Barker Megan S, Shneider Neil A, Manoochehri Masood, Huey Edward D, Lindenberger Elizabeth C
Memory and Aging Program, Butler Hospital, 345 Blackstone Blvd, Providence, RI 02906, United States.
Department of Psychiatry and Human Behavior, Alpert Medical School of Brown University, Providence, RI, United States.
Psychiatry Res Case Rep. 2025 Jun;4(1). doi: 10.1016/j.psycr.2025.100247. Epub 2025 Jan 15.
Although amyotrophic lateral sclerosis (ALS) is defined as a neuromuscular disease, cognitive and/or behavioral symptoms are relatively common, and a portion of ALS patients will meet criteria for behavioral variant frontotemporal dementia (bvFTD). In this report, we describe the case of a man with ALS with bvFTD (ALS-FTD) presenting with excessive emotional reactivity, including severe anger, aggression, and obsessive thoughts. We contrast this case with the decreased emotional reactivity that is usually observed in patients with bvFTD without ALS. We discuss possible explanations including that: 1) the behavioral symptoms of bvFTD and ALS-FTD are the same, but the motor dysfunction influences the clinical manifestations of the behavioral symptoms in ALS-FTD; 2) the emotional and behavioral symptoms of bvFTD and ALS-FTD are the same, but ALS-FTD patients come to clinical attention earlier in the course of their FTD than bvFTD patients without ALS; and 3) the emotional and behavioral symptoms of bvFTD and ALS-FTD could differ.
尽管肌萎缩侧索硬化症（ALS）被定义为一种神经肌肉疾病，但认知和/或行为症状相对常见，一部分ALS患者会符合行为变异型额颞叶痴呆（bvFTD）的标准。在本报告中，我们描述了一名患有bvFTD的ALS患者（ALS-FTD）的病例，该患者表现出过度的情绪反应，包括严重的愤怒、攻击性和强迫观念。我们将此病例与通常在无ALS的bvFTD患者中观察到的情绪反应降低进行对比。我们讨论了可能的解释，包括：1）bvFTD和ALS-FTD的行为症状相同，但运动功能障碍影响了ALS-FTD中行为症状的临床表现；2）bvFTD和ALS-FTD的情绪和行为症状相同，但ALS-FTD患者在其FTD病程中比无ALS的bvFTD患者更早引起临床关注；3）bvFTD和ALS-FTD的情绪和行为症状可能不同。
