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...
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doaj-b1eb0396c4bf4bf49f363de045dfc0432020-11-25T01:57:47ZengSociedade Brasileira de Reumatologia Revista Brasileira de Reumatologia1809-457056436637010.1016/j.rbre.2014.05.007S0482-50042016000400366Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous systemMarco Felipe SilvaAna Raquel FeitosaJosé A. PazNádia Emi AikawaClovis A. SilvaABSTRACT 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 10 mm deviation to the left from the midline. C-reactive protein (9.2 mg/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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042016000400366&lng=en&tlng=enChildhood primary angiitisCentral nervous systemVasculitisIntracerebral hemorrhageStroke |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Marco Felipe Silva Ana Raquel Feitosa José A. Paz Nádia Emi Aikawa Clovis A. Silva |
spellingShingle |
Marco Felipe Silva Ana Raquel Feitosa José A. Paz Nádia Emi Aikawa Clovis A. Silva Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system Revista Brasileira de Reumatologia Childhood primary angiitis Central nervous system Vasculitis Intracerebral hemorrhage Stroke |
author_facet |
Marco Felipe Silva Ana Raquel Feitosa José A. Paz Nádia Emi Aikawa Clovis A. Silva |
author_sort |
Marco Felipe Silva |
title |
Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system |
title_short |
Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system |
title_full |
Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system |
title_fullStr |
Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system |
title_full_unstemmed |
Intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system |
title_sort |
intracerebral hemorrhage with a favorable outcome in a patient with childhood primary angiitis of the central nervous system |
publisher |
Sociedade Brasileira de Reumatologia |
series |
Revista Brasileira de Reumatologia |
issn |
1809-4570 |
description |
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 10 mm deviation to the left from the midline. C-reactive protein (9.2 mg/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. |
topic |
Childhood primary angiitis Central nervous system Vasculitis Intracerebral hemorrhage Stroke |
url |
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0482-50042016000400366&lng=en&tlng=en |
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