I found an Alzheimer’s Weekly in my inbox that gave me hope that scientists may be on track to find effective treatment for the rare type of dementia Jim had. While Jim was living, we didn’t know exactly what disease he had, only that it was an Alzheimer’s type of dementia. Early on, the neurologist suspected Jim might have Pick’s disease, or a disease that didn’t even have a name.
When the autopsy report came in, I sent it to the neurologist to find out exactly what disease Jim had. All I knew for sure after reading the report was that Jim had no evidence of Alzheimer’s. The neurologist called me back in a few minutes and gave me the name of a disease I had never heard of: corticobasal degeneration, sometimes called corticobasal ganglionic degeneration.
The 7th Annual International Conference on Frontotemporal Dementia focused on a disease that is so rare it affects only about 20,000 people.
Frontotemporal Dementia Facts
Accounts for 2 – 10% of dementia
The onset is usually between 40 and 65 years of age
The expected lifespan is 5-10 years
Can run in families
Familial FTD is linked to the “tau” gene, chromosome 17
The Frontotemporal Family of diseases affects behavior and causes problems with language. Jim developed dementia at 49 years old and died at 59. His personality changed and his behavior became childlike at time. One of Jim’s first symptoms was problems with language—spoken and written. Jim could write letters and sometimes even have the correct letters in a word, but they might be jumbled. I had him write a grocery list for me once and he wrote “taper powels.” He later looked at the list and said, “Why did I write it like that?” He spoke in familiar phrases for a long time and was almost totally silent the last two years of his life.
The FTD diseases include
Primary Progressive Aphasia
Some doctors propose adding other diseases to the FTD classification and call the group Pick’s Complex. The diseases that may be soon be included in FTD beneath the umbrella of Pick’s Complex are corticobasal degeneration and progressive supranuclear palsy.
I’ve never understood why corticalbasal degeneration was not included in the various Alzheimer’s type of dementia. Jim's diagnosis after the mental exam was “dementia of the Alzheimer’s type.” And corticobasal is definitely that.
One of the differences between FTD and Alzheimer’s is that FTD usually begins with behavior and communication problems, where Alzheimer’s begins with memory loss.
Personality and behavior changes
Lack of empathy and impaired social interactions
Compulsive behavior and repetitive actions
As the disease progresses—impaired motor skills, speech, and movement
Corticobasal degeneration is considered a movement disorder. Late in the disease, Jim kept falling and used a device that looked much like a giant baby walker to keep him in an upright position. He also had what is known as “alien limb” and didn’t seem to know what to do with his right arm. He clinched his right hand so hard for so long that he developed a pressure sore and had to have his little finger amputated.
People with FTD cannot stop or control their behavior—the frontal and temporal lobes are the first part of the brain to sustain damaged. Since FTD is typically a younger onset dementia it is often mistaken for a mental disease rather than a degenerative brain disorder. Out of control behavior, personality changes, and poor financial judgment are some of the symptoms that create strained relationships with loved ones. Caregivers are only human and although logically we know our loved one is not deliberately being difficult, sometimes feelings are hurt, or the caregiver feels unappreciated, unloved, and becomes depressed.
My experience is that it is best to take one day at a time—or even one hour at a time. I accepted Jim for the person he became, knowing that he would never again be the man he had been. It helps to know that some of the behavior can be controlled with medication and from time-to-time you see a fleeting glimpse of a familiar expression, or a genuine smile.
I always cherished the moments when I said, “I love you” and Jim replied, “I love you too”--especially, when he said it like he meant it.