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

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  • Onozawa Rieko
    Department of Neurology, Fukuoka University School of Medicine
  • Tsuboi Yoshio
    Department of Neurology, Fukuoka University School of Medicine
  • Obata Toyoshi
    Department of Neurology, Fukuoka University School of Medicine
  • Inoue Hirosato
    Department of Neurology, Fukuoka University School of Medicine
  • Yamada Tatsuo
    Department of Neurology, Fukuoka University School of Medicine
  • Miyake Katsuhisa
    Division of Nephrology and rheumatology department of internal medicine, Fukuoka University school of Medicine

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Other Title
  • Wegener肉芽腫症治療中に発症したReversible posterior leukoencephalopathy syndrome
  • 症例報告 Wegener肉芽腫症治療中に発症したReversible posterior leukoencephalopathy syndrome
  • ショウレイ ホウコク Wegener ニクガ シュショウ チリョウ チュウ ニ ハッショウ シタ Reversible posterior leukoencephalopathy syndrome

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Abstract

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 T2 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.<br>

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 52 (8), 567-570, 2012

    Societas Neurologica Japonica

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