Series: What's Under the Dementia Umbrella?

Part 4.1: Pick’s Disease Overview and Breakdown

In this series we are taking an in-depth look at the various types of dementia and how to improve treatment in a long-term care setting. There are several lesser known dementias affecting our seniors. As part of their care team we need to educate ourselves on how to better understand and meet the needs of those living with these dementias.

We have a large amount of important information to share with you concerning Pick’s Disease, or Frontotemporal Dementia, so we’ve opted to break it down into three shorter posts. Today we’ll take an overview of what FTD is and how it affects the brain.

Pick’s Disease, aka Frontotemporal Dementia (FTD)

You may have heard FTD referred to as frontotemporal disorders, frontotemporal degenerations, frontal lobe disorders or Pick’s Disease. Arnold Pick, in 1892, was the first physician to identify symptoms affecting language in a patient whose brain, after death, was found to have shrunk.

The name and classification of this dementia refers to a progressive loss of nerve cells in the brain’s frontal and temporal lobes. Let’s take a look at what these parts of the brain do.

Front

The frontal lobes are involved in motor function, problem solving, spontaneity, memory, language, initiation, judgment, impulse control and social and sexual behavior. They also help people to understand another person’s perspective.

Temporal (Side)

The two temporal lobes have very different functions and it’s important to remember that dementia will rarely attack the brain symmetrically. The left lobe controls vocabulary, speech production and comprehension, helps interpret visual data and processes speech. When a resident has damage to their left temporal lobe, they experience difficulty understanding. This is often initially attributed to hearing and visual problems while the real issue is comprehension.

The right temporal lobe handles the recognition and interpretation of sound. It also processes nonverbal memories, such as pictures, visual scenes, routes or directions, familiar faces and music. Usually the right side is less affected by dementia than the left. The person can still respond well to rhythm, pitch and tone and perform automatic social chitchat such as, “Hi. How are you?”, “I’m fine and you?” and use words that are strongly tied to emotion. Unfortunately, these words tend to be ugly—curses and ethnic or religious epithets.

The Alzheimer’s Association explains frontotemporal dementia this way:

“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.”

FTD is divided into three subcategories of disorders:

1. Behavior variant frontotemporal dementia (bvFTD)

In bvFTD, nerve cell loss is most highly concentrated in the areas of the brain that control conduct, judgment, empathy and foresight, leading to prominent changes in the patient’s personality and relationships. The patient can act strangely around others and cause embarrassment without knowing it or without caring about how they might make other people feel.

While this form of FTD can occur in patients young and old, the majority of are in their fifties and sixties.

2. Primary progressive aphasia (PPA)

PPA breaks down into three further subtypes, all of which affect language skill, speaking, writing and comprehension.

• The semantic variant causes people to lose their ability to understand or form sentences.

• The nonfluent/agrammatic variant causes hesitant, labored or ungrammatical speech.

• Logopenic PPA doesn’t cause grammatical or understanding issues in conversation, but patients have difficulty finding the right words.

PPA can occur later in life, but generally onset occurs during midlife, prior to age 65.

3. Disturbances of motor (movement or muscle) function

This form of FTD is also broken down further into three disorders that involve changes in muscle or motor functions but lack the behavior and language problems associated with bvFTD and PPA.

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

• Corticobasal syndrome causes stiffness or a lack of coordination in the arms and legs.

• Progressive supranuclear palsy (PSP) affects eye movement and posture. It also leads to difficulty walking and stiffness in the muscles.

According to the Alzheimer’s Association, “both bvFTD and PPA are far less common than Alzheimer’s disease in those over age 65 years. However, in the 45 to 65 age range, bvFTD 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 bvFTD and PPA in the United States, the majority of whom are between 45 and 65 years of age.”

In our next post, we’ll look at the symptoms associated with FTD. Learn more online: Frontotemporal Disorders Guide from the National Institute on Aging (NIA).