Ouvrier R A, Billson F
Department of Neurology, Royal Alexandra Hospital for Children, Camperdown, Australia.
J Child Neurol. 1988 Jul;3(3):177-80. doi: 10.1177/088307388800300305.
Four cases of an apparently benign ocular motor syndrome of childhood are reported. The features of the disorder are: (1) onset in early life; (2) periods of constant or variably sustained tonic conjugate upward deviation of the eyes; (3) down-beating saccades in attempted downgaze, which are difficult to sustain below the neutral positions; (4) apparently normal horizontal eye movements; (5) frequent relief by sleep; (6) otherwise normal neurological findings apart from mild ataxia, chronic in one boy and at times of illness in one of the other patients; (7) absence of deterioration during observation spanning up to 15 years; (8) eventual improvement but with some residual ocular movement problems in two cases; (9) normal metabolic, electroencephalographic, and neuroradiologic investigations; (10) normal brain examination findings in one patient who died accidentally; and (11) an apparently good response to levodopa therapy in one patient. To the authors' knowledge, this condition has not been described previously. It may be a new levodopa-responsive condition, secondary to a localized neurotransmitter deficiency.
报告了4例儿童期明显良性的眼球运动综合征病例。该疾病的特征为：（1）发病于早年；（2）双眼持续或可变的强直性共轭向上偏斜期；（3）试图向下注视时出现向下跳动性扫视，难以维持在中立位置以下；（4）水平眼球运动明显正常；（5）睡眠常可缓解；（6）除一名男孩有慢性轻度共济失调、另一名患者在患病时有轻度共济失调外，其他神经系统检查结果正常；（7）在长达15年的观察期内无病情恶化；（8）两例最终有所改善，但仍有一些残留的眼球运动问题；（9）代谢、脑电图和神经放射学检查正常；（10）一名意外死亡患者的脑部检查结果正常；（11）一名患者对左旋多巴治疗反应明显良好。据作者所知，此前尚未描述过这种疾病。它可能是一种新的对左旋多巴有反应的疾病，继发于局部神经递质缺乏。
