U.O.C. Neurologia, Fondazione IRCCS Ospedale Maggiore Policlinico, Università degli Studi di Milano, Milan, Italy.
U.O.C., Neurologia & Stroke Unit, A.S.S.T. Lecco, Merate, LC, Italy.
Neurol Sci. 2022 Dec;43(12):6741-6760. doi: 10.1007/s10072-022-06340-0. Epub 2022 Sep 2.
Among clinicians and researchers, it is common knowledge that, in ALS, cognitive and behavioral involvement within the spectrum of frontotemporal degenerations (FTDs) begun to be regarded as a fact in the late 1990s of the twentieth century. By contrast, a considerable body of evidence on cognitive/behavioral changes in ALS can be traced in the literature dating from the late nineteenth century.
Worldwide reports on cognitive/behavioral involvement in ALS dating from 1886 to 1981 were retrieved thanks to Biblioteca di Area Medica "Adolfo Ferrate," Sistema Bibliotecario di Ateneo, University of Pavia, Pavia, Italy and qualitatively synthetized.
One-hundred and seventy-four cases of ALS with co-occurring FTD-like cognitive/behavioral changes, described in Europe, America, and Asia, were detected. Neuropsychological phenotypes were consistent with the revised Strong et al.'s consensus criteria. Clinical observations were not infrequently supported by histopathological, post-mortem verifications of extra-motor, cortical/sub-cortical alterations, as well as by in vivo instrumental exams-i.e., assessments of brain morphology/physiology and psychometric testing. In this regard, as earlier as 1907, the notion of motor and cognitive/behavioral features in ALS yielding from the same underlying pathology was acknowledged. Hereditary occurrences of ALS with cognitive/behavioral dysfunctions were reported, as well as familial associations with ALS-unrelated brain disorders. Neuropsychological symptoms often occurred before motor ones. Bulbar involvement was at times acknowledged as a risk factor for cognitive/behavioral changes in ALS.
Historical observations herewith delivered can be regarded as the antecedents of current knowledge on cognitive/behavioral impairment in the ALS-FTD spectrum.
在临床医生和研究人员中，有一种共识，即在 ALS 中，认知和行为方面的问题涉及额颞叶退行性变（FTD）的范围，这一认识始于 20 世纪 90 年代末。相比之下，从 19 世纪末的文献中可以找到大量关于 ALS 中认知/行为变化的证据。
通过意大利帕维亚大学的“Adolfo Ferrate”医学图书馆、大学图书馆系统，检索了从 1886 年到 1981 年有关 ALS 认知/行为改变的全球报告，并进行了定性综合。
在欧洲、美洲和亚洲发现了 174 例伴有 FTD 样认知/行为改变的 ALS 病例。神经心理学表型与修订后的 Strong 等人的共识标准一致。临床观察经常得到组织病理学、运动外皮质/皮质下改变的死后验证以及体内仪器检查的支持，即大脑形态/生理学的评估和心理测试。在这方面，早在 1907 年，就已经认识到 ALS 中的运动和认知/行为特征源自相同的潜在病理。报道了伴有认知/行为功能障碍的遗传性 ALS 病例，以及与 ALS 无关的脑疾病的家族相关性。神经心理学症状通常在运动症状之前出现。球部受累有时被认为是 ALS 认知/行为改变的一个危险因素。
本文提供的历史观察结果可以被视为当前对 ALS-FTD 谱中认知/行为障碍的认识的前身。
