Clinical evaluation of eye movements in spinocerebellar ataxias: a prospective multicenter study.
Clinical evaluation of eye movements in spinocerebellar ataxias: a prospective multicenter study.
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脊椎动物共济失调中眼动运动的临床评估：一项前瞻性多中心研究。
DOI：
10.1097/wno.0000000000000167
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发表时间：
2015-03
期刊：
影响因子：
--
通讯作者：
Subramony SH
中科院分区：
文献类型：
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作者：
Moscovich M;Okun MS;Favilla C;Figueroa KP;Pulst SM;Perlman S;Wilmot G;Gomez C;Schmahmann J;Paulson H;Shakkottai V;Ying S;Zesiewicz T;Kuo SH;Mazzoni P;Bushara K;Xia G;Ashizawa T;Subramony SH
眼部运动异常反映了脊髓小脑共济失调 (SCAs) 的不同神经病理学，可能有助于临床区分不同的 SCA。我们分析了在一项针对 SCA 1、2、3 和 6 的大型多中心自然史研究中在基线访视时前瞻性检测到的各种眼球运动异常。这些数据是作为脊髓小脑性共济失调临床研究联盟 (NIH-CRC-SCA) 的一部分，前瞻性地从美国 12 个中心收集的 SCA 1、2、3 和 6 患者的数据。使用患者特征、共济失调评定量表、统一亨廷顿病评定量表功能检查和临床分期。记录眼球运动异常，包括眼球震颤、扫视和追踪障碍以及眼肌麻痹，并检查影响其发生的因素。共有 301 名患者参与了这项研究，其中包括 52 名 SCA 1 患者、64 名 SCA 2 患者、117 名 SCA 3 患者和 68 名 SCA 6 患者。虽然没有特定的眼部运动异常是任何 SCA 的特征，但不同疾病之间的发生率存在显着差异。 SCA 6 的特点是频繁出现眼球震颤和异常追视以及罕见的慢速眼跳和眼肌麻痹；SCA 2 的特点是频繁出现慢速眼跳和不频繁的眼球震颤和眼肌麻痹。 SCA 1 和 SCA 3 受试者的眼球运动异常分布更加均匀。来自大量 SCA 1、2、3 和 6 患者的前瞻性数据提供了统计验证，表明 SCA 表现出明显的眼动异常，这有助于识别基因型。许多异常与更严重的疾病严重程度相关。
Ocular motor abnormalities reflect the varied neuropathology of spinocerebellar ataxias (SCAs) and may serve to clinically distinguish the different SCAs. We analyzed the various eye movement abnormalities detected prospectively at the baseline visit during a large multicenter natural history study of SCAs 1, 2, 3, and 6. The data were prospectively collected from 12 centers in the United States in patients with SCAs 1, 2, 3, and 6, as part of the Clinical Research Consortium for Spinocerebellar Ataxias (NIH-CRC-SCA). Patient characteristics, ataxia rating scales, the Unified Huntington Disease Rating Scale functional examination, and clinical staging were used. Eye movement abnormalities including nystagmus, disorders of saccades and pursuit, and ophthalmoparesis were recorded, and factors influencing their occurrence were examined. A total of 301 patients participated in this study, including 52 patients with SCA 1, 64 with SCA 2, 117 with SCA 3, and 68 with SCA 6. Although no specific ocular motor abnormality was pathognomonic to any SCA, significant differences were noted in their occurrence among different disorders. SCA 6 was characterized by frequent occurrence of nystagmus and abnormal pursuit and rarity of slow saccades and ophthalmoparesis and SCA 2 by the frequent occurrence of slow saccades and infrequent nystagmus and dysmetric saccades. SCA 1 and SCA 3 subjects had a more even distribution of eye movement abnormalities. Prospective data from a large cohort of patients with SCAs 1, 2, 3, and 6 provide statistical validation that the SCAs exhibit distinct eye movement abnormalities that are useful in identifying the genotypes. Many of the abnormalities correlate with greater disease severity measures.