Saxon Jennifer A, Thompson Jennifer C, Jones Matthew, Harris Jennifer M, Richardson Anna Mt, Langheinrich Tobias, Neary David, Mann David Ma, Snowden Julie S
Manchester Academic Health Sciences Centre, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK.
Faculty of Biology, Medicine and Health, Division of Neuroscience and Experimental Psychology, University of Manchester, Manchester, UK.
J Neurol Neurosurg Psychiatry. 2017 Aug;88(8):675-680. doi: 10.1136/jnnp-2017-315667. Epub 2017 Jun 8.
A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone.
A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms. Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment.
A number of behavioural and affective changes were reported more frequently in bvFTD than ALS-FTD: social disinhibition (p<0.001), inertia (p<0.001), loss of sympathy and empathy (p0.008), repetitive behaviours (p<0.001) and dietary changes (p<0.001). Warmth of affect demonstrated in the clinic setting was reported more often in ALS-FTD than bvFTD (p<0.001). Executive impairments occurred equally in both groups. Language impairments were more common in ALS-FTD than bvFTD: agrammatism (p<0.017) and impaired sentence comprehension (p<0.036). Psychotic features were relatively rare and did not distinguish the groups.
Our findings suggest differences between bvFTD and ALS-FTD. In particular, while changes in social behaviour are prominent in bvFTD alone, there may be a comparatively greater degree of language impairment in ALS-FTD. Prospective exploration of the pattern of differences between these groups will be essential. Identification of a distinct neuropsychological phenotype in ALS-FTD may have clinical implications for early diagnosis, disease management and care planning and theoretical implications for our understanding of the relationship between ALS and FTD.
一部分行为变异型额颞叶痴呆（bvFTD）患者会发展为肌萎缩侧索硬化症（ALS）。目前尚不清楚合并ALS的bvFTD（ALS-FTD）的行为和认知综合征是否与单纯的bvFTD无法区分。
对241例临床诊断为bvFTD（n = 185）或ALS-FTD（n = 56）的患者进行回顾性队列研究，检查其行为、认知和神经精神症状。根据临床访谈和详细神经心理学评估记录的信息，将特征评定为存在或不存在。
与ALS-FTD相比，bvFTD患者报告的一些行为和情感变化更为频繁：社交抑制（p<0.001）、惰性（p<0.001）、同情心和同理心丧失（p = 0.008）、重复行为（p<0.001）和饮食变化（p<0.001）。与bvFTD相比，ALS-FTD患者在临床环境中表现出的情感温暖更为常见（p<0.001）。两组执行功能障碍的发生率相同。语言障碍在ALS-FTD中比bvFTD更常见：语法缺失（p<0.017）和句子理解受损（p<0.036）。精神病性特征相对罕见，无法区分两组。
我们的研究结果表明bvFTD和ALS-FTD之间存在差异。特别是，虽然社交行为变化仅在bvFTD中突出，但ALS-FTD中可能存在相对更严重的语言障碍。对这些组之间差异模式的前瞻性探索至关重要。识别ALS-FTD中独特的神经心理学表型可能对早期诊断、疾病管理和护理计划具有临床意义，并对我们理解ALS和FTD之间的关系具有理论意义。
