What is Frontotemporal Dementia?

The family of actor Bruce Willis recently announced he has been diagnosed with primary progressive aphasia (PPA), a type of frontotemporal dementia (FTD). FTD refers to a group of disorders that are caused by progressive nerve cell loss in your brain’s frontal and temporal lobes — the areas behind your forehead and your ears.

While many people are familiar with more common types of dementia, such as Alzheimer’s disease, FTD is widely unknown among the general population. But it’s important to be familiar with the signs and symptoms of FTD, especially if you have a family history of dementia, so you know what to look out for.

Differences Between FTD and Alzheimer’s Disease 

FTD differs from Alzheimer’s disease in more ways than one. “Frontotemporal dementia is diverse in its presentation and symptoms can vary greatly from person to person,” says Katherine Amodeo, MD, a neurologist at WMCHealth’s MidHudson Regional Hospital, who specializes in movement disorders and dementia. “There are a number of FTD variants which can present with varying early features, whereas individuals with Alzheimer’s disease typically present with features of memory loss first.”  

Memory and word-finding impairment are common early features of Alzheimer’s disease, while early signs of FTD may be changes in personality, mood or loss of fluency in language. Early hallucinations in setting of cognitive impairment are most likely signs of an alternative diagnosis, dementia with Lewy bodies.

Frontotemporal dementia disorders also tend to appear at a younger age than Alzheimer’s disease. FTD commonly presents in your 50s, on average, but Alzheimer’s disease is more common in people older than age 70.

Another significant difference between FTD and Alzheimer’s disease is family history. “While having a family history of Alzheimer’s disease may increase your risk of developing AD depending on age of onset among family members and degree of relatedness, there’s a more predominant genetic relationship when it comes to FTD,” says Dr. Amodeo.

Symptoms and Types of FTD

The most common early symptom of FTD is a change in mood or personality, caused by nerve cell loss mostly in parts of the brain that control judgment, empathy and foresight. This type of FTD is known as behavior variant frontotemporal dementia (bvFTD).

“Sometimes bvFTD is mistakenly misdiagnosed as a psychiatric disorder,” says Dr. Amodeo. “It may appear a person with this condition has suddenly become apathetic or disinhibited. Generally, concern for a neurodegenerative disease arises when other symptoms surface, like trouble with language, multitasking and planning or memory.”

While bvFTD is most common, other variants of FTD may accompany it or can appear on their own. Primary progressive aphasia (PPA), the variant with which Willis was reportedly diagnosed, affects language skills, speaking, writing and comprehension. Other variants include disruptions of motor movements, like muscle weakness as observed in those with overlapping amyotrophic lateral sclerosis (ALS) pathology, uncoordinated or "alien limb phenomena” as observed in those with overlapping corticobasal syndrome, or difficulty with walking and postural stability as observed in those with overlapping progressive supranuclear palsy (PSP).

Diagnosing and Treating FTD

An FTD diagnosis is usually made by a specialist based on history and exam. Your provider may also conduct imaging or labs to rule out other conditions.

“Unfortunately, FTD is a neurodegenerative disease, which means it gets progressively worse over time,” says Dr. Amodeo. But there are things that you can do to slow the rate of progression, including getting plenty of exercise and eating a low-inflammatory diet like the Mediterranean diet. While a diagnosis of dementia can be difficult, Dr. Amodeo recommends a focus on living well. “You can still live your best life with this disease,” she says.

If you’re concerned about FTD or begin to recognize symptoms in your loved ones, make an appointment with WMCHealth Physicians and Advanced Neurology Associates.