Article

Reversible posterior leukoencephalopathy syndrome: a possible manifestation of Wegener's granulomatosis-mediated endothelial injury.

Department of Internal Medicine, Osaka City General Hospital, 2-13-22 Miyakojima-Hondori, Miyakojima-ku, Osaka, Japan.
Modern Rheumatology (impact factor: 1.58). 02/2008; 18(3):309-14. DOI:10.1007/s10165-008-0052-1 pp.309-14
Source: PubMed

ABSTRACT We present the case of a 15-year-old girl who had Wegener's granulomatosis with severe intestinal involvement. During the clinical course, she developed generalized seizures and was diagnosed with reversible posterior leukoencephalopathy syndrome (RPLS). Plasma exchange combined with steroid pulse therapy was initiated and showed marked improvement. This is one of the few cases of RPLS without severe hypertension or renal failure, suggesting that RPLS is likely to be a manifestation of Wegener's granulomatosis-mediated endothelial injury.

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Keywords

clinical course
 
generalized seizures
 
reversible posterior leukoencephalopathy syndrome
 
severe hypertension
 
severe intestinal involvement
 
Wegener's granulomatosis-mediated endothelial injury
 

Minako Nishio