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Neuropediatrics 2005; 36(1): 40-44
DOI: 10.1055/s-2004-830532
Short Communication

Georg Thieme Verlag KG Stuttgart · New York

Cerebral Thrombotic Microangiopathy and Antiphospholipid Antibodies in Aicardi-Goutieres Syndrome - Report of Two Sisters

M. Rasmussen1 , K. Skullerud2 , S. J. Bakke3 , P. Lebon4 , F. L. Jahnsen2
  • 1Department of Pediatrics, Rikshospitalet, Oslo, Norway
  • 2Department and Institute of Pathology, Rikshospitalet, University of Oslo, Norway
  • 3Department of Radiology, Rikshospitalet, Oslo, Norway
  • 4Service de Virologie, Hôpital Saint-Vincent-de-Paul, Université Paris V, Paris, France
Further Information

Publication History

Received: July 15, 2004

Accepted after Revision: November 12, 2004

Publication Date:
09 February 2005 (online)

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Abstract

Cerebral thrombotic microangiopathy was found at autopsy in one of two sisters with Aicardi-Goutieres syndrome, whereas the other revealed increased serum levels of anticardiolipin IgG antibodies (measured only in the living sister); both typical features of systemic lupus erythematosus. These findings add support to the suggestion that Aicardi-Goutieres syndrome and systemic lupus erythematosus are closely related disorders in which dysregulated production of interferon-α might play a crucial role.

Key words

Aicardi-Goutieres syndrome - thrombotic microangiopathy - antiphospholipid antibodies - systemic lupus erythematosus - interferon

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Magnhild Rasmussen

Department of Pediatrics
Rikshospitalet

0027 Oslo

Norway

Email: magnhild.rasmussen@rikshospitalet.no