Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis--a review

Childs Nerv Syst. 2012 Aug;28(8):1141-8. doi: 10.1007/s00381-012-1827-4. Epub 2012 Jun 9.


Purpose: 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.

Method: 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.

Conclusion: 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.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Cerebral Hemorrhage / epidemiology*
  • Cerebral Hemorrhage / etiology*
  • Cerebral Hemorrhage / therapy
  • Child
  • Humans
  • Risk Factors
  • Treatment Outcome
  • Vasculitis, Central Nervous System / complications*

Supplementary concepts

  • Primary angiitis of the central nervous system