A case report of progressive multifocal leukoencephalopathy during steroid treatment for ANCA-associated renal vasculitis

CEN Case Reports volume 9, pages354–358(2020)Cite this article

Abstract

Case report: an 80-year-old woman presented with rapidly progressive glomerulonephritis and was admitted to our hospital. Myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) was positive. We diagnosed ANCA-associated renal vasculitis (ANCA-RV). Treatment was initiated with intravenous methylprednisolone pulse therapy, followed by prednisolone (PSL) at 30 mg/day. We gradually reduced the PSL dose to 7.5 mg/day over 6 months. At that time, the patient developed disturbances of consciousness which progressed subacutely. MRI revealed regions of patchy white matter with an increased signal on T2-weighted, fluid attenuated inversion recovery (FLAIR) sequences and diffusion-weighted sequences. JC virus DNA was detected in the cerebrospinal fluid (CSF) by polymerase chain reaction (PCR), leading to a diagnosis of progressive multifocal leukoencephalopathy (PML). PML is a rare infectious demyelinating disease of the central nervous system caused by JC virus infection, occurring in highly immunosuppressed individuals such as HIV-infected patients and patients using some biological agents, and having a very poor prognosis. In the present case, PML may have been associated with steroid use, although there are very few case reports of PML in patients taking only steroids. We report progressive multifocal leukoencephalopathy during steroid treatment of ANCA-RV. When patients show progressive disturbance of consciousness during treatment for ANCA-RV, we need to take PML into consideration for differential diagnosis.

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Acknowledgements

This survey was supported in part by a Grant-in-Aid for Intractable (former name, Progressive) Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan.

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  1. Division of Clinical Medicine, Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennoudai, Tsukuba, Ibaraki, 305-8575, Japan

    Takashi Tawara, Hirayasu Kai, Mikiko Kageyama, Tomoki Akiyama, Takahiro Matsunaga, Aki Sakuma, Ryota Ishii, Ryouya Tsunoda, Tetusya Kawamura, Akiko Fujita, Shuzo Kaneko, Naoki Morito, Chie Saito, Joichi Usui & Kunihiro Yamagata

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  1. Takashi Tawara
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  2. Hirayasu Kai
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Correspondence to Kunihiro Yamagata.

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Tawara, T., Kai, H., Kageyama, M. et al. A case report of progressive multifocal leukoencephalopathy during steroid treatment for ANCA-associated renal vasculitis. CEN Case Rep 9, 354–358 (2020). https://doi.org/10.1007/s13730-020-00482-w

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Keywords

  • Progressive multifocal leukoencephalopathy
  • ANCA-associated renal vasculitis
  • Steroid treatment
  • Rapidly progressive glomerulonephritis