Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Idiopathic CD4-positive lymphocytopenia-associated progressive multifocal leukoencephalopathy confirmed by brain biopsy following negative results of repeated CSF-JC-virus tests: a case report

Yuya Kano, M.D.1), Hiroyasu Inoue, M.D.2), Keita Sakurai, M.D., Ph.D.3), Mari Yoshida, M.D., Ph.D.4), Yoshiharu Miura, M.D., Ph.D.5), Kazuo Nakamichi, Ph.D.6), Masayuki Saijo, M.D., Ph.D.6) and Hiroyuki Yuasa, M.D., Ph.D.1)

1)Department of Neurology, Tosei General Hospital
2)Department of Neurology, Nagoya City East Medical Center
3)Department of Radiology, Teikyo University School of Medicine
4)Medical Science of Aging, Aichi Medical University
5)Department of Neurology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital
6)Department of Virology 1, National Institute of Infectious Diseases

A 75-year-old man presented with dysarthria and left facial paralysis. Brain diffusion-weighted MRI revealed a highsignal intensity in the right precentral gyrus, and he was hospitalized under the diagnosis of cerebral infarction. His symptoms worsened and brain MRI findings were consistent with progressive multifocal leukoencephalopathy (PML). Cerebrospinal fluid (CSF) JC virus (JCV) was undetectable in the DNA polymerase chain reaction (PCR) test four times, but brain biopsy revealed typical PML histopathology. He had no human immunodeficiency virus infection and history of immunosuppressive treatment, but he was found to have CD4+ lymphocytopenia. He was treated with mefloquine and mirtazapine, and died 29 months after symptoms onset. In cases whose repeated DNA PCR results are negative for CSF JCV, brain biopsy may be useful for the diagnosis of PML.
Full Text of this Article in Japanese PDF (832K)

(CLINICA NEUROL, 58: 750|755, 2018)
key words: progressive multifocal leukoencephalopathy, idiopathic CD4+ lymphocytopenia, CSF-JCV DNA PCR, brain biopsy

(Received: 18-Sep-18)