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

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Abstract

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.

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Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Neurosurgery, Medical University, Auenbruggerplatz 29, 8036, Graz, Austria

    Karin Pistracher, Verena Gellner, Stefan Riegler, Bernadette Schökler & Senta Kurschel

  2. Institute of Pathology, Medical University, Graz, Austria

    Michael Scarpatetti

Authors
  1. Karin Pistracher
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  2. Verena Gellner
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  3. Stefan Riegler
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  4. Bernadette Schökler
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  5. Michael Scarpatetti
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  6. Senta Kurschel
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Correspondence to Senta Kurschel.

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Pistracher, K., Gellner, V., Riegler, S. et al. Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis—a review. Childs Nerv Syst 28, 1141–1148 (2012). https://doi.org/10.1007/s00381-012-1827-4

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  • DOI: https://doi.org/10.1007/s00381-012-1827-4

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