FTD. And Lewy Body Dementia

Brain Science:

FTD in this case has nothing to do with flowers being delivered. Frontotemporal dementia (FTD) or frontotemporal degenerations refers to a group of disorders caused by progressive nerve cell loss in the brain's frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears.The nerve cell damage caused by frontotemporal dementia leads to loss of function in these brain regions, which causes deterioration in behavior, personality and/or difficulty with producing or comprehending language.There are a number of different diseases that cause frontotemporal degenerations. The two most prominent are 1) a group of brain disorders involving the protein tau and 2) a group of brain disorders involving the protein called TDP43. For reasons that are not yet known, these two groups have a preference for the frontal and temporal lobes that cause dementia. There are three disorders that are variants. Frontotemporal dementia used to be called Pick's disease after Arnold Pick, M.D., a physician who in 1892 first described a patient with distinct symptoms affecting language. Some doctors still use the term "Pick's disease." Variants:Types 1.Behavior variant frontotemporal dementia (bvFTD) is characterized by prominent changes in personality and behavior that often occur in people in their 50s and 60s, but can develop as early as their 20s or as late as their 80s. In behavior variant frontotemporal dementia, the nerve cell loss is most prominent in areas that control conduct, judgment, empathy and foresight, among other abilities. 2.Primary progressive aphasia (PPA) is the second major form of frontotemporal degeneration that affects language skills, speaking, writing and comprehension. PPA normally comes on in midlife, before age 65, but can occur in late life also. The two most distinctive forms of PPA have somewhat different symptoms.

• A semantic variant of PPA, individuals lose the ability to understand or formulate words in a spoken sentence.

• In nonfluent/agrammatic variant of PPA, a person’s speaking is very hesitant, labored or ungrammatical.

Disturbances of motor (movement or muscle) function include three disorders that are a part of the frontotemporal degeneration spectrum that produce changes in muscle or motor functions with or without behavior (bvFTD) or language (PPA) problems:

• Amyotrophic lateral sclerosis (ALS), which causes muscle weakness or wasting. ALS is a motor neuron disease also known as Lou Gehrig’s disease.

• Corticobasal syndrome, which causes arms and legs to become uncoordinated or stiff.

• Progressive supranuclear palsy (PSP), which causes muscle stiffness, difficulty walking and changes in posture. It also affects eye movements.

Brain Facts:

• Both behavior variant frontotemporal dementia and PPA are far less common than Alzheimer’s disease in those over age 65 years.

• However, in the 45 to 65 age range, behavior variant frontotemporal dementia and PPA are nearly as common as younger-onset Alzheimer’s. Only rough estimates are available, but there may be 50,000 to 60,000 people with behavior variant frontotemporal dementia and PPA in the United States, the majority of whom are between 45 and 65 years of age.

• Age at diagnosis may be an important clue. Most people with FTD are diagnosed in their 40s and early 60s. Alzheimer's, on the other hand, grows more common with increasing age.

• Memory loss tends to be a more prominent symptom in early Alzheimer's than in early FTD, although advanced FTD often causes memory loss in addition to its more characteristic effects on behavior and language.

• Behavior changes are often the first noticeable symptoms in bvFTD, the most common form of FTD. Behavior changes are also common as Alzheimer's progresses, but they tend to occur later in the disease.

• Problems with spatial orientation — for example, getting lost in familiar places — are more common in Alzheimer's than in FTD.

• Problems with speech: Although people with Alzheimer's may have trouble thinking of the right word or remembering names, they tend to have less difficulty making sense when they speak, understanding the speech of others, or reading than those with FTD.

• Hallucinations and delusions are relatively common as Alzheimer's progresses, but relatively uncommon in FTD.

• Treatment and outcomes There are no specific treatments for any of the frontotemporal subtypes. There are medications that can reduce agitation, irritability and/or depression. These treatments should be used to help improve quality of life.

• Frontotemporal dementia inevitably gets worse over time and the speed of decline differs from person to person. For many years, individuals with frontotemporal dementia show muscle weakness and coordination problems, leaving them needing a wheelchair — or bedbound. These muscle issues can cause problems swallowing, chewing, moving and controlling bladder and/or bowels. Eventually people with frontotemporal degenerations pass away.

SoWhat: This is a horrible disease with no positive outcome. While in some cases the new drugs coming out might help.But it will be a while. Frontotemporal is not as common as Alzheimer's but just as serious. My wife, Sue, received the following from a friend of hers here in Florida.

There are days when we think we have had a bad day. Those are the days that we might want to stop and think of others who are going through this disease and particularly the care givers. Our lives aren't near as hard as these people and their caregivers. It might be a brighter day if we let the care givers know we care about them and the patient. Maybe giving the care giver a symbolic shot in the arm might give our bad day a shot in the arm.