Abstract Ref Number = APCP1121
SEIZURE IN JUVENILE DERMATOMYOSITIS
Praevilia Salendu,Citra Raditha,Hardiono D Pusponegoro,Dina Muktiarti
Neurology Pediatric Division Department of Pediatric Faculty of Medicine Universitas Indonesia Cipto Mangunkusumo General Hospital Jakarta
Background : Juvenile dermatomyositis (JDM) is a rare disorder, with an incidence of 1.9/1000000 children aged < 16 years. Though JDM may be thought of as a homogeneous condition, it is in fact rather heterogeneous, not only in marker of disease activity, as has been alluded to previously, but also in the clinical profile. This is evidenced by the highly variable presentation, course and degree of systematic or internal organ involvement. Seizure in JDM can be caused by some etiology. Neurological dysfunction is reported infrequently, usually as isolated case reports.
Case Presentation Summary : A 7-yr-old girl was diagnosed with juvenile dermatomyositis from December 2017. She presented generalized weakness, difficulty walking, painful joints, a typical dermatomyositis rash over the extensor surfaces, including a heliotrope rash over the eyelids, and proximal muscle weakness. Her muscle enzymes were elevated. Electromyography showed myopathy. She was treated with oral metilprednisolone and ciclosporin oral for its disease-modifying and steroid-sparing effect. Readmission to hospital because of repetitive jerking movements of the limbs and rolling of the eyes, lasting for 3 minutes, without accompanying fever. She developed hypertension. Neurological examination including fundal examination was normal. All biochemical investigations were normal. Seizure was treated with phenytoin. Magnetic resonance imaging presented demyelinitation and vasculitis with left hemisphere brain atrophy.
Learning Points/Discussion : Investigation of central nervous system involvement (CNS) should be done in JMD patient with seizure. Though rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JMD caused by autoimmune inflammation. Aggressive immunosupresion and multi-organ support are critical to the outcome of this rare but potentially fatal neurologic and systemic complication of JMD.
Keywords: JUVENILE DERMATOMYOSITIS Seizure CNS Involvement