クロイツフェルト・ヤコブ病の神経耳科学的所見

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  • Neuro-Otological Evaluation of Creutzfeldt-Jacob Disease
  • 臨床 クロイツフェルト・ヤコブ病の神経耳科学的所見
  • リンショウ クロイツフェルト ヤコブビョウ ノ シンケイ ジ カガクテキ ショケン

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A 66-year-old man visited our Medical Center at the end of June, 2009 with an approximately 3 week history of general fatigue, feeling depression and dizziness. First, general and neurological examinations were performed, but there were no abnormal findings. Within 1 week after the first examination, the patient's family noticed that he was talking and acting strangely. These symptoms rapidly worsened, and he was admitted on July 1st. At that time, he showed the cerebellar ataxia such as the poorness of the finger to nose test and wide based gait. Diffusion-weight imaging (DWI) Magnetic resonance imaging (MRI) showed a high intensity change in the right side of the cerebral cortex and basal ganglia but no remarkable change in the cerebellum except for slight atrophy. Electroencephalography showed a typical periodic synchronous discharge. Based on the above findings, the patient was diagnosed as having probable Creutzfeldt-Jacob disease (CJD).<br> We performed neuro-otological examinations on the 9-10th July. They showed bilateral gaze evoked nystagmus and ataxic pursuit, diminished optokinetic nystagmus, and the disappearance of visual suppression on a caloric test. By the end of July, following the rapid development of dementia and generalized myoclonus, the patient became bedridden. By mid-August, he had become akinetic and mute. Based on our neuro-otological evaluation the final diagnosis was CJD. In the early period of onset of CJD, the cerebellar ataxia was not reflected on the MRI findings, but this diagnosis was suggested by the abnormal eye movement. Dizziness is one of the initial signs of CJD, so otorhinolaryngologists should bear CJD in mind if a patient shows symptoms of cerebellar ataxia<br>

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