Ono S, Takahashi K, Jinnai K, Kanda F, Fukuoka Y, Kurisaki H, Mitake S, Inagaki T, Yamano T, Shimizu N, Nagao K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Chiba, Japan.
Neurology. 1998 Oct;51(4):1121-4. doi: 10.1212/wnl.51.4.1121.
To clarify the possible relation between the extent of involvement of catecholaminergic neurons and the presence of alveolar hypoventilation in patients with myotonic dystrophy (MyD).
Respiratory insufficiency has been reported frequently in MyD patients. Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the CNS.
The authors performed a quantitative immunoreactive study of tyrosine hydroxylase immunoreactive (TH+) neurons linked to hypoventilation in the dorsal central medullary nucleus (DCMN), the ventral central medullary nucleus (VCMN), and the subtrigeminal medullary nucleus (SMN)--where the autonomic respiratory center is thought to be located--in eight MyD patients and in 10 age-matched control subjects. Alveolar hypoventilation of the central type was present in three of the MyD patients but not in the remaining MyD patients or the control subjects.
The densities of TH+ neurons of the DCMN, the VCMN, and the SMN in MyD patients with hypoventilation were significantly lower than in those without hypoventilation (p < 0.02, p < 0.01, and p < 0.01, respectively) and control subjects (p < 0.01, p < 0.01, and p < 0.01, respectively).
These data suggest that the loss of TH+ neurons of the DCMN, the VCMN, and the SMN is associated with the presence of hypoventilation in MyD and may be an important feature of MyD.
阐明强直性肌营养不良（MyD）患者中儿茶酚胺能神经元受累程度与肺泡低通气之间的可能关系。
MyD患者中呼吸功能不全的报道屡见不鲜。近期数据支持这样一种假说，即这种呼吸衰竭源于中枢神经系统的原发性功能障碍。
作者对8例MyD患者和10例年龄匹配的对照受试者进行了一项定量免疫反应性研究，研究与低通气相关的酪氨酸羟化酶免疫反应阳性（TH+）神经元，这些神经元位于延髓背侧中央核（DCMN）、延髓腹侧中央核（VCMN）和三叉神经下核（SMN），自主呼吸中枢被认为位于这些部位。8例MyD患者中有3例存在中枢型肺泡低通气，其余MyD患者及对照受试者均无此情况。
存在低通气的MyD患者中，DCMN、VCMN和SMN的TH+神经元密度显著低于无低通气的MyD患者（分别为p < 0.02、p < 0.01和p < 0.01）及对照受试者（分别为p < 0.01、p < 0.01和p < 0.01）。
这些数据表明，DCMN、VCMN和SMN的TH+神经元缺失与MyD患者的低通气有关，可能是MyD的一个重要特征。
