Ono S, Kanda F, Takahashi K, Fukuoka Y, Jinnai K, Kurisaki H, Mitake S, Inagaki T, Nagao K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
Neurology. 1996 Jan;46(1):228-31. doi: 10.1212/wnl.46.1.228.
Respiratory insufficiency occurs frequently in patients with myotonic dystrophy (MyD). We have performed a quantitative study of neurons linked to respiratory function in the dorsal central medullary nucleus (DCMN), the ventral central medullary nucleus (VCMN), and the subtrigeminal medullary nucleus (SMN) in seven patients with MyD and eight age-matched controls. Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The densities of neurons of the DCMN, the VCMN, and the SMN in MyD patients with hypoventilation were significantly lower than in MyD without hypoventilation and controls. These data suggest the neuronal loss of the DCMN, VCMN, and SMN is associated with the presence of hypoventilation in MyD and may be an important feature of MyD.
呼吸功能不全在强直性肌营养不良（MyD）患者中经常出现。我们对7例MyD患者和8例年龄匹配的对照者的延髓背侧中央核（DCMN）、延髓腹侧中央核（VCMN）和三叉神经下核（SMN）中与呼吸功能相关的神经元进行了定量研究。3例MyD患者出现中枢型肺泡通气不足，其余MyD患者和对照者未出现。通气不足的MyD患者的DCMN、VCMN和SMN神经元密度显著低于无通气不足的MyD患者和对照者。这些数据表明，DCMN、VCMN和SMN的神经元丢失与MyD患者通气不足的存在有关，可能是MyD的一个重要特征。
