在两例进展缓慢的额颞叶痴呆综合征中，经过 20 年的疾病进程后确定了潜在的病理学。
Underlying pathology identified after 20 years of disease course in two cases of slowly progressive frontotemporal dementia syndromes.
机构信息
Departments of Neurology, Mayo Clinic Rochester, Minnesota, USA.
Department of Radiology, Mayo Clinic Rochester, Minnesota, USA.
出版信息
Neurocase. 2021 Apr;27(2):212-222. doi: 10.1080/13554794.2021.1918723. Epub 2021 Apr 27.
Abstract
We report two cases from the frontotemporal lobar degeneration (FTLD) spectrum with remarkably slow progression. The first case demonstrated insidious-onset behavioral symptoms and personality changes resembling behavioral variant of frontotemporal dementia, followed a benign course over 26 years, his brain autopsy revealed the diffuse form of argyrophilic grain disease. The second case presented with slowly progressive cognitive and motor deficits, reminiscent of the corticobasal syndrome, deteriorated slowly over 22 years, his brain autopsy revealed FTLD-TDP with C9ORF72 pathology. These two cases confirm the notion of slowly progressive frontotemporal lobar degeneration caused by an underlying FTLD pathology, rather than a phenocopy.
摘要
我们报告了两例来自额颞叶变性（FTLD）谱的病例，其进展非常缓慢。第一例表现为隐匿性发作的行为症状和人格改变，类似于额颞叶痴呆的行为变异型，经过 26 年的良性病程，其大脑尸检显示弥漫性颗粒状嗜银病。第二例表现为进行性认知和运动功能障碍，类似于皮质基底节综合征，经过 22 年的缓慢恶化，其大脑尸检显示 FTLD-TDP 伴有 C9ORF72 病理学。这两个病例证实了由潜在的 FTLD 病理学引起的缓慢进展性额颞叶变性的概念，而不是表型模拟。
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