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.
Underlying pathology identified after 20 years of disease course in two cases of slowly progressive frontotemporal dementia syndromes / Hokelekli, Fatma Ozlem; Whitwell, Jennifer L; Machulda, Mary M; Jones, David T; Uitti, Ryan J; Pham, Nha Trang Thu; Giannini, Caterina; Baker, Matthew; Lowe, Val J; Dickson, Dennis W; Josephs, Keith A. - In: NEUROCASE. - ISSN 1355-4794. - STAMPA. - 27:2(2021), pp. 212-222. [10.1080/13554794.2021.1918723]
Underlying pathology identified after 20 years of disease course in two cases of slowly progressive frontotemporal dementia syndromes
Giannini, Caterina;
2021
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
