Recurrent Epstein-Barr Virus-positive (EBV+) Primary Central Nervous System Lymphoma (PCNSL) in a Patient with Clinical Features of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)

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  • Nagano Masahiro
    Department of Neurology, Kyoto University Graduate School of Medicine, Japan
  • Ayaki Takashi
    Department of Neurology, Kyoto University Graduate School of Medicine, Japan
  • Koita Natsuko
    Department of Neurology, Kyoto University Graduate School of Medicine, Japan
  • Kitano Toshiyuki
    Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan
  • Nishikori Momoko
    Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan
  • Goda Naoki
    Department of Diagnostic Pathology, Kyoto University Graduate School of Medicine, Japan
  • Minamiguchi Sachiko
    Department of Diagnostic Pathology, Kyoto University Graduate School of Medicine, Japan
  • Ikeda Akio
    Department of Neurology, Kyoto University Graduate School of Medicine, Japan Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine, Japan
  • Takaori-Kondo Akifumi
    Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Japan
  • Takahashi Ryosuke
    Department of Neurology, Kyoto University Graduate School of Medicine, Japan

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Abstract

<p>Primary central nervous system lymphoma (PCNSL) and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) can share clinical features and may be indistinguishable, even after brain biopsy. We encountered a case of Epstein-Barr virus-positive (EBV+) PCNSL recurrence in a patient with clinical features of CLIPPERS, and repeat brain biopsy was required to reach the correct diagnosis. Four years after the initial diagnosis and treatment of PCNSL, "peppering" punctate enhanced lesions with transient steroid responsiveness were detected during brain magnetic resonance imaging (MRI). A second brain biopsy supported a diagnosis of CLIPPERS, while a third biopsy confirmed the diagnosis of recurrent PCNSL. </p>

Journal

  • Internal Medicine

    Internal Medicine 58 (6), 849-854, 2019-03-15

    The Japanese Society of Internal Medicine

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