Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10mm deviation to the left from the midline. C-reactive protein (9.2mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.

(15,2%); e da depressão foi 12/90 (13,3%), sendo mais prevalente em pacientes com DC (14%) do que na RCUI (12,1%). Em relação à qualidade do sono, a prevalência geral de pacientes com dificuldade pra dormir foi 48 (53,3%), sendo maior na DC (56,1%) do que na RCUI (48,5%); e com sonolência diurna foi 13 (14,4%), sendo maior na RCUI (21,1%) do que na DC (10,5%). Quando comparada a qualidade de vida com a ansiedade e depressão entre os dois grupos, houve diferença significativa (p=0,000) entre os pacientes com DC em relação aos pacientes com RCUI. A pior qualidade do sono foi significativamente maior entre pacientes com DC (p=0,000). Não houve significância em relação a sonolência diurna entre os grupos. CONCLUSÃO: A pior QV de pacientes portadores de DII estava associada com ansiedade, depressão e/ou pior qualidade do sono, principalmente entre os pacientes com DC, mesmo em remissão clínica. ABCDExpress 2017;1(2):1099Codigo: 61336 Acesso está disponível em www.revistaabcd.com.br e www.sbad2017.com.br Acesso pelo

No abstract

Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory brain disease of unknown etiology. Of note, brain hemorrhage has been rarely reported in cPACNS patients, generally associated with a delayed clinical diagnosis, or with a diagnosis only at necropsy. We present the case of a boy with cPACNS that previously suffered an ischemic stroke. At the age of 7 years and 10 months, he presented a sudden and severe headache, vomiting and reduction in consciousness level (Glasgow coma scale 7), requiring prompt tracheal intubation. Brain computed tomography demonstrated intraparenchymal hematoma in the right parieto-occipital lobe and a small focus of bleeding in the right frontal lobe, vasogenic edema, herniation of the uncus and a 10mm deviation to the left from the midline. C-reactive protein (9.2mg/dL) and von Willebrand factor (vWF) antigen (202%) were elevated. Decompressive craniotomy was performed and methylprednisolone and cyclophosphamide were administered. One week later, the patient had left hemiparesis without other sequelae. Importantly, motor deficits have been improving progressively. Our case reinforces the inclusion of this vasculitis as a differential diagnosis in children and adolescents with CNS hemorrhage.