Grant: CIHR Fellowship

In 2016, neurodegenerative diseases were affecting the lives of approximately 564 000 Canadians. Amongst these diseases, frontotemporal dementia (FTD) is the most frequent in individuals under the age of 60 years old. The very early onset and the complex nature of this disease bring significant and different challenges both in its diagnosis and management. FTD can manifest as a spectrum of clinical syndromes, and two of them, behavioral frontotemporal dementia (bvFTD) and semantic variant primary progressive aphasia (svPPA), particularly pose a challenge in terms of differential diagnosis. bvFTD is characterized by prominent and progressive behavioral changes, more specifically interpersonal and emotional behavior as well as deficits in executive functioning. svPPA is characterized by a prominent and progressive deterioration of semantic memory and language. Despite these clear-cut definitions, many studies have demonstrated that there is substantial overlap between bvFTD and svPPA (both in terms of symptoms and of changes in the brain). However, the nature and the underlying mechanisms of this clinical overlap are still unclear, and several unresolved issues remain in the literature. Therefore, the aim of this project is to characterize and compare patients with different FTD variants on social cognition and semantic/language measures, and to identify the most sensitive and specific tests for a better differential diagnosis. We also aim to investigate network-based brain correlates of social cognition and semantic/language in FTD variants. This project is critical to improve the differential diagnosis in these diseases and could also help to better direct patients towards appropriate therapeutic and behavioural intervention, as well as adequate counselling of families and caregivers. With the potential development of protein-specific therapies for neurodegenerative diseases, a better understanding of the brain processes involved in these diseases is also essential.