Massman P J, Sims J, Cooke N, Haverkamp L J, Appel V, Appel S H
Department of Neurology, Baylor College of Medicine, Houston, USA.
J Neurol Neurosurg Psychiatry. 1996 Nov;61(5):450-5. doi: 10.1136/jnnp.61.5.450.
To determine the prevalence and correlates of neuropsychological impairment in a large cohort (n = 146) of patients with typical, sporadic (non-familial) amyotrophic lateral sclerosis.
A battery of neuropsychological tests was administered to patients with amyotrophic lateral sclerosis who were attending a monthly outpatient clinic or who were in hospital undergoing diagnostic tests.
Comparing individual patient's scores with relevant normative data, 35.6% of the patients displayed evidence of clinically significant impairment, performing at or below the 5th percentile on at least two of the eight neuropsychological measures. Deficits were most common in the areas of problem solving, attention/mental control, continuous visual recognition memory, word generation, and verbal free recall. Impairment was most prevalent in patients with dysarthria (48.5%), but 27.4% of non-dysarthric patients were also impaired. Impaired patients had more severe or widespread symptoms of amyotrophic lateral sclerosis than non-impaired patients, and had fewer years of education.
Neither the conventional wisdom that cognition is intact in nearly all patients with amyotrophic lateral sclerosis, nor more recent suggestions that cognition is often at least mildly impaired seems to be correct. A minority of patients with amyotrophic lateral sclerosis displayed evidence of significant impairment. Dysarthria, low education, and greater severity of motor symptoms were risk factors for impairment.
确定一大群（n = 146）典型的散发性（非家族性）肌萎缩侧索硬化患者中神经心理学损害的患病率及其相关因素。
对每月门诊就诊或住院接受诊断检查的肌萎缩侧索硬化患者进行一系列神经心理学测试。
将个体患者的分数与相关的常模数据进行比较，35.6%的患者表现出具有临床意义的损害证据，在八项神经心理学测量中的至少两项上得分处于或低于第5百分位数。缺陷在解决问题、注意力/心理控制、连续视觉识别记忆、词语生成和言语自由回忆等方面最为常见。构音障碍患者中损害最为普遍（48.5%），但27.4%的非构音障碍患者也存在损害。与未受损患者相比，受损患者的肌萎缩侧索硬化症状更严重或更广泛，且受教育年限更少。
几乎所有肌萎缩侧索硬化患者认知功能均完好这一传统观点，以及近期认为认知功能通常至少有轻度损害的观点似乎都不正确。少数肌萎缩侧索硬化患者表现出明显损害的证据。构音障碍、低教育程度和更严重的运动症状是损害的危险因素。
