FEAT DAILY NEWSLETTER
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November 28, 2001
News Morgue Search www.feat.org/search/news.asp
·
Getting Emotional Is a Rational Decision
·
Music, the Brain, and Williams Syndrom
·
Innovatively Nutritional, Behavior Program for Autism
Coming to Anaheim
·
Autism Autoimmunity Project has Raised $110k+ for
Autism Research
[This may shed some insight into the workings of the
autistic/Asperger mind. By Lee Bowman, Scripps Howard News Service.] http://www.brainconnection.com/SITEWare/2001/11/26/a/0000-0017-rationalmind. ph <-- address ends here.
Sorry, Mr. Spock. It appears that even the most analytical
of people turn to the emotional part of their brain when making personal
decisions.
In the brain-imaging study, described Monday at a
radiology meeting, 11 volunteers made choices between the better of two
desirable things or picking between two undesirable events.
The functional magnetic resonance imaging found that the
ventromedial frontal lobe - the part of the brain typically associated with
emotions - was highly active even when volunteers were making what typically
would be considered rational decisions.
In such studies, the brain’s metabolic activity is shown
in a distinctive color on a computer screen, allowing scientists to see what parts
of the brain are working during a given moment of thought.
“There’s an increasingly accepted school of thought in
neuropsychology that there is a significant emotional component to all personal
decision-making, and the brain scans in our study support that,” said Dr. Dean Shibata, who carried out the study at
the University of Rochester School of Medicine in New York.
Until recently, many researchers felt that the brain and decision-making
were divided into separate “rational” and “emotional” components, rather than
integrated and overlapping, said Shibata. He presented his findings before the
Radiological Society of North America meeting in Chicago.
In the study, six women and five men each underwent two
brain scans. During each session, they
were asked to choose between two desirable events, like taking a warm bath or
eating a good meal, or had to select which was the worse of two undesirable
events, such as being in a car accident or a robbery victim.
First, they were asked to make the decisions based on how
it would affect them personally. During the second scan, they were given
similar choices, but asked to base their decisions solely on cost, not as
though the decision would affect their lives.
The scans showed there was significantly more activity in
the frontal lobe when they were making the personal decisions than when they
made choices based on cost.
Shibata, now an assistant professor of radiology at the
University of Washington in Seattle, pointed out that other scientists have
found similar evidence in people who have suffered brain injuries to the
prefrontal lobes due to strokes or tumors.
“They have a very difficult time making even routine
personal decisions, such as scheduling a doctor’s appointment,” but can
objectively make decisions that affect someone else’s life, like recommending
what type of car to buy, Shibata said. But faced with such a choice themselves,
they’re frozen.
“If you eliminate the emotional guiding factors, it’s
impossible to make decisions in daily life,” Shibata said. “Even while making
decisions such as ‘should I put on my seat belt?’ you intuitively realize that
without the seat belt, you might get hurt in a crash. That’s an emotional
image. If you can’t envision that, you can’t make the decision to wear the seat
belt.” The study underscores that surgeons need to be wary around the
prefrontal lobe, the researcher said, and suggests that brain imaging may be
useful in diagnosing psychiatric disorders like schizophrenia or depression,
which may involve abnormal metabolism in the frontal lobes.
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* * *
Music, the Brain, and Williams Syndrome
Rare disorder offers insight into the genetic basis of
cognition
[By Brendan A. Maher in The Scientist 15[23]:20,
Nov. 26, 2001.]
http://www.the-scientist.com/yr2001/nov/research_011126.html
Gloria Lenhoff is a 46-year-old lyric soprano singer who
has performed with such diverse groups as the San Diego Master Chorale and
members of Aerosmith. She can sing nearly 2,500 songs in more than 25
languages, reportedly in a perfect accent. She even has perfect pitch.
But the rest of her world is not perfect. Gloria is
affected by a rare genetic disorder called Williams syndrome. With an IQ of
about 55, Gloria literally cannot subtract three from five or make change for a
dollar. But what she and others with her affliction share is music.
Innately connected, they often have an astute grasp of
music’s
technical aspects-the beat, rhythm, tone, and timbre.
Identified more than
40 years ago, Williams syndrome results from non-homologous
recombination
during gametogenesis that deletes about 20 genes on one copy
of chromosome
7.1 Characteristics of
Williams syndrome include pixie-like features-upturned nose, small chin,
protrusive ears-as well as stunted growth, heart problems, poor visuospatial
cognition, sensitivity to loud noises, a gregarious personality, and an average
IQ of about 60.
Many of these individuals have difficulty with the
simplest of mental and physical tasks, but some abilities, especially verbal
skills, appear to be spared. Classified by some as a nonverbal learning
disability, this syndrome allows speech and language aptitude that far exceeds
their other cognitive functions.
A Williams-afflicted person, for example, couldn’t
scribble more than
a few lines to depict an elephant but could describe one in
expressive,
almost lyrical detail. “It has long gray ears, fan ears,
ears that can blow
in the wind. It has a long trunk that can pick up grass or
pick up hay,”
said a patient in an experiment conducted by Ursula Bellugi,
director,
Laboratory for Cognitive Neuroscience at the Salk Institute
for Biological
Studies.1
The dissociation between language and spatial cognition in
Williams syndrome is evident in the contrast between a stick figure drawing and
verbal description of an elephant by an 18-year-old with Williams syndrome.
To some, perhaps the most striking distinction is the
extraordinary
connection that these people have with music. All exhibit a
strong affinity
for music, and while their attention span for many tasks is
fleeting, they
will spend hours listening to or making music. Research is
scarce, but some
evidence shows a high incidence of perfect pitch, and an
uncanny sense of
rhythm among this group.2,3 One boy with Williams syndrome
was taught to tap
a complicated 7/4-time rhythm with one hand while keeping
4/4-time with the
other.4
Some researchers will not use the word “savant,” but all
admit that a connection with music exists, and that it and the other anomalies
in this syndrome might help to further knowledge about disease and how the
brain develops and works.
Genetic discoveries of Williams syndrome began at the
heart. “We were interested-still are interested-in obstructive vascular
disease,” says Mark T. Keating, Howard Hughes Medical Institute investigator
and professor of cell biology at Harvard Medical School. One such disease,
supravalvular aortic stenosis (SVAS), exists in many Williams syndrome patients
but also occurs in otherwise healthy patients.
For the latter, this genetic disorder results from a
defective copy of
the ELN gene that encodes for elastin, a substance that
comprises about half
of the dry weight of arteries. While conducting linkage
analysis and
fluorescence in situ hybridization (FISH), Keating, then at
the University
of Utah, and his team traced Williams syndrome to a de novo
deletion of ELN
on Chromosome 7. They discovered that the responsible
microdeletion at
7q11.23, unseen without FISH, included about 2 million base
pairs that were
flanked by highly duplicative chromosome regions.1
Using FISH to identify the deletion region has reigned as a
diagnostic tool for Williams syndrome, although work done by Stephen Scherer at
Toronto’s Hospital for Sick Children department of genetics and genomic biology,
recently uncovered a 1.5 million-base pair inversion of the deletion area that
occurs in roughly 5 percent of Williams patients.5 Scherer says, “There’s this
fallacy that you have to have the deletion to have the disease,” which can
cause health insurance problems.
In 30 percent of these cases, the parents were found to
have the inversion without the clinical manifestations of Williams. This
inversion increases the likelihood of unequal crossing over and may be a
mechanistic explanation for the Williams deletion. Genes in the usual deletion
region include the Drosophila homologue, frizzled (FZD3), syntaxin 1A (STX1A), replication
factor C2 (RFC2), the gene encoding for LIM-kinase 1 (LIMK1).
Rare partial deletions, smaller than the typical 2MB
standard, exist, and the varying degrees of Williams syndrome characteristics
they produce offer important insight in connecting cognitive function and
genetics. Individuals with a deletion
that included only ELN and LIMK1 had the heart problems and the impaired
visuospatial constructive cognition associated with Williams syndrome, but no
other symptoms.
It’s believed, says Keating, that LIMK1’s role in
cytoskeletal control and actin formation is responsible for developmental
deficiencies in the posterior parietal cortex. Though work from a UK lab
refutes this evidence,1 examining those rare cases of partial deletions and the
traits they produce can lead to previously unconsidered gene-brain connections.
“For instance,” says Colleen A. Morris, professor of pediatrics, University of
Nevada School of Medicine and clinical collaborator with Keating, “most
children with Williams syndrome have anxiety, but anxiety is also common in the
general population. Might there be a gene within the Williams deleted region
that is important in the general population in terms of anxiety?”
It’s a story that will continue to unfold as new
technology becomes available. Eric Green, director, NIH Intramural Sequencing
Center, presented six previously unreported genes in the deletion area at the
American Society of Human Genetics meeting in October. His lab has been
studying the deleted region in humans and 11 other non-human vertebrates.
“In primates,” Green says, “this is a very complicated
region with these large duplicated blocks. In lower vertebrates it’s not so
complicated and it’s not duplicated.” The evolutionary implications of this
have incited Green to study this gene dense region on chromosome seven, “in
everything from chimpanzees on down to pufferfish.”
Anecdotal evidence of an intimate connection with music, a
great memory for songs, and the kind of auditory finesse that can discern the differences
between vacuum cleaner brands, has followed Williams people for some time, but
little evidence has been published. Neuropsychologist Audrey Don, now at the
children’s therapy unit at Good Samaritan Hospital in Seattle, was one of the
first to explore the relationship. “Cognitively, kids with Williams syndrome
are better with verbal skills. Their word knowledge and use of words is better
than their nonverbal type of thinking,” she says.
She administered a simple musical test of tones and
beats to people
with Williams syndrome and a control group matched for
vocabulary level. She
found that musical ability matches verbal ability and was
higher than the
Williams’ children overall cognitive abilities.2
Their parents, providing further survey information,
reported an extremely strong and emotional connection with music. A lullaby
tape, says Don, made one infant cry. When the child was older, she was asked
why she wept; the child said the songs were too sad. An impromptu study
conducted at the Williams Syndrome Music and Arts Camp in Massachusetts’
Berkshire Mountains gave another inkling into this particular peak of Williams cognition.
The experimenters asked eight children to imitate clapped
rhythms. They performed as well as
normal, musically trained students who were matched to their mental age of five
to seven years.3 But, the professional musicians that coded the responses
qualified the mistakes of Williams subjects as “wrong in an interesting way.”3
They often missed the exact sequences, but creatively kept within the realm of
the time signature, much like a jazz musician will jam.
The Williams subjects were three times as likely as
controls to offer what the researchers called “creative completion” to the test
rhythm when giving an incorrect response. Howard M. Lenhoff, professor
emeritus, School of Biological Science, University of California, Irvine,
recently completed a study linking Williams syndrome to a higher incidence of
absolute or perfect pitch, a condition that normally occurs in one out of
10,000 people in Western populations; these people often study music from a
very early age.
In numerous trials, five musically trained Williams
subjects, including Lenhoff’s daughter, Gloria, displayed near-ceiling levels
of absolute pitch.2 Of the subjects, which represent about 1/1000 of the Williams
population, four could read music and name notes, a rare ability in Williams
people. Gloria, says Lenhoff, was the only one unable to read music and had to
be taught, but she still performed within the acceptable range of absolute
pitch. Lenhoff chose a nonrandom sample of subjects because of their ability to
name notes. While criticized for choosing outliers, he says, “If you look for
the average, you’ll find the average.”
The age at which these participants began to study
music raises other
questions. It’s commonly accepted that to develop perfect
pitch, one has to
study music before age six, yet all of the subjects, save
one, started after
this critical period. Lenhoff predicts that this period is
extended in those
with the syndrome. “The open window gets jammed,” he says, “It’s
open in
extended years, and I think into adulthood.” Lenhoff and
others hypothesize
that this open window may be critical for language
acquisition in early
years, but in normal populations it often fades with
disuse-somewhat less
often, incidentally, in populations that speak tonal
languages such as
Mandarin or Vietnamese.2
·
Article and references continue at:
http://www.the-scientist.com/yr2001/nov/research_011126.html
* * *
Innovatively Nutritional, Behavior Program for Autism
Coming to Anaheim
January 11-12, 2002
Using Applied Behavior Analysis as a treatment base that
is fully integrated with the medical and nutritional treatments offered by its Center,
the International Child Development Resource Center (ICDRC) is marketing a
program which begins touring the country the first of next year. ICDRC’s focus is to lead families to
comprehensive, integrated treatment soon after the child is diagnosed and at a
cost they can afford.
The first stop is in Anaheim, California January
11-12, 2002.
The seminars, a program called Open Windows Essential
Training, educates parents, professionals and educators on what issues may
exist with their children with autism and how to address them. The two-day
session is not just another conference. It’s practical training that can be
applied immediately.
For the Registration Brochure for more information
go to:
http://www.angelfire.com/on/FEATNews/ICDRC_Brochure.html
* * *
[From AAP’s Ray Gallup.]
We are proud to announce that due to the September
fund-raiser by Casi’s Quest on September 8th, that the Autism
Autoimmunity Project has now raised over $110,000.00 for research.
The Autism Autoimmunity Project started raising funds for
research in 1999. Congratulations to all the parents that have made this
possible. Special thanks and gratitude
to April and Ronnie Oakes and all their board members at Casi’s Quest for what
they have done and are doing for our children.
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