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 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 FTD diseases include:
- Pick’s Disease
- Primary Progressive Aphasia
- Semantic Dementia
I’ve never understood why corticobasal 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
- Language difficulty
- Compulsive behavior and repetitive actions
- As the disease progresses—impaired motor skills, speech, and movement
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.
Copyright © December 2010 L. S. Fisher
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