Case Report

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, and WIETSE KUIS

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. (J Rheumatology 2003;30:2059-63)

Key Indexing Terms:

JUVENILE DERMATOMYOSITIS
CENTRAL NERVOUS SYSTEM
VASCULITIS

---

From the Departments of Pediatric Immunology, Neurology, and Physiotherapy, University Medical Center of Utrecht, Wilhelmina Children's Hospital, Utrecht, The Netherlands.

E.F. Elst, MD, Fellow; S.S.M. Kamphuis, MD; B.J. Prakken, MD, PhD, Associate Professor of Pediatrics; N.M. Wulffraat, MD, PhD, Associate Professor of Pediatrics; J.J. van der Net, PhD, Physiotherapist; A.C.B. Peters, MD, PhD, Professor of Child Neurology; W. Kuis, MD, PhD, Professor of Pediatrics.

Address reprint requests to Dr. E.F. Elst, University Medical Center Utrecht, Wilhelmina Children's Hospital mailbox KC.03.063.0, PO Box 85090, 3508 AB Utrecht, The Netherlands. E-mail: e.elst@wkz.azu.nl

Submitted June 24, 2002; revision accepted December 9, 2002.

---