Anti-Hu-associated Paraneoplastic Encephalomyelitis with Esophageal Small Cell Carcinoma

  • Shirafuji Toshihiko
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Kanda Fumio
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Sekiguchi Kenji
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Higuchi Masatsugu
    Division of Neurology, Kobe University Graduate School of Medicine, Japan
  • Yokozaki Hiroshi
    Division of Surgical Pathology, Kobe University Graduate School of Medicine, Japan
  • Tanaka Keiko
    Department of Neurology, Kanazawa Medical University, Japan
  • Takahashi Hitoshi
    Department of Pathology, Brain Research Institute, University of Niigata, Japan
  • Toda Tatsushi
    Division of Neurology, Kobe University Graduate School of Medicine, Japan

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Abstract

A 63-year-old woman had anti-Hu-associated paraneoplastic encephalomyelitis (anti-Hu syndrome) caused by esophageal small cell carcinoma (SCC). The patient developed bilateral limbic encephalitis, followed by myelitis, brain stem encephalitis, and autonomic failure. Extensive examination demonstrated SCC of the abdominal lymph nodes that was retrospectively diagnosed as metastasis of esophageal SCC on autopsy. The neuropathological findings were characterized by widespread neuronal loss and gliosis in the central nervous system, as well as patchy loss of myelin and axons in the spinal nerve roots with perivascular lymphocytic infiltration. This is the first detailed clinical and neuropathological report of anti-Hu syndrome caused by esophageal SCC.<br>

Journal

  • Internal Medicine

    Internal Medicine 51 (17), 2423-2427, 2012

    The Japanese Society of Internal Medicine

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