Frontotemporal dementia

FTD – an overview

Frontotemporal dementia (FTD), like Alzheimer’s, is a progressive brain disease that causes progressive loss of cognitive, emotional, and social abilities. What makes it particularly challenging is that patients usually develop symptoms before the age of 65 and suffer more from progressive personality changes, behavioral issues, and/or language disorders than from memory problems.

FTD affects people in their most productive phase of life – in the middle of their careers and family lives. It typically strikes individuals between the ages of 50 and 60, though the age range is very broad (20-85 years). The average life expectancy is eight to ten years, and there is still no cure.

The first signs of the most common form of FTD, the so-called behavioral variant or bvFTD, are often confused with mental illnesses, a midlife crisis, or relationship conflicts. The affected individuals initially appear physically healthy, and their memory often remains intact for a long time. Early-phase characteristics include emotional blunting, impulsivity, and a decline in social empathy. Subtle changes in eating habits and compulsive, restless behavior are also typical early symptoms. The individuals themselves are usually unable to recognize these changes – a particularly insidious aspect of FTD. One caregiver describes it this way: “Suddenly, there was something unfamiliar between us, I could feel it, but for years, I couldn’t find anyone who truly understood me, who could explain what was happening.”

The symptoms are difficult to categorize, and many doctors have hardly heard of the condition. On average, it takes up to four years to make an accurate diagnosis, and misdiagnoses are common. During this time, tragic misunderstandings occur: relationships break down, people lose their jobs, and children experience the lack of empathy as a painful rejection.

People with behavioral FTD are often considered antisocial or driven by their impulses, while intact functions like orientation and memory are overlooked. The symptoms, which are challenging and distressing for the affected person's environment, can sometimes be alleviated with the use of pharmacotherapy or managed through behavioral and environmental interventions.

As the disease progresses, the question of the right care becomes ever more pressing: Who can raise children, work a job, and simultaneously care for a partner who is aggressive, uninhibited or completely apathetic and needs full support – day and night? Admission to care facilities is often refused due to the complex symptoms and the young age of the patients – suitable facilities are lacking.

Family members bear the financial and emotional burden of the necessary individual care, while children suffer from the insensitive and disturbing behavior of the sick parent and the extreme strain on the healthy parent. Due to the lack of social awareness of the disease, those affected are often stigmatized. Because of the immense emotional and financial burden they carry, their families are pushed to the margins of society.

Both men and women are equally affected by FTD. In at least twenty percent of affected individuals, a hereditary genetic cause can be identified. Genetic testing and counseling can provide clarity. Around two thirds of all FTD cases are considered sporadic, meaning that no other family members are affected. Specific risk factors that could contribute to the manifestation of frontotemporal dementia, such as smoking or high cholesterol levels in Alzheimer’s, have not yet been identified.

In addition to the four times more common behavioral variant of FTD, there are also so-called language variants: the semantic variant of primary progressive aphasia (ppA) beginning with word-finding difficulties, and the non-fluent variant of primary progressive aphasia (svPPA), which involves problems with grammar and pronunciation.