KW - Vasculitis
UR - http://www.scopus.com/inward/record.url?scp=0043195651&partnerID=8YFLogxK
M3 - Article
C2 - 12966616
AN - SCOPUS:0043195651
SN - 0315-162X
VL - 30
SP - 2059
EP - 2063
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 9
ER -