Reversible posterior leukoencephalopathy syndrome: a possible manifestation of Wegener's granulomatosis-mediated endothelial injury.
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.
Nishio
Minako
M
Department of Internal Medicine, Osaka City General Hospital, 2-13-22 Miyakojima-Hondori, Miyakojima-ku, Osaka, Japan. chaby_john@yahoo.co.jp
Yoshioka
Katsunobu
K
Yamagami
Keiko
K
Morikawa
Takashi
T
Konishi
Yoshio
Y
Hayashi
Noriko
N
Himuro
Kimihide
K
Imanishi
Masahito
M
D000293:Adolescent; D004727:Endothelium / Q000473:pathology; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging; D010951:Plasma Exchange; D054038:Posterior Leukoencephalopathy Syndrome / Q000209:etiology* / Q000473:pathology* / Q000628:therapy; D020551:Pulse Therapy, Drug; D013256:Steroids / Q000627:therapeutic use
D013256:Steroids