<Case report>Creutzfeldt-Jakob disease preceded by anesthetic blocks and surgical decompression of the facial nerve

  • IiDA Masashi
    Department of Neuropsychiatry, Kinki University School of Medicine
  • YOKOUCHI Toshio
    Department of Neuropsychiatry, Kinki University School of Medicine
  • KUSUBE Takeshi
    Department of Neuropsychiatry, Kinki University School of Medicine
  • YANAI Mika
    Department of Neuropsychiatry, Kinki University School of Medicine
  • SUGI Akira
    Department of Neuropsychiatry, Kinki University School of Medicine
  • TAKANO Morihide
    Department of Neuropsychiatry, Kinki University School of Medicine
  • HITOMI Kazuhiko
    Department of Neuropsychiatry, Kinki University School of Medicine
  • HIGASHITANI Norihiro
    Tamura-Kai Clinic
  • YAGI Yuji
    (Present addres) Department of Neurology, Kinki University School of Medicine
  • MAEDA Mitsuyo
    Second Department of Pathology, Kinki University School of Medicine
  • HASHIMOTO Shigeo
    Second Department of Pathology, Kinki University School of Medicine

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A 63-year-old woman had undergone ten facial nerve blocks and surgical decompression with Jannetta's manipulation of the facial nerve to treat right hemifacial spasms before the onset of Creutzfeldt-Jakob disease (CJD). The patient exhibited a prolonged clinical course extending over 49 months, during which she gradually manifested the following typical clinical features of CJD : rapidly advancing dementia resulting in an apallic state, myoclonic jerks synchronizing with periodic synchronous discharges on electroencephalography, and progressive cerebral atrophy on X-ray computerized tomography and magnetic resonance imaging. Postmortem examination revealed diffuse severe cerebral atrophy, particularly of the frontal and temporal lobes. The cerebellum and brain stem were moderately atrophic. The brain weighed 760 g. Microscopically, diffuse degeneration and loss of neurons and spongiform changes were observed in the cerebral cortex. Proliferation of hypertrophic and gemistocytic astrocytes was seen in the cerebral white matter. We attributed the extended course to strong biological resistance to CJD and other infectious diseases in the patient, and emphasized the risk of transmission of CJD agents by the nerve block procedure as well as the neurosurgical operation.

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