Yamada A, Kamoda M, Ikezoe K, Tsukaguchi M, Katanaka J, Deguchi K, Miki H, Takeuchi H
Third Department of Internal Medicine, Kagawa Medical School, Japan.
No To Shinkei. 1993 Mar;45(3):273-6.
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 respiratory acidosis. Disorders of peripheral organs such as lung, airway, thorax and neuromuscular diseases were ruled out. Brain MRI showed cerebral infarction in the brain stem. We diagnosed him as CAH associated with brain stem infarction.
中枢性肺泡低通气综合征(CAH),即翁丁氏 curse,是一种非常罕见的疾病,其特征为脑干呼吸中枢功能障碍。在此,我们报告一例与脑梗死相关的 CAH 病例。一名 59 岁男性在 56 岁时出现右侧面部感觉减退。随后,面部感觉减退蔓延至左侧,同时出现构音障碍和吞咽困难。自 58 岁起,他经常发生呼吸衰竭和意识障碍。动脉血气分析显示肺泡低通气和呼吸性酸中毒。排除了肺、气道、胸廓等外周器官疾病以及神经肌肉疾病。脑部 MRI 显示脑干脑梗死。我们将其诊断为与脑干梗死相关的 CAH。