Beauvais P, Roubergue A, de Villemeur T B, Richardet J M
Service de Pédiatrie, Neurologie et Pathologie du développement, Hôpital Trousseau, Paris.
Arch Fr Pediatr. 1988 Nov;45(9):653-5.
A case of pons-bulbar palsy in a 10 year-old girl is reported. The authors insist on the fact that motor cranial nerve nuclear dysfunction was isolated, without any pyramidal tract disorder. They also emphasize the slow course of the disease, which after one year of quick progression was stable for the next 5 years. Nineteen identical cases were found in the literature. From this nosographic group, one must exclude patients with associated lateral spinal tract dysfunction: these patients must be discussed with the juvenile or infantile amyotrophic lateral sclerosis group. The heterogeneity of the pons-bulbar paralysis group is also underlined: the course may be quickly fatal or, on the contrary, protracted for a long time; genetic transmission is also quite variable.
报告了一例10岁女孩的脑桥延髓麻痹病例。作者强调运动性颅神经核功能障碍是孤立存在的，没有任何锥体束疾病。他们还强调了疾病进展缓慢，在快速进展一年后，接下来的5年病情稳定。文献中发现了19例相同病例。在这个疾病分类组中，必须排除伴有脊髓侧束功能障碍的患者：这些患者应与青少年或婴儿型肌萎缩侧索硬化症组一起讨论。脑桥延髓麻痹组的异质性也得到了强调：病程可能很快致命，或者相反，会长期迁延；遗传传递也有很大差异。
