Chowdhury Abhishek, Mukherjee Adreesh, Sinharoy Uma, Pandit Alak, Biswas Atanu
Department of Neurology, Institute of Post Graduate Medical Education and Research (IPGME&R) and Bangur Institute of Neurosciences, Kolkata, West Bengal, India.
Ann Indian Acad Neurol. 2021 Sep-Oct;24(5):745-753. doi: 10.4103/aian.AIAN_51_21. Epub 2021 May 21.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of motor neurons. Spread of pathology to other brain areas leads to development of non-motor symptoms (NMSs). These usually remain undiagnosed because of overwhelming motor problem and are responsible for significant distress to the patient. Our objective was to explore the burden of various NMSs of patients with ALS, compare between limb-onset and bulbar-onset patients, and to correlate with severity and duration of disease.
Fifty patients with ALS diagnosed according to revised El Escorial Criteria and 50 healthy controls were included in this study. They were assessed with NMS Questionnaire, Beck's Depression Inventory, Center for Neurologic Study-Lability Scale, Drooling Frequency and Severity Scale, Epworth Sleepiness scale, Bengali Mental State Examination, and Frontal Assessment Battery and relevant statistical analyses were carried out.
The patients with ALS had significantly increased prevalence of almost all NMSs compared to controls. There was also significant increase in depression, suicidal ideation, pseudobulbar affect, and daytime sleepiness in patients with ALS. The bulbar onset subgroup had significantly increased daytime drooling, dysphagia, nausea and vomiting, whereas the limb onset subgroup reported increased frequency of leg swelling. Executive dysfunction was detected in 24% of patients with ALS and 9.8% had mild cognitive impairment. Weight loss, frequency of falling, insomnia, unpleasant nocturnal leg sensations, difficulty having sex, depression, and cognitive impairment increased significantly with an increase in severity of the disease.
NMSs were significantly more prevalent in patients with ALS. Some NMSs worsened with advancement of the disease.
肌萎缩侧索硬化症(ALS)是一种运动神经元的进行性神经退行性疾病。病理变化扩散到其他脑区会导致非运动症状(NMSs)的出现。由于严重的运动问题,这些症状通常未被诊断出来,给患者带来极大痛苦。我们的目的是探讨ALS患者各种NMSs的负担,比较肢体起病和延髓起病患者之间的情况,并与疾病的严重程度和病程相关联。
本研究纳入了50例根据修订的埃尔埃斯科里亚尔标准诊断的ALS患者和50名健康对照者。使用NMS问卷、贝克抑郁量表、神经学研究易激惹量表、流涎频率和严重程度量表、爱泼华嗜睡量表、孟加拉精神状态检查和额叶评估量表对他们进行评估,并进行相关统计分析。
与对照组相比,ALS患者几乎所有NMSs的患病率显著增加。ALS患者的抑郁、自杀观念、假性球麻痹情感和日间嗜睡也显著增加。延髓起病亚组的日间流涎、吞咽困难、恶心和呕吐显著增加,而肢体起病亚组报告腿部肿胀频率增加。24%的ALS患者检测到执行功能障碍,9.8%有轻度认知障碍。随着疾病严重程度的增加,体重减轻、跌倒频率、失眠、夜间腿部不适感觉、性功能障碍、抑郁和认知障碍显著增加。
NMSs在ALS患者中更为普遍。一些NMSs随着疾病进展而恶化。