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

Onozawa, R.; Tsuboi, Y.; Obata, T.; Inoue, H.; Yamada, T.; Miyake, K.

Rinsho Shinkeigaku 52(8): 567-570


ISSN/ISBN: 0009-918X
DOI: 10.5692/clinicalneurol.52.567
Accession: 068511079

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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 occipital lobe. Several different causes including malignant hypertension, eclampsia, renal failure, and use of immunosuppressants have 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 developed tension-type headache, nausea and vomiting, and bilateral loss of visual acuity. On admission, his visual acuity was markedly decreased without any abnormal findings in the optic fundus. There was no neurological deficit except the visual symptoms. Imaging of the head revealed multiple lesions in the white and gray matter of the bilateral occipital lobe and cerebellar hemisphere, which proved vasogenic edematous lesions by the hyperintense signals in T(2) weighted, FLAIR, and diffusion weighted images, suggesting the diagnosis of RPLS. Treatment with antihypertensive drug and glycerol was initiated and the patient made a full clinical recovery within a few days. The pathogenesis of RPLS is not fully understood. Our case was not on any immunosuppressant therapy at the time of onset of RPLS, and his hypertension was mild and transient without renal failure. It is possible that RPLS in our patient might be a manifestation related to Wegener's granulomatosis-mediated vascular endothelial injury.