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

  • Kano Yuya
    Department of Neurology, Tosei General Hospital
  • Inoue Hiroyasu
    Department of Neurology, Nagoya City East Medical Center
  • Sakurai Keita
    Department of Radiology, Teikyo University School of Medicine
  • Yoshida Mari
    Medical Science of Aging, Aichi Medical University
  • Miura Yoshiharu
    Department of Neurology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital
  • Nakamichi Kazuo
    Department of Virology 1, National Institute of Infectious Diseases
  • Saijo Masayuki
    Department of Virology 1, National Institute of Infectious Diseases
  • Yuasa Hiroyuki
    Department of Neurology, Tosei General Hospital

Bibliographic Information

Other Title
  • 髄液JCウイルス(JCV)-DNA PCR陰性で脳生検で診断した,特発性CD4陽性リンパ球減少症が関連した進行性多巣性白質脳症の1例

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<p>A 75-year-old man presented with dysarthria and left facial paralysis. Brain diffusion-weighted MRI revealed a high-signal 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.</p>

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 58 (12), 750-755, 2018

    Societas Neurologica Japonica

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