Rakowicz W P, Hodges J R
Department of Neurology, Norfolk and Norwich Health Care NHS Trust, UK.
J Neurol Neurosurg Psychiatry. 1998 Dec;65(6):881-9. doi: 10.1136/jnnp.65.6.881.
To determine the prevalence and nature of global cognitive dysfunction and language deficits in an unselected population based cohort of patients with motor neuron disease (MND).
A battery of neuropsychological and language tests was administered to patients presenting consecutively over a 3 year period to a regional neurology service with a new diagnosis of sporadic motor neuron disease.
The 18 patients could be divided on the basis of their performance into three groups: Three patients were demented and had impaired language function (group 1); two non-demented patients had an aphasic syndrome characterised by word finding difficulties and anomia (group 2). Major cognitive deficits were therefore found in five of the 18 patients (28%). The remaining 13 performed normally on the test battery apart from decreased verbal fluency (group 3).
The prevalence of cognitive impairment in MND in this population based study of an unselected cohort was higher than has been previously reported. Language deficits, especially anomia, may be relatively frequent in the MND population. Aphasia in MND may be masked by dysarthria and missed if not specifically examined.
确定运动神经元病（MND）患者未经选择的基于人群队列中全球认知功能障碍和语言缺陷的患病率及性质。
对在3年期间连续就诊于某地区神经科服务机构且新诊断为散发性运动神经元病的患者进行了一系列神经心理学和语言测试。
18名患者可根据其表现分为三组：3名患者存在痴呆且语言功能受损（第1组）；2名非痴呆患者患有以找词困难和命名障碍为特征的失语综合征（第2组）。因此，18名患者中有5名（28%）存在主要认知缺陷。其余13名患者除言语流畅性下降外，在测试组合中表现正常（第3组）。
在这项对未经选择队列进行的基于人群的MND研究中，认知障碍的患病率高于先前报道。语言缺陷，尤其是命名障碍，在MND人群中可能相对常见。MND中的失语可能被构音障碍掩盖，如果未进行专门检查则可能被漏诊。
