Case report: severe central nervous system involvement in juvenile dermatomyositis

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 adm...

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Bibliographic Details
Published in:Journal of rheumatology 2003-09, Vol.30 (9), p.2059-2063
Main Authors: Elst, Elisabeth F, Kamphuis, Sylvia S M, Prakken, Berent J, Wulffraat, Nicolas M, van der Net, Janjaap, Peters, A C Boudewyn, Kuis, Wietse
Format: Article
Language:English
Subjects:
Angiokeratoma - complications
Angiokeratoma - diagnosis
Central Nervous System Diseases - complications
Central Nervous System Diseases - diagnosis
Central Nervous System Diseases - therapy
Child
Child, Preschool
Dermatomyositis - complications
Dermatomyositis - diagnosis
Dermatomyositis - therapy
Disease Progression
Fatal Outcome
Humans
Magnetic Resonance Angiography - methods
Magnetic Resonance Imaging - methods
Male
Risk Assessment
Seizures - complications
Seizures - diagnosis
Severity of Illness Index
Vasculitis - complications
Vasculitis - diagnosis
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Summary: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.
ISSN:0315-162X
1499-2752