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Monday, October 3, 2011

Frontotemporal Degeneration: Rare Brain Disorder Is Highly Hereditary

Frontotemporal Degeneration: Rare Brain Disorder Is Highly Hereditary

ScienceDaily (Nov. 4, 2009) — New research shows that a rare brain disorder that causes early dementia is highly hereditary. The study is published in the November 3, 2009, issue of Neurology®, the medical journal of the American Academy of Neurology.

The brain disorder, called frontotemporal dementia, is formerly known as Pick's disease and destroys parts of the brain, leading to dementia, including problems with language or changes in behavior and personality. The disease often affects people under the age of 65.

"Knowing your family's health history may be one way for people to better predict their risk of developing dementia," said study author Jonathan Rohrer, MRCP Clinical Research Fellow at the Dementia Research Center at the University College London in the United Kingdom.

For the study, blood was drawn from 225 people who were diagnosed with frontotemporal dementia. The people were asked about family history of dementia and given a score of one through four. A score of one represents a person who had at least three relatives with dementia and an autosomal dominant inheritance, meaning that an affected person has one mutant gene and one normal gene and has a 50-percent chance of passing the mutant gene and therefore the disorder on to their offspring. A score of four represents a person with no family history of dementia.

The study found that nearly 42 percent of participants scored between a one and a 3.5, meaning they had some family history of dementia. However, only 10 percent had an autosomal dominant gene history.

The people in the study also had their DNA tested for five gene mutations thought to cause frontotemporal dementia. Mutations were found in two of the five genes.

"Many people were still found to have a strong family history of dementia even without having any of the five known gene mutations, suggesting that there are still unknown genes that cause frontotemporal dementia," said Rohrer.

"Discovering new genes and gene mutations could provide another key to unlocking the doors to new treatments and prevention strategies for dementia."

The study also found that behavioral problems associated with frontotemporal dementia were the most likely to be hereditary, while language problems were the least likely to be hereditary.

The study is supported by the United Kingdom Department of Health's NIHR Biomedical Research Centers, the Medical Research Council UK and the Alzheimer's Research Trust in the United Kingdom.

Comments are welcome.


  1. Heartbreaking. May be good for the research, but heartbreaking nevertheless.

  2. God I hope that is not true! My Dad has FTD and he put us through hell for years with it. It has destroyed our family and he never was able to see that he was doing anything wrong. In reading your blog and others I see that some people with FTD do realize that they are different now and they do try to fight it. My dad never did, he just let it take him over and had a fine time saying whatever he wanted to people, sexually and physically assaulting children and animals, grabbing at his adult daughters and doing everything he could to upset my mom. He thought it was funny and never showed any remorse, even in the early stages. I don't ever want my kids to feel the way I feel about my dad!

  3. I'd like to see the referenced article in the Neurology journal. Maybe I'm not understanding, but it seems that all this is saying is that there are likely more genes involved in FTD than the 5 that have already been identified. There's a lot they don't know.

    As for heredity-
    It seems if you have more than one close relative with FTD, maybe it's familial but the actual risk is unclear. But if you have just one it may not be familial.

    That's what I got from the topic of FTD and Heredity on the UCF web site which is quoted here:


    Approximately 20-50% of individuals with frontotemporal dementia (FTD) have an affected first-degree-relative. Conversely, 50-80% of individuals appear to be the first person with FTD in the family, also called sporadic or nonfamilial FTD. In these cases, other individuals in the family do not appear to be at increased risk for developing the condition.

    Familial FTD is suspected when more than one family member is affected, often in two or more generations. The underlying reason for FTD within the family is not always known. Although there does appear to be an increased chance for other family members to develop FTD, the exact risk is often difficult to assess. In these cases, it can be helpful to meet with a genetic counselor to review the family history and discuss possible implications for other family members.