Benedict Ralph H B, Bobholz Julie H
Department of Neurology, State University of New York (SUNY) at Buffalo, Jacobs Neurological Institute, Buffalo, New York, USA.
Semin Neurol. 2007 Feb;27(1):78-85. doi: 10.1055/s-2006-956758.
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system that commonly leads to inflammatory and atrophic brain pathology, often causing cognitive impairment. MS-associated cognitive impairment was first described over a century ago. However, with the advent of standardized neuropsychological testing and quantitative brain imaging, the frequency, quality, and correlates of cognitive impairment are better understood. Dementia is rare in MS, although it is known to occur in 10 to 25% of patients. Our data suggest a frequency of 22% among clinic attendees. In addition to the cognitive impairments evident in MS dementia, changes in personality and social behavior also occur. For example, some patients develop euphoria sclerotica and marked deficiency in social empathy, conditions that in combination with executive dysfunction cause considerable hardship for patients and caregivers. These neuropsychiatric manifestations of MS dementia are correlated with magnetic resonance imaging indicators of brain atrophy, including ventricle enlargement, neocortical volume, and normalized whole brain volume. Recent developments in pharmacological treatment for disease progression and management of cognitive symptoms hold promise for patients suffering from the degenerative aspects of MS.
多发性硬化症（MS）是一种中枢神经系统的脱髓鞘疾病，通常会导致炎症性和萎缩性脑病变，常引起认知障碍。与MS相关的认知障碍在一个多世纪前就首次被描述。然而，随着标准化神经心理学测试和定量脑成像技术的出现，人们对认知障碍的频率、性质及相关因素有了更深入的了解。痴呆在MS中较为罕见，尽管已知10%至25%的患者会出现痴呆症状。我们的数据显示，门诊患者中痴呆的发生率为22%。除了MS痴呆中明显的认知障碍外，人格和社会行为也会发生变化。例如，一些患者会出现欣快性硬化症和明显的社会共情缺陷，这些情况与执行功能障碍一起给患者和照顾者带来了极大的困难。MS痴呆的这些神经精神表现与脑萎缩的磁共振成像指标相关，包括脑室扩大、新皮质体积和全脑体积标准化。疾病进展的药物治疗和认知症状管理方面的最新进展为患有MS退行性病变的患者带来了希望。
