Tuesday, March 27, 2012

Friendly-to-a-fault, yet tense: personality traits traced in brain, March 22, 2012 News Release - National Institutes of Health (NIH)

Friendly-to-a-fault, yet tense: personality traits traced in brain, March 22, 2012 News Release - National Institutes of Health (NIH):

 "Magnetic resonance imaging (MRI) revealed that patients had decreased gray matter — the brain's working tissue — in the bottom front of the insula, which integrates mood and thinking. By contrast, they had increased gray matter in the top front part of the insula, which has been linked to social/emotional processes."

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Friday, March 23, 2012

Kandoo Band - Watch these WS folks rock out!

My daughter has Williams syndrome

Mom Story: My daughter has Williams syndrome:

"Tatum is in a regular kindergarten class with a full-time aide. The kids love her. She’s pulled from class for occupational therapy, physical therapy, special education and speech. Tatum will repeat kindergarten again next year.
Tatum loves singing, jumping on a trampoline, swimming, playing in the sandbox, going to the park and watching Mickey Mouse and Dora on TV. In the last six months, she started talking in short two-word sentences such as, “Cereal, please,” or “Swim, please.” Tatum’s reasoning skills are above her verbal skills -- she understands a lot more than she can communicate.
My little girl is happy, positive, loving, caring and well-adjusted. She’s very social, and everyone adores her. She hugs you if you’re sad, and she tries to engage anybody. She doesn’t worry that anything’s wrong with her because I don’t think she knows that anything is wrong with her -- I’d never tell her something is wrong."

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Tuesday, March 20, 2012

Imaging study reveals insula disruption in Williams syndrome — SFARI

Imaging study reveals insula disruption in Williams syndrome — SFARI:

"They studied 13 people with Williams syndrome and 23 controls, only a subset of whom participated in each of the various imaging modalities.
It’s only recently that newer techniques, such as DTI and functional connectivity, which infers connectivity based on correlated activity of different brain regions, have become reliable enough to be used more widely, says Paul Thompson, professor of neurology at the University of California, Los Angeles, who was not involved in the study.
The study is unique not only in its target and combination of imaging technologies, but in the population under study. Berman’s team chose to focus only on individuals who have Williams syndrome and a normal IQ, a relatively small percentage of people with this rare disorder, to eliminate the possible effects of IQ.
Individuals with Williams syndrome have differences in brain structure, connectivity and function compared with controls, the study found. Specifically, the researchers found that the gray matter of the dorsal anterior insula, a region associated with integrating emotions and cognition, is smaller in people with the disorder, while the ventral anterior insula, an area implicated in social and emotional processes, is larger.
Functional measures corroborated these results. Blood flow is reduced in the dorsal anterior insula and increased in the ventral anterior insula.
The white-matter tract that connects the anterior insula to the amygdala and orbitofrontal cortex, known as the uncinate fasciculus, is more disorganized in people with Williams syndrome. And activity in the anterior insula is less well correlated with activity in the orbitofrontal cortex, amygdala and anterior cingulate cortex.
Perhaps most significant, the degree of alteration is linked to the magnitude of the individual’s Williams syndrome personality, as measured by a personality test.
The link between personality traits and the insula is specific: Researchers did not find a similar correlation with the hippocampus, part of the brain involved in learning and memory. And verbal IQ, which is unrelated to personality, was not linked to changes in the insula. 

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Brain Architecture and Williams Syndrome | Psychology Today

Brain Architecture and Williams Syndrome | Psychology Today:

"As brain studies continue, we are learning more about the neurological basis of WS. This week, an important new report was published in the Proceedings of the National Academy of Sciences. A team of scientists led by the National Institutes of Health has now found in Williams syndrome cases changes in the connectivity and volume of gray matter in a brain region called the anterior insula (AI), which is thought to control emotion and personality.

The researchers found an overall decrease in gray-matter volume in one region of the AI, along with locally increased volume in another area; compromised white-matter integrity of the structure that connects the insula with some other brain regions; and disturbed neuron interactions between the AI and limbic regions known to be involved in processing emotions. Perhaps most important, the team found that differences in the AI correlate with the extent of WS characteristics. The greater the number and degree of the AI changes, the more of the typical WS behaviors that can be observed and measured in the person."

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Friday, March 16, 2012

Williams Syndrome Grows in Awareness, Research - ABC News

Williams Syndrome Grows in Awareness, Research - ABC News:

"“Awareness has skyrocketed, for a relatively rare syndrome,” said Terry Monkaba, executive director of the Williams Syndrome Association (WSA). Affecting one in 7,500 newborns, Williams Syndrome (WS) causes a combination of ebullience, empathy, fearlessness, linguistic and musical talent, elfin features, heart conditions and bad teeth.
After the “20/20″ story last June, Williams Syndrome was in the top 10 Google trends for three straight days, Monkaba said. The website averaged more than 1,000 hits per day for a month after the broadcast, she added. On an average day before that, it would get 200.
In addition, WSA fundraising is up 35 percent over last year, Monkaba said. The scholarship budget for WSA summer camps has jumped to $135,000 from $35,000 two years ago. The number of camp weeks has grown from four to eight over that period."

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Tuesday, March 13, 2012

Brain scans offer insight into Williams syndrome

Brain scans offer insight into Williams syndrome - health - 12 March 2012 - New Scientist:

"Mbemba Jabbi at the National Institutes of Health in Bethesda, Maryland, and colleagues studied MRI scans of 14 people with the syndrome. They found that the insula – a brain region involved in emotion – was smaller than in 23 people without the syndrome.
When Jabbi's team used PET scans to examine the insula in more detail, though, they found that one area of the right insula was larger in people with Williams syndrome. Those with more extreme personality differences had more grey matter here."

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Wednesday, March 7, 2012

UPDATE: Family’s joy at U-turn on mobility car

Family’s joy at U-turn on mobility car - News - Shields Gazette:

"A SOUTH Tyneside mum has told of her delight after Government bosses did a U-turn on the decision to take away her disabled daughter’s mobility car.

Little Amarni Gibson was told by the Department of Work and Pensions, that the VW Golf her mum Gillian uses to ferry her about would have to go – because they no longer considered her disabled enough to qualify for it.

Mrs Gibson was devastated as her eight–year-old daughter suffers from the rare genetic condition Williams syndrome and is hooked up to a feeding machine 20 hours a day.

The Gazette revealed Amarni’s plight last month, and after Mrs Gibson contacted MP David Miliband her case was referred to the Citizens Advice Bureau in Station Approach, South Shields.

But now the family have been told they will be able to keep the car plus Amarni’s disability living allowance – which had been reduced to the lowest level two years ago – would also be increased."

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Friday, March 2, 2012

A Bear and a Buddy: A Delivery that Changed a Boy's Life

A Bear and a Buddy: A Delivery that Changed a Boy's Life:

"Imagine being a child and never meeting anyone like you. Imagine being a child that doesn't feel special, but ostracized. Imagine being unable to contain your excitement at meeting strangers, wanting to make friends, and seeing them turn away.

According to Holly Knaak, her son Kyle is all of those things. He's never had a true best friend. He's never known anyone his own age who looks, sounds, or behaves like him.

A week with a teddy bear has changed his life forever. Here's the story of why."

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Williams Syndrome, Their Child’s Disorder, Mobilized the Kievs - NYTimes.com

Williams Syndrome, Their Child’s Disorder, Mobilized the Kievs - NYTimes.com:

"A day after their daughter was born, Mrs. Kiev was resting in her hospital room when the cardiologist stopped in. “Marshall had gone home and I was alone, and the cardiologist asked me if I had heard of Williams Syndrome, and my heart started to thump and I said to him, ‘What does that mean?’ ” She called her husband immediately."

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