Ono S, Kurisaki H, Sakuma A, Nagao K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
J Neurol Sci. 1995 Feb;128(2):225-31. doi: 10.1016/0022-510x(94)00244-i.
We present a case of myotonic dystrophy accompanied by alveolar hypoventilation and hypersomnia. Case history, pulmonary function tests, polygraphic recording, and multiple sleep latency test, concomitant with a restrictive ventilatory abnormality, suggested a central origin of alveolar hypoventilation and hypersomnia in our case. The most significant neuropathological findings were in the tegmentum of the brain stem. Severe neuronal loss and gliosis were observed in the midbrain and pontine raphe, particularly in dorsal raphe nucleus and superior central nucleus. Pontine and medullary reticular formation also showed a marked cell loss and fibrillary gliosis. The alveolar hypoventilation and the hypersomnia in our case may be attributed to these morphological abnormalities, and would appear to be central in nature.
我们报告一例伴有肺泡低通气和发作性睡病的强直性肌营养不良病例。病史、肺功能测试、多导睡眠图记录以及多次睡眠潜伏期测试，同时伴有限制性通气异常，提示在我们的病例中肺泡低通气和发作性睡病起源于中枢。最显著的神经病理学发现位于脑干被盖部。中脑和脑桥中缝，尤其是背侧中缝核和中央上核，观察到严重的神经元丢失和胶质细胞增生。脑桥和延髓网状结构也显示出明显的细胞丢失和纤维性胶质细胞增生。我们病例中的肺泡低通气和发作性睡病可能归因于这些形态学异常，并且似乎本质上是中枢性的。
