Acadia Touch Points Blog

Acadia Hospital Psychiatrist Clifford Singer, MD

Just a few days ago, Rachel Wing, RNC, the principal nurse for the Older Adult Mood and Memory Clinic at The Acadia Hospital, remarked on how many patients we follow with frontotemporal dementia. I had been thinking the same thing. We seem to follow a lot of patients with this condition, considering it is a rare disease (actually a group of diseases). I wondered if it just seems like a lot of patients because each case leaves a strong impression. Each case seems particularly tragic. Frontotemporal dementia (FTD), unlike Alzheimer’s disease and most other causes of dementia, tends to develop in people aged 40 to 70 years. They are usually still working, have kids at home or even recently married. Then everything changes. They develop dementia; a condition they otherwise would not have worried about getting for several more decades. And here are spouses and teenage or young adult children having to cope with a progressive and fatal neurodegenerative disease in a loved one. I find myself getting choked up every time I tell a patient and family that this is the diagnosis.

But why do we have so many? Rachel and I counted six we follow routinely and two more we follow from a distance. Am I over-diagnosing FTD? I reviewed the diagnostic criteria in my mind……middle-age onset of altered personality and impulsive behavior leading, at first to a psychiatric diagnosis, being fired from jobs, even legal problems. Aphasia starts with word-finding problems leading to muteness. Dementia gradually develops with executive dysfunction, memory loss and a dense lack of insight into the problem. Despite normal muscle strength, they lose the ability to do routine tasks. The “software” driving motor skills is lost so they eventually lose the ability to walk and swallow. Imaging studies such as brain CT and MRI scans may be normal or show general atrophy, more pronounced in the frontal areas of the brain. After the initial erroneous psychiatric diagnosis, most FTD patients will also mistakenly be diagnosed with Alzheimer’s disease.

I thought about the cases I’ve diagnosed at Acadia. In only one patient had I changed my original diagnosis; a 72 year-old woman I now think has Alzheimer’s disease. All the others, most are in their 50’s, one in his late 40’s, have FTD. And then this week, Rod O’Keefe RNC and I saw a 30 year-old man diagnosed with FTD by a neurologist at the Harvard Alzheimer’s Research Center, which is a leading site for FTD-related research. “No way!” I thought. “This is from the traumatic brain injury he sustained in a snow-boarding accident.” But as I read through this young man’s records, I realized his accident caused only a mild concussion. The dementia started long after that. His history, as presented in the records and confirmed by his family, was a classic progression of FTD. Personality changes, self-centered and impulsive behavior, substance abuse, homelessness, self-neglect, then inability to speak or care for himself. He attended college on a performing arts scholarship and three years ago was a dancer for a professional ballet company in Maine. Now, he has severe dementia. We got him to sing “Piano Man” but say little else. He is big and strong and requires two caregivers at all times.  

This is such a tragic disease. We can treat some of the psychiatric symptoms and offer advice and support to the family. We can make a difference. Families are grateful for the help we provide in improving sleep, impulsivity and agitation, but there is nothing to alter the course of the disease. The Sunday New York Times had a cover story on FTD on May 6^th. The video that accompanies the story is particularly touching in its perspective of a wife caring for her husband near the end of the long process of dying with FTD. Have a tissue handy:

(http://video.nytimes.com/video/2012/05/05/health/100000001343193/in-love-and-loss.html?ref=health)

So, is FTD rare? Do I over-diagnosis FTD? I don’t think so. I’ve done some quick calculations. Although we don’t really know how many people in Maine have FTD, it’s probably about 600, based on national prevalence data. Since Acadia is becoming known as a place to go for unusual cases of dementia, I think our eight patients (now nine) are only the “tip of the ice berg”.  We’re likely to see quite a few more cases in the future. Rachel and I are discussing what more we can do to help. Since the families touched by FTD are generally in a different phase of life than Alzheimer’s disease families, they struggle with different issues. Having a 52 year-old spouse with dementia is different than having an 82year-old parent with dementia. If you or someone you know is interested in helping to start a family support group for FTD, please let us know by contacting Alan Comeau at acomeau@emhs.org.

Clifford Singer, MD

Adjunct Professor, University of Maine, Orono

Chief, Division of Geriatric Mental Health and Neuropsychiatry

The Acadia Hospital and Eastern Maine Medical Center