Case report: Progressive multifocal leukoencephalopathy co-occurring with neurosarcoidosis: early brain biopsy and appropriate therapy for PML resulted in a favorable prognosis

Qiannan Wang; Shintaro Tsuboguchi; Kouichirou Okamoto; Mari Tada; Akiyoshi Kakita; Kazuo Nakamichi; Makoto Oishi; Masato Kanazawa; Osamu Onodera

doi:10.3389/fimmu.2024.1447992

Case report: Progressive multifocal leukoencephalopathy co-occurring with neurosarcoidosis: early brain biopsy and appropriate therapy for PML resulted in a favorable prognosis

Front Immunol. 2024 Oct 11:15:1447992. doi: 10.3389/fimmu.2024.1447992. eCollection 2024.

Authors

Qiannan Wang¹, Shintaro Tsuboguchi¹, Kouichirou Okamoto², Mari Tada³, Akiyoshi Kakita³, Kazuo Nakamichi⁴, Makoto Oishi⁵, Masato Kanazawa¹, Osamu Onodera¹

Affiliations

¹ Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan.
² Department of Translational Research, Brain Research Institute, Niigata University, Niigata, Japan.
³ Department of Pathology, Brain Research Institute, Niigata University, Niigata, Japan.
⁴ Department of Virology 1, National Institute of Infectious Diseases, Tokyo, Japan.
⁵ Department of Neurosurgery, Brain Research Institute, Niigata University, Niigata, Japan.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare central nervous system disease caused by JC virus (JCV) infection. Human immunodeficiency virus (HIV) infection is the greatest risk factor for PML. Other immunological diseases, including systemic sarcoidosis, have also been reported as risk factors for PML. Herein, we report a case of PML co-occurring with neurosarcoidosis. Early diagnosis using brain biopsy and appropriate therapeutic interventions achieved favorable outcomes. PML in patients with active intracranial neurosarcoidosis is extremely rare. We believe that it is important to perform brain biopsy at an early stage to allow diagnosis, even for central nervous system involvement with a progressive parenchymal lesion in patients with sarcoidosis, if PML is possible.

Keywords: PML; avoiding immunosuppressive therapy; brain biopsy; favorable prognosis; neurosarcoidosis.

Publication types

Case Reports

MeSH terms

Biopsy
Brain* / pathology
Central Nervous System Diseases* / diagnosis
Central Nervous System Diseases* / etiology
Central Nervous System Diseases* / pathology
Central Nervous System Diseases* / therapy
Female
Humans
JC Virus / isolation & purification
Leukoencephalopathy, Progressive Multifocal* / diagnosis
Leukoencephalopathy, Progressive Multifocal* / pathology
Leukoencephalopathy, Progressive Multifocal* / therapy
Magnetic Resonance Imaging
Male
Middle Aged
Prognosis
Sarcoidosis* / complications
Sarcoidosis* / diagnosis
Treatment Outcome

Supplementary concepts

Neurosarcoidosis

Grants and funding

The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This work was partly supported by the Research Committee of Prion Disease and Slow Virus Infection, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health and Labor Sciences Research Grants, Ministry of Health, Labor and Welfare, Japan, and JSPS KAKENHI (grant no. 21K07450).