Case Report

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

Authors

Minako Nishio¹, Katsunobu Yoshioka¹, Keiko Yamagami¹, Takashi Morikawa¹, Yoshio Konishi¹, Noriko Hayashi², Kimihide Himuro² and Masahito Imanishi¹

1. Department of Internal Medicine, Osaka City General Hospital, 2-13-22 Miyakojima-Hondori, Miyakojima-ku Osaka, 534-0021, Japan
2. Department of Neurology, Osaka City General Hospital, 2-13-22 Miyakojima-Hondori, Miyakojima-ku Osaka, 534-0021, Japan

Received:

25 December 2007

Accepted:

30 January 2008

Published online:

17 April 2008

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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.

Key words

Reversible posterior leukoencephalopathy syndrome (RPLS) - Vasculitis - Wegener’s granulomatosis