Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
J Neurol Sci. 2012 Mar 15;314(1-2):97-101. doi: 10.1016/j.jns.2011.10.016. Epub 2011 Dec 21.
Ocular motility abnormalities may be a marker of neuro-degeneration beyond motor neurons in amyotrophic lateral sclerosis (ALS). We formally compared clinical neuro-ophthalmic abnormalities in ALS patients and a control population.
Patients attending a multidisciplinary ALS clinic (n=63, age 60.8+/-16.4 years) and their caregivers serving as controls (n=37, ages 55.0+/-12.7 years) participated in this cross-sectional study. Visual acuity was assessed. Video recordings of a standardized ocular motility exam including gaze fixation, voluntary saccades, reflex saccades, smooth pursuit, eyelid opening and Bell's phenomenon were rated by two senior neuro-ophthalmologists who were masked to subject group.
Visual acuity was lower in ALS patients versus control subjects (OR 0.81 (0.71-0.93), p=0.003, logistic regression). Inter- and intra-rater reliability for ocular motility examination ratings were good (Cohen's Kappa>0.6). Findings observed only in ALS subjects included gaze impersistence (14%, p=0.01), moderately or severely restricted voluntary upgaze (13%, p=0.01), and moderate or severe eyelid opening apraxia (27%, p=0.0002). Accounting for age, moderately or severely saccadic horizontal smooth pursuits distinguished ALS from control subjects (OR 3.6 (1.2-10.9), p=0.02, logistic regression).
Clinical findings of decreased visual acuity, gaze impersistence, voluntary upgaze restriction, eyelid opening apraxia, and saccadic horizontal smooth pursuits are more frequent in patients with ALS than in similar-aged controls. These findings are potential clinical markers of neurodegeneration beyond upper and lower motor neuron disease in ALS. Further study is warranted regarding their application to disease categorization and outcomes assessment.
眼球运动异常可能是肌萎缩侧索硬化症（ALS）中除运动神经元外神经退行性变的标志物。我们对 ALS 患者和对照组的临床神经眼科异常进行了正式比较。
本横断面研究纳入了在多学科 ALS 诊所就诊的患者（n=63，年龄 60.8+/-16.4 岁）及其护理人员作为对照组（n=37，年龄 55.0+/-12.7 岁）。评估了视力。通过视频记录标准化的眼球运动检查，包括注视固定、随意扫视、反射扫视、平滑追踪、眼睑张开和贝尔现象，由两位资深神经眼科医生进行评估，他们对受试者分组情况进行了盲法。
与对照组相比，ALS 患者的视力较低（OR 0.81（0.71-0.93），p=0.003，logistic 回归）。眼球运动检查评分的组内和组间可靠性良好（Cohen's Kappa>0.6）。仅在 ALS 患者中观察到的发现包括注视不持久（14%，p=0.01）、中度或重度自主上视受限（13%，p=0.01）和中度或重度眼睑张开失用症（27%，p=0.0002）。考虑到年龄因素，中度或重度水平扫视平滑追踪可将 ALS 与对照组区分开来（OR 3.6（1.2-10.9），p=0.02，logistic 回归）。
与年龄相似的对照组相比，ALS 患者的视力下降、注视不持久、自主上视受限、眼睑张开失用症和水平扫视平滑追踪等临床发现更为频繁。这些发现可能是 ALS 中除上下运动神经元疾病外神经退行性变的潜在临床标志物。进一步研究这些发现应用于疾病分类和预后评估是必要的。
