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Successful treatment of non-HIV progressive multifocal leukoencephalopathy: case report and literature review

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Abstract

Background

Progressive multifocal leukoencephalopathy (PML) is a subacute onset demyelinating disease caused by JC virus and characterized by multifocal involvement of the subcortical white matter and cerebellar hemispheres or peduncles on magnetic resonance imaging (MRI). However, non-HIV PML patients with brain lesions limited to the cerebellum and brainstem have not been well characterized.

Methods

We report a 68-year-old man with systemic lupus erythematosus under treatment with immunosuppressants who developed non-HIV PML with brain lesions limited to the cerebellum and brainstem and successfully treated with a combination of mefloquine and mirtazapine. We performed a literature review to characterize patients with non-HIV PML with brain lesions limited to the cerebellum and brainstem.

Results

Eight cases with non-HIV brainstem/cerebellar form PML were identified including our case. All cases had compromised status related underlying diseases. Four (50%) had a good prognosis. Five cases were treated, including 3 with favourable outcomes. Between the good prognosis group (n = 4) and the poor prognosis group (n = 4), treatment status for PML and the interval between the initial manifestation and diagnosis did not differ. Among those who performed contrast-enhanced brain imaging, lesion enhancement was related to good prognosis (good prognosis group vs. poor prognosis group; 100% vs. 0%).

Conclusion

PML should be considered in the differential diagnosis of brain lesions limited to the cerebellum and brainstem in immunocompromised patients. The presence of immune response against JC virus and inflammatory reactions may indicate good prognosis in non-HIV brainstem/cerebellar form PML

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Acknowledgements

We thank the patient for his participation in this study. We wish to thank Dr. Nobuaki Funata, Department of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital for his help with pathological diagnosis.

Funding

This work was partly supported by a Grant-in-Aid for the Research Committee of Prion Disease and Slow Virus Infection, Research on Policy Planning and Evaluation for Rare and Intractable Diseases from the Ministry of Health, Labour and Welfare of Japan (Grant Number H29-Nanchitou (Nan)-Ippan-036) and by JSPS KAKENHI (Grant Number 17K09768).

Author information

Authors and Affiliations

  1. Department of Neurology, Dokkyo Medical University, Shimotsuga, 321-0293, Tochigi, Japan

    Mai Hamaguchi, Keisuke Suzuki, Hiroaki Fujita & Koichi Hirata

  2. Department of Neurosurgery, Dokkyo Medical University, Shimotsuga, Japan

    Takeo Uzuka, Hadzki Matsuda & Shigeru Kikuchi

  3. Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Kyoto, Japan

    Yukiko Shishido-Hara

  4. Department of Rheumatology, Dokkyo Medical University, Shimotsuga, Japan

    Satoko Arai

  5. Department of Neurology, Rehabilitation Amakusa Hospital, Saitama, Japan

    Toshiki Nakamura

  6. Department of Virology 1, National Institute of Infectious Diseases, Tokyo, Japan

    Kazuo Nakamichi & Masayuki Saijo

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  1. Mai Hamaguchi
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  2. Keisuke Suzuki
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  3. Hiroaki Fujita
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  4. Takeo Uzuka
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  10. Kazuo Nakamichi
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  11. Masayuki Saijo
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  12. Koichi Hirata
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Contributions

All authors have read and approved the manuscript and contributed to the design of the study and interpretation of data. MH, KS and HF drafted the manuscript. MH, TU, HM, SA, SK, Y S–H, KN and MS contributed to the diagnosis and treatment of the patient. TU, Y S–H, TN and KH revised the manuscript.

Corresponding author

Correspondence to Keisuke Suzuki.

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The authors declare no potential conflicts of interest in relation to this article.

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Not applicable.

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Written informed consent was obtained from the patient included in the study.

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Hamaguchi, M., Suzuki, K., Fujita, H. et al. Successful treatment of non-HIV progressive multifocal leukoencephalopathy: case report and literature review. J Neurol 267, 731–738 (2020). https://doi.org/10.1007/s00415-019-09629-x

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  • DOI: https://doi.org/10.1007/s00415-019-09629-x

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