Shawkat F S, Harris C M, Taylor D S, Kriss A
Department of Ophthalmology, Great Ormond Street Hospital for Children, London, UK.
Eye (Lond). 1996;10 ( Pt 1):53-60. doi: 10.1038/eye.1996.8.
Ocular motor apraxia (OMA) is characterised by an intermittent inability to initiate voluntary saccades, and a failure to produce optokinetic and vestibular quick phases. Some patients have no other abnormalities (idiopathic OMA), whereas in others it appears associated with a variety of neurological conditions which may affect the sensory visual pathway. Electroretinograms (ERGs), flash and pattern visual evoked potentials (VEPs) and eye movements were assessed in 53 children with OMA (age range 17 days to 14 years) to determine their efficacy in helping to distinguish between idiopathic and non-idiopathic cases. Seven patients (13.2%) had idiopathic OMA and the remaining 46 (86.8%) had other associated clinical conditions. All patients had episodes of absent quick phases ('lock up') during optokinetic nystagmus (OKN) and/or vestibular testing. Flash ERGs were abnormal in only 7 patients (13.2%); 6 had syndromes involving a pigmentary retinopathy (Joubert's, Bardet-Biedl, infantile Refsum's, Kearns-Sayre's), and the seventh had a cone dystrophy with vermis hypoplasia. VEPs were normal in all 7 patients with idiopathic OMA. Thirty-three (72%) patients with OMA in association with neurological conditions had abnormal VEPs and 13 had normal VEPs (28%). There was a significant positive correlation between VEP abnormality and poor OKN gain. VEP/ERG testing and eye movement studies are useful when OMA is suspected as they help in distinguishing isolated idiopathic cases from those with more widespread neurological abnormalities.
眼球运动失用症（OMA）的特征是间歇性无法启动随意性扫视，以及不能产生视动性和前庭性快相。一些患者没有其他异常（特发性OMA），而在另一些患者中，它似乎与多种可能影响感觉视觉通路的神经系统疾病有关。对53例OMA患儿（年龄范围为17天至14岁）进行了视网膜电图（ERG）、闪光和图形视觉诱发电位（VEP）以及眼动评估，以确定它们在区分特发性和非特发性病例方面的效用。7例患者（13.2%）患有特发性OMA，其余46例（86.8%）有其他相关临床疾病。所有患者在视动性眼球震颤（OKN）和/或前庭测试期间均有快相缺失（“锁定”）发作。仅7例患者（13.2%）的闪光ERG异常；6例患有涉及色素性视网膜病变的综合征（Joubert综合征、Bardet-Biedl综合征、婴儿型Refsum综合征、Kearns-Sayre综合征），第7例患有伴有小脑蚓部发育不全的视锥营养不良。7例特发性OMA患者的VEP均正常。33例（72%）与神经系统疾病相关的OMA患者VEP异常，13例VEP正常（28%）。VEP异常与OKN增益不佳之间存在显著正相关。当怀疑有OMA时，VEP/ERG测试和眼动研究很有用，因为它们有助于区分孤立的特发性病例和那些有更广泛神经系统异常的病例。
