The symptoms of FTD result from the shrinking of the outer layers of your brain in the areas just above your eyes (the frontal lobes) and/or close to your ears (the temporal lobes). As the healthy neurons slowly degenerate, they often accumulate abnormally folded proteins. The names for these protein clumps depends on the chemical makeup of the clump. One type of inclusion is called the Pick body, named for the neurologist who first described this disease, Arnold Pick. In addition to the neuronal inclusions, scar tissue (gliosis) develops in the damaged region. The areas damaged in this disease lead to changes in behavior, language and/or motor skills. The posterior part of the brain is spared in FTD, so many day-to-day functions are normal.
Basic Biology of FTD
Basic Anatomy and Biology
Personality and Creativity
Most of the people we see at our clinic experience personality changes with dementia. One interesting change is that several people have become prolific artists after being diagnosed with FTD, particularly with the type known as semantic dementia which affects the ability to understand words and recognize people. Seeing this change raises questions about how changes in the brain lead to changes in personality.
Speech and Language
Language functions includes speaking, understanding speech, repeating things we have heard, naming objects, reading and writing. The majority of patients with the language variant of FTD experience problems expressing language, marked by problems using the correct word, including naming objects or expressing oneself. Less commonly, patients present with severe problems naming and understanding word meaning. The precise symptoms affected in these variations has helped us gain a better understanding of how the brain organizes language.
The term “Executive Functions” refers to higher-level cognitive abilities that enable you to successfully engage in independent goal-directed behavior. These capacities are most commonly linked to the frontal cortex and guide complex behavior over time through planning, decision-making and self-monitoring of judgments and impulses. The term is a business metaphor, where the executive monitors all of the different departments so the company can move forward efficiently and effectively. Who we are, how we organize our lives, and how we plan and then execute those plans is largely guided by the frontal regions of our brain.
Approximately 40% of people with FTD have a family history of FTD or another related dementia, while 5-10% of patients have a family history that shows an autosomal dominant inheritance pattern. This means that a child of someone with a disease has a 50% chance of developing it themselves. Five genetic mutations have been linked to FTD so far. The symptoms and pathology vary depending on the specific mutation.
Medical Terms in FTD
Browse this list of terms and definitions for words often used when discussing FTD.