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症例は59歳男性。56歳時より多発性脳幹梗塞による顔面の感覚鈍麻,構音・嚥下障害が出現し徐々に進行した。58歳時より呼吸困難感を伴わずに意識障害を来すようになり,肺胞低換気による呼吸不全が明らかとなった。しかし,肺・気道・胸郭系に異常はなく,また,横隔膜やその他の呼吸筋の運動障害を来す神経・筋疾患もみられず,脳幹梗塞による中枢性肺胞低換気症候群と診断した。本症候群は稀な病態であるが,脳血管障害に伴って発現することがあり,特に脳幹梗塞においては注意を払うべき病態と考えられた。
Central alveolar hypoventilation syndrome (CAH), or Ondine's curse, is a very rare disease characterized by dysfunction of respiratory center in the brain stem. Here, we report a case of CAH associated with cerebral infarction. A 59-year-old man developed right facial sensory deficit at age 56. Then, the facial sensory deficit spread to the left side and dysarthria and dysphagia also developed. Since age 58, he often developed respiratory failure and consciousness disturbance. Arterial blood gas analysis revealed alveolar hypoventilation and res-piratory acidosis. Disorders of peripheral organs such as lung, airway, thorax and neuromuscular diseases were ruled out. Brain MRI showed cerebrar infarction in the brain stem. We diagnosed him as CAH associated with brain stem infarction.
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