Frontotemporal dementia (FTD) is a form of dementia that typically affects individuals in their 50s and 60s. It is therefore, commonly referred to as young onset dementia. Clinically, FTD can present in two ways; some patients present with behavioral impairment and are referred to as behavioral variant FTD (bvFTD). The other main presentation involves language decline and is called primary progressive aphasia (PPA). Both conditions start insidiously and accurate diagnosis can be a challenge, especially at early stages of the disease. In bvFTD, patients can present with apathy, lack of empathy, increased appetite, preference for sweet tooth, new onset of obsession, socially inappropriate behavior, and impaired ability in multitasking. In PPA on the other hand, language impairment is the main feature and can involve impairment of expression (finding words), comprehension (understanding words), or both.
Abnormal aggregation of two proteins that are naturally present in all nerve cells of the brain is the root cause of FTD. Accumulation of these abnormal proteins, tau and TDP-43, cause shrinkage of frontal or temporal lobes of the brain. The symptoms that patients suffer depend on the site of brain shrinkage (atrophy). Involvement of temporal lobe (sides of the brain) typically leads to PPA whereas atrophy of frontal lobe is associated with bvFTD.
FTD is less common than Alzheimer’s disease, but certain features of the disease make its consequences particularly challenging. Most patients are still in the active years of their lives when they start suffering the symptoms of FTD and many have young families that still depend on their support. Moreover, in many instances, the cognitive impairment is accompanied by other devastating consequences of the disease including inability to move, swallow, or losing dexterity and use of limbs leading to significant dependence and shorter life span. Unfortunately, we have no effective treatments for this condition at present. Despite this, supportive treatments such as speech therapy, physical therapy, and occupational therapy can be very helpful in select group of patients. Much remains to be done for patients suffering from FTD and research at UCI and other academic centers around the globe focus on better understanding the disease and developing effective treatments for its various forms and consequences.