Marr J E, Green S H, Willshaw H E
Hallamshire Hospital, Sheffield, UK.
Dev Med Child Neurol. 2005 Dec;47(12):815-9. doi: 10.1017/S0012162205001726.
Ocular motor apraxia (OMA), a disorder of saccadic initiation, may be congenital or acquired. While the acquired form is frequently associated with significant neuropathology, the congenital form is often regarded as relatively benign. Many children with congenital OMA who were observed clinically have shown neurodevelopmental disturbance over time. A retrospective review was taken of 34 consecutive patients (22 males and 12 females), seen over a 20-year period, to evaluate the frequency and type of associated neurodevelopmental problems. Age at presentation ranged from 8 weeks to 14 years, with a mean age of 10 years. Of 29 children with congenital OMA, 15 had imaging evidence of structural central nervous system abnormalities (with cerebellar hypoplasia the most frequent abnormality detected). Eleven of the 14 patients with no structural abnormality showed abnormal neurodevelopment. This study suggests that congenital OMA is not a benign diagnosis, even in the absence of overt neurological disturbance at the time of presentation.
眼球运动失用症（OMA）是一种扫视启动障碍，可分为先天性或后天性。虽然后天性形式常与严重的神经病理学相关，但先天性形式通常被认为相对良性。许多临床上观察到的先天性OMA患儿随着时间推移出现了神经发育障碍。对20年间连续就诊的34例患者（22例男性和12例女性）进行了回顾性研究，以评估相关神经发育问题的频率和类型。就诊时年龄范围为8周至14岁，平均年龄为10岁。在29例先天性OMA患儿中，15例有中枢神经系统结构异常的影像学证据（小脑发育不全是最常见的异常）。14例无结构异常的患者中有11例神经发育异常。这项研究表明，即使在就诊时没有明显的神经功能障碍，先天性OMA也不是一个良性诊断。
