Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis--a review.
OBJECTIVE
The aim of the study was to evaluate frequency and clinical relevance of haemorrhagic events associated with primary angiitis of the central nervous system in childhood (cPACNS), a rare but increasingly recognized disease with varying clinical presentations.


METHODS
A systematic literature review from 1990 onwards was conducted to identify reported cases of cPACNS.


RESULTS
A total of 110 paediatric patients met the inclusion criteria. The median age was 9.5 years. Seven children (7/110, 6.4 %) demonstrated cerebral haemorrhage. Death occurred only in children with cerebral haemorrhage (4/110, 3.6 %); both a sudden and prolonged course of disease was observed.


CONCLUSIONS
PACNS is a rare disease and the occurrence of haemorrhage with this condition is even rarer; however, the risk of an unfavourable outcome under these circumstances seems to be increased. PACNS adds to the list of differential diagnoses of intracerebral haemorrhage in the paediatric population.
Department of Neurosurgery, Medical University, Auenbruggerplatz 29, 8036 Graz, Austria.
Karin
K
Pistracher
Verena
V
Gellner
Stefan
S
Riegler
Bernadette
B
Schökler
Michael
M
Scarpatetti
Senta
S
Kurschel
D002543:Cerebral Hemorrhage / Q000453:epidemiology* / Q000209:etiology* / Q000628:therapy; D002648:Child; D006801:Humans; D012307:Risk Factors; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000150:complications*