Frontotemporal lobar degeneration (FTLD) covers a whole spectrum of neurodegenerative disorders which principally affect the frontal and temporal lobes of the brain.

Corticobasal syndrome (CBS) belongs to the category of FTD disorders that primarily affect movement. Some symptoms of both CBS and progressive supranuclear palsy, another FTD disorder associated with a decline in motor function, resemble those often seen in people with Parkinson’s disease.

2018年10月18日 · 额颞叶变性（frontotemporal lobar degeneration，FTLD）的临床表现为额颞叶痴呆（frontotemporal dementia，FTD），是一组以进行性精神行为异常、执行功能障碍和语言损害为主要特征的痴呆症候群，其病理特征为选择性的额叶和（或）颞叶进行性萎缩。 FTLD的病因尚未明确，其在临床、病理和遗传方面具有异质性。 目前关于FTLD的全球流行病学研究并不多，我国尚无FTLD的流行病学数据。 西方国家的数据显示，FTLD发病年龄为40-80岁，以45 …

Fig. 1 Change of disease concept of corticobasal degeneration (CBD). Modified from reference 15. The term corticobasal syndrome (CBS) is used for clinical diagnosis of CBD. Patients with CBS include patients with pathologically-confirmed CBD and those with non- CBD (#). The latter patients are called CBD-mimics.

2009年10月30日 · Sporadic corticobasal syndrome (CBS) has been associated with diverse pathological substrates, but frontotemporal lobar degeneration with TDP-43 immunoreactive inclusions (FTLD-TDP) has only been linked to CBS among progranulin mutation carriers.

2016年5月3日 · The prevalence of FTD, PSP, and CBS increases beyond 65 years, with frequent genetic causes. The time from onset to diagnosis and from diagnosis to death varies widely among syndromes, emphasizing the challenge and importance of accurate and timely diagnosis.

Frontotemporal Lobar Degeneration (FTLD) refers to a clinically, pathologically and genetically heterogeneous group of disorders that affect principally the frontal and temporal lobes of the brain. After Alzheimer's disease, it is the second most common cause of dementia in …

2023年8月10日 · Frontotemporal lobar degeneration (FTLD) is one of the most common causes of early-onset dementia and presents with early social–emotional–behavioural and/or...

2007年8月1日 · Frontotemporal lobar degeneration (FTLD) is a syndromic diagnosis that encompasses at least three different variants. Imaging modalities are clinically useful in FTLD, although pathology remains the gold standard for definitive diagnosis. To date, four different genes have been identified that account for FTLD. Previous Next

FTD/CBS is rarely associated with MSA pathology. The cell seeding characteristics and cryo-EM findings support the classification of FTLD-synuclein as a subtype of MSA, differentiating it from genetic synucleinopathies, such as those with SNCA variants G51D and A53E, which have neuropathological fea …