Wang P J, Chen R L, Shen Y Z
Department of Pediatrics, National Taiwan University Hospital, Taipei, Republic of China.
Pediatr Neurol. 1989 Nov-Dec;5(6):381-3. doi: 10.1016/0887-8994(89)90054-4.
A Taiwanese boy, 1 year, 8 months of age, is reported with poor weight gain, hypotonia, trunk ataxia, motor developmental retardation, and horizontal pendular nystagmus with only wave I on auditory brainstem responses. Our patient clinically resembled 9 patients reported in the Japanese literature. Because of its male predominance, occurrence in siblings, early onset, nonprogressive course, and characteristic auditory brainstem response findings, the syndrome may be of genetic origin and attributable to a dysgenetic brainstem lesion.
据报道，一名1岁8个月大的台湾男孩体重增加缓慢、肌张力减退、躯干共济失调、运动发育迟缓，且听觉脑干反应仅见I波的水平摆动性眼球震颤。我们的患者在临床上与日本文献报道的9例患者相似。由于该综合征男性居多、在兄弟姐妹中出现、发病早、病程无进展以及具有特征性的听觉脑干反应表现，可能起源于遗传，归因于脑干发育异常性病变。
