1Objective: To compare demographic characteristics of patients in the 3 FTLD subgroups.
2Results: The frontotemporal dementia subgroup represented approximately half of all FTLD diagnoses.
3The publication of consensus criteria for FTLD, however, prompted systematic studies.
4FTLD is a more common form of dementia than previously recognized.
5Recent findings have shown that this domain is hypomethylated in patients with FTLD-FUS.
6In most ALS and FTLD cases, the predominant pathological species are RNA-binding proteins.
7Extrapyramidal motor symptoms were more likely to be present in FTLD.
8However, no increases were observed in any of the five factor scores in FTLD.
9Autopsy in three family members showed a consistent and unique subtype of FTLD-TDP pathology.
10Hence, ALS and FTLD converge in pathogenic pathways disrupting the regulation of RNA processing.
11The sex and age-at-onset differences suggest that there may be biological differences among FTLD subgroups.
12There was no significant FTLD or motor neuron disease.
13This suggests additional variants and genes that remain to be identified as risk factors for FTLD.
14These findings highlight this process as a novel and promising therapeutic target for ALS and FTLD.
15Moreover, 19 proteins were selectively decreased in FTLD-U.
16The present review aims to underscore the necessity of further elucidation of PGRN biology in FTLD-related neurodegeneration.