Abstract
Progressive multifocal leukoencephalopathy occurs almost exclusively in immunosuppressed individuals. Mirtazapine, a 5-hydroxytryptamine-2A antagonist, has been used empirically against progressive multifocal leukoencephalopathy. A 60-year-old man who was diagnosed with sarcoidosis at 20 years-of-age and had not taken immunosuppressive therapies developed dysarthria, left hemiparesis and dressing apraxia. Cranial ?uid-attenuated inversion-recovery magnetic resonance imaging showed hyperintense areas in the right frontal and temporoparietal white matter. The initial diagnosis was neurosarcoidosis, based on non-caseating granuloma in a lymph node biopsy. Immunosuppressive therapy was initiated; however, this aggravated his neurological symptoms. Immunohistochemistry of the brain biopsy showed John Cunningham virus infected large ballooned oligodendrocytes, leading to a diagnosis of progressive multifocal leukoencephalopathy. Treatment with mirtazapine improved the neurological symptoms, and the magnetic resonance imaging abnormality did not progress. We stress the diagnostic difficulties of distinguishing progressive multifocal leukoencephalopathy from neurosarcoidosis, and suggest that mirtazapine treatment is effective against progressive multifocal leukoencephalopathy associated with sarcoidosis.
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