Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Reversible posterior leukoencephalopathy syndrome (RPLS) associated with Wegener's granulomatosis:A case report and review of the literature

Rieko Onozawa, M.D1), Yoshio Tsuboi, M.D1), Toyoshi Obata, M.D1), Hirosato Inoue, M.D1), Tatsuo Yamada, M.D1) and Katsuhisa Miyake, M.D2)

1)Department of Neurology, Fukuoka University School of Medicine
2)Division of Nephrology and rheumatology department of internal medicine, Fukuoka University school of Medicine

Reversible posterior leukoencephalopathy syndrome (RPLS) is characterized by sudden onset of headaches,visual disorders, decreased consciousness, and convulsion associated with brain edema occurring in the occipitallobe. Several different causes including malignant hypertension, eclampsia, renal failure, and use of immunosuppressantshave been reported in patients with RPLS. Our patient was a 45-year-old man who presented with fever,arthralgia, and melena approximately 1 year previously and received the diagnosis of Wegener's granulomatosis.Following steroid therapy his symptoms ameliorated; however, during the course of the illness he developedtension-type headache, nausea and vomiting, and bilateral loss of visual acuity. On admission, his visual acuity wasmarkedly decreased without any abnormal findings in the optic fundus. There was no neurological deficit exceptthe visual symptoms. Imaging of the head revealed multiple lesions in the white and gray matter of the bilateraloccipital lobe and cerebellar hemisphere, which proved vasogenic edematous lesions by the hyperintense signalsin T2 weighted, FLAIR, and diffusion weighted images, suggesting the diagnosis of RPLS. Treatment with antihypertensivedrug and glycerol was initiated and the patient made a full clinical recovery within a few days. Thepathogenesis of RPLS is not fully understood. Our case was not on any immunosuppressant therapy at the time ofonset of RPLS, and his hypertension was mild and transient without renal failure. It is possible that RPLS in ourpatient might be a manifestation related to Wegener's granulomatosis-mediated vascular endothelial injury.
Full Text of this Article in Japanese PDF (325K)

(CLINICA NEUROL, 52: 567|570, 2012)
key words: Wegener's granulomatosis, Reversible posterior leukoencephalopathy syndrome (RPLS), visual disorders

(Received: 11-Jan-12)