Grant: CIHR Fellowship

Frontotemporal dementia (FTD) affects up to 37,000 Canadians. Many people develop FTD during their 40's and 50's, accompanied by a broad range of behavioral and personality changes as well as speech and language difficulties. Looking from outside, it is difficult to distinguish FTD from other types of dementias such as Alzheimer's disease; what's more, FTD might be mistakenly perceived as psychiatric diseases with detrimental delay in diagnosis. FTD is marked by progressive nerve cell death in the frontal and temporal regions of the brain, which influence personality, behavior, emotion and speech. Once the symptoms set in, it is impossible to reverse FTD. This means it is crucial to treat patients at risk of developing FTD as early as possible before their brains suffer irreversible damage. But how do we know if someone is at risk if one is not yet showing any symptoms? People with mutations in specific genes are at risk of developing FTD in the future. In these people, abnormal changes in brain structures can be found years before the full symptoms appear. If we can follow how the abnormal changes propagate during this early period, we may be able to predict who may develop the disease before the deficits set in. We will use magnetic resonance imaging (MRI brain scans) and other tools to understand the sites and kinds of brain injury and define their relationships to behavioral and cognitive abnormalities. This will be done in a unique group of subjects recruited at our clinic at UBC who are potential carriers of genetic mutations that cause FTD due to their family history. Our findings will help clinicians and patients to recognize the changes in brain structures that may occur before FTD symptoms develop. This information will be necessary to make informed decisions in determining the timing and type of therapies that can best suit the needs of the patients, with the ultimate goal of facilitating testing of new treatments for people at risk of developing FTD.