FTL Dementia update from Dementia Weekly

Frontotemporal dementias are a set of devastating, progressive diseases. They attack the parts of the brain responsible for judgment, empathy, and social behavior. People with FTD often develop personality changes, make ruinous financial decisions, become sexually inappropriate, or engage in compulsive behavior. Some patients with AD exhibit some of those behaviors, too; however, with FTDs they are front and center. Some forms of FTD rob people of normal speech, while others impair movement, similar to Parkinson’s disease and amyotrophic lateral sclerosis. FTD typically strikes at younger ages than does AD, often in a person’s late forties or fifties. It involves less memory loss than AD, but much worse behavior problems. Patients, in early stages of FTD, often seem indifferent to what would be a mortifying situation for a healthy person.

The disease is particularly devastating to families, said Susan Dickinson, who heads the Association for Frontotemporal Dementias, because it often goes unrecognized or misdiagnosed. Families, therefore, have no recourse if a spouse with FTD blows all his or her money on a sports car, or makes advances to a neighbor, since the spouse is not recognized as ill. Friends may withdraw because of the rude or disturbing behavior of the person with FTD. “Living with these diseases is incredibly isolating,” Dickinson said. “These families are losing their loved ones, and nobody will listen. We have a lot of people who get divorced.” Earlier diagnosis of the disease would help families to understand and cope with what is happening, Dickinson said. “As soon as there is a medical diagnosis, a lot of people get right back together to care for their spouse.”

FTDs are fairly rare. Scientists estimate as many as 20,000 Americans suffer from them, said David Knopman of the Mayo Clinic in Rochester, Minnesota, reviewing the findings of several recent papers. This is similar to the number of Americans with amyotrophic lateral sclerosis, but about 200-fold less than people with Alzheimer’s disease. FTD most often hits between 45 and 64 years old, and people typically survive six to nine years after diagnosis, Knopman said, similar to Alzheimer’s.

Frontotemporal dementia is an umbrella term that encompasses a large number of clinical syndromes and distinct pathologies. This includes a confusing lexicon of terms such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), primary progressive aphasia (PPA), behavioral variant FTD (bvFTD), and Pick’s disease. The distinctions among these syndromes are often hazy, and diagnosis can only be reliably made after death by examining brain tissue changes. Until quite recently, the fundamental biology at the root of many of these syndromes was a mystery.