Disease has been highlighted because of actor Bruce Willis, who recently was diagnosed with it
Years ago, one of my patients was brought in by his wife. He couldn’t seem to get the words out when he talked. His speech was slow and hesitant. Because his job involved interacting with the public, his work life was in shambles. She was going crazy because he couldn’t tell her what was going on. He couldn’t understand what she told him. He lost interest in the activities they used to do together. He was only about 50 years old.
After some testing, his neurologist determined that he had primary progressive aphasia. This disorder is a subtype of frontotemporal dementia (FTD).
Frontotemporal dementia is a group of disorders that affects the frontal and temporal lobes of the brain, causing them to shrink. These parts of the brain involve language, personality and behavior.
FTD causes a small minority of cases of dementia. But sadly, these disorders often strike younger people, typically between the ages of 40 and 65. It’s tough for the patient and a nightmare for their families. Society tends to be less forgiving of a person who looks physically healthy but exhibits bizarre behaviors.
There’s no known cure. Medications prescribed for Alzheimer’s disease help symptoms in some patients.
We don’t know what causes FTD. There is probably a genetic component. There are some families with mutations that to forms of early onset dementia.
Symptoms of frontotemporal dementia cluster into three groups: behavioral, language and motor.
The most common signs of FTD are abnormal behaviors and personality changes, such as:
• Inappropriate social behavior. A once polite individual might become rude and callous.
• Loss of empathy and insensitivity to the feelings of others. This could take the form of blurting out their first thoughts without a filter.
• Poor judgment. In the case of my patient, he lost the ability to drive safely as he couldn’t manage intersections, traffic signals, and other cars.
• Loss of inhibition. A once normally functioning person might start with inappropriate sexual remarks, advances, or undressing themselves in public.
• Apathy or loss of interest. This can seem like depression.
• Repetitive compulsive behaviors – tapping, clapping, lip smacking, etc.
• Decreased personal hygiene.
• Altered eating habits. Overeating, preferring sweets and carbohydrates, or eating non-food items.
• Compulsively putting things into the mouth.
Some variants of FTD chiefly impair speech and language, with symptoms of:
• Trouble using and understanding language. This may manifest as trouble finding the correct word when speaking and difficulty in naming objects.
• Replacing the specific name of an object with a general term like “it.”
• Forgetting word meanings.
• Hesitant speech.
• Mistakes in sentence construction.
Motor symptoms are less common and consist of movement problems that can resemble Parkinson’s disease or amyotrophic lateral sclerosis (ALS):
• Tremors.
• Rigidity.
• Muscle spasms or twitches.
• Trouble swallowing.
• Muscle weakness
• Falls or walking problems.
• Inappropriate laughing or crying. (Officially termed pseudobulbar affect).
Because FTD and other early onset dementias are rare, the diagnosis can take months or years to figure out.
Here are some online resources for affected patients and their families.
• The multisite research consortium on longitudinal frontotemporal lobar degeneration can be found at allftd.org.
• The Mesulam Center for Cognitive Neurology and Alzheimer’s disease at Northwestern University provides information on language dementias (www.brain.northwestern.edu/dementia/ppa/research.html)
And for caregivers Duet Partners in health and aging has a virtual program for family and caregivers (DuetAZ.org).
Eva Briggs is a retired medical doctor who practiced in Central New York for several decades. She lives in Marcellus.