Meltem EROL, Dilara FUCUCUOĞLU, Özgül YİĞİT, Emine Erdoğan KUTANİS, Özlem Bostan GAYRET, Ramazan ALBAYRAK

Henoch schönlein purpurası ilişkili posterior reversible ensefalopati sendromu

Giriş: Henoch-Schönlein purpurası (HSP) primer olarak çocukları etkileyen akut bir vaskülittir. Merkezi sinir sistemi (MSS) tutulumu ve karakteristik döküntülerden önce nörolojik komplikasyonların ortaya çıkması nadirdir. Posterior reversibl ensefalopati sendromu (PRES) ve serebral vaskülit HSP nın nörolojik komplikasyonlarıdır.Olgu sunumu: konvülziyon, bilinç kaybı ve total görme kaybı ile başvuran 5 yaşındaki bir kız olgusu sunulmuştur. Nörolojik semptomlar ön planda olup, karekteristik döküntü yoktu. Radyolojik bulguları PRES ile uyumluydu. Klinik bulgular HSP için atipikti. Böbrek tutulumu ve hipertansiyon yoktu. Klinik bulgular kortikosteroid tedavisi ile düzeldi.Sonuç: Hastamız tipik HSP döküntülerinden önce santral sinir sistemi bulgularının ortaya çıkması ve hipertansiyonun olmaması nedeniyle ilginçtir. HSP nin santral sinir sistemi komplikasyonu ile başvurmuştur.

Anahtar Kelimeler:

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Posterior reversible encephalopathy syndrome associated with Henoch–Schönlein purpura

Introduction: Henoch–Schönlein purpura (HSP) is an acute vasculitis that primarily affects children. Central nervous system (CNS) involvement and the appearance of neurological complications preceding the characteristic rash are rare. Posterior reversible encephalopathy syndrome (PRES) and cerebral vasculitis are neurological complications of HSP.Case presentation: We describe the case of a 5-year-old girl who presented with seizures, loss of consciousness, and total loss of vision. Her HSP was atypical because the presenting symptoms were neurological complications rather than the characteristic rash, and her radiological features were compatible with PRES. She did not have renal impairment or hypertension. Her abnormal findings resolved following a standard course of corticosteroids.Conclusion: Our case is atypical because CNS involvement occurred before the characteristic rash in the absence of hypertension. The patient’s HSP presented as a complication of cerebral vasculitis.

Keywords:

Henoch–Schönlein purpura, cerebral vasculitis posterior reversible encephalography syndrome,

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