APHP GHU Nord Lariboisière Fernand-Widal, Cognitive Neurology Centre, Paris, France.
Université de Paris Inserm UMR S1144 Optimization in Neuropsychopharmacology, Paris, France.
J Alzheimers Dis. 2021;84(4):1439-1446. doi: 10.3233/JAD-215226.
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia have a strong clinical, genetic, and pathological connection but association of ALS with Alzheimer's disease (AD) is seldom reported. We report a series of 5 cases of AD associated with ALS. Our patients presented with cognitive deterioration with episodic memory impairment meeting criteria for AD. ALS occurred subsequently in all cases and its phenotype was not homogenous. Amyloid process was confirmed in four cases with cerebrospinal fluid biomarkers. One case underwent postmortem exam, demonstrating hallmarks lesions of both diseases. This series highlights that ALS-AD phenotype could be a specific underexplored entity.
肌萎缩侧索硬化症（ALS）和额颞叶痴呆具有很强的临床、遗传和病理学联系，但 ALS 与阿尔茨海默病（AD）的关联很少被报道。我们报告了一系列 5 例 AD 合并 ALS 的病例。我们的患者表现为认知功能恶化，伴有符合 AD 标准的情景记忆障碍。所有病例随后均发生 ALS，其表型并不单一。4 例患者的淀粉样蛋白过程得到了脑脊液生物标志物的证实。1 例进行了尸检，显示出两种疾病的特征性病变。本系列强调 ALS-AD 表型可能是一种特定的未被充分研究的实体。
