title: Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system.
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.
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Marco Felipe
authors.initials: MF
authors.lastname: Silva
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Ana Raquel
authors.initials: AR
authors.lastname: Feitosa
authors.affiliation: Pediatric Neurology Unit, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: José A
authors.initials: JA
authors.lastname: Paz
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil; Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Nádia Emi
authors.initials: NE
authors.lastname: Aikawa
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil; Division of Rheumatology, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil. Electronic address: clovisaasilva@gmail.com.
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D002097:C-Reactive Protein; D002490:Central Nervous System; D002543:Cerebral Hemorrhage / Q000175:diagnosis* / Q000628:therapy; D003399:Craniotomy; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis*
chemical_list: D003520:Cyclophosphamide; D002097:C-Reactive Protein
keywords: Acidente vascular encefálico; Angiíte primária na infância; Central nervous system; Childhood primary angiitis; Hemorragia intracerebral; Intracerebral hemorrhage; Sistema nervoso central; Stroke; Vasculite; Vasculitis