Q11 Williams vs. Downs syndromes Day 37

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Q11Williams vs. Downs syndromesDay 37

• Brain Language
• LING 411/412/489
• NSCI 411/611/489/689
• Harry Howard
• Tulane University

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Q11
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Q11 answers

• Indexes a metaphoric or anomalous meaning.
• c) N400
• Indexes a parsing breakdown in a garden-path
  sentence
• d) P600
• Indexes a subcategorization violation.
• b) LAN
• Indexes an agreement violation.
• b) LAN
• Indexes initial syntactic structure produced by a
  regular grammar.
• a) ELAN
• Indexes the verb that subcategorizes for the gap
  in a filler gap-dependency.
• d) P600
• Observed from 500-1000 ms
• d) P600
• Peaks at 100 ms
• a) ELAN
• Peaks at 150-200 ms
• b) LAN
• 10. Peaks at 400 ms

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Course administration

• Course home page
• http//www.tulane.edu/ling/LING411/
• Readings are found
• MyTulane gt BrainLanguage_CC Brain and
  Language(Combined) gt Course Documents
• I move slides around to make topics of
  presentations consistent
• See link Oral Presentations on Assignment page
  
• http//www.tulane.edu/ling/LING411/ppt/d99-OralP
  resent.ppt
• Course evaluations are online! -- see next slide

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Course evaluations

• All students will receive an e-mail notice on
  December 2, asking them to go to My Tulane and
  fill out the evaluation form.
• The period to fill out the forms is one week
  long, during which time they will receive two
  more reminder notices.
• There is nothing required of faculty to complete
  the evaluation process.
• The questions on the evaluation form are
  unchanged.
• The new process is completely confidential and
  anonymous, more so now than ever.
• While your are at it RateMyProfessors.com

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Course evaluations

• Please respond to the following questions on the
  back of the evaluation form
• Were the books other readings useful?
• Would you prefer to have the additional readings
  as pdf files (free) or a photocopied course pack
  (costs money)?
• Was the assignment web page helpful?
• Did you look at the slides on the internet or
  download the file? Was that helpful?
• Did you ever download listen to a mp3? Was that
  helpful?
• Was the quiz format helpful?
• Do you have any other suggestions for
  improvement?
• I cant improve the class unless I know something
  is wrong.

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Grades

• All the quiz grades that I have are now posted to
  Blackboards grade book
• Please check them for accuracy
• On Monday I will convert them to 100 pts to
  figure out which one should be dropped
• Quizzes 75/(11-1) 7.5 each
• Blackboards grade book cannot drop the lowest
  grade, so it will not be accurate, but it will be
  fairly close

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Presentations

• Monday, Dec 15, 8-12, here
• 40 people x 6 minutes 240 min 4 hours!
• Please keep to your time!
• Undergraduates 4-5 minutes per person a little
  less if you are in a group
• Graduates 6-7 minutes
• Bring refreshments!
• Guidelines through this link
• Sample presentations
• Sample 1 Feral children
• Sample 2 RH recovery

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Citation

• Bellugi, U., Lichtenberger, L., Mills, D.,
  Galaburda, A. M., Korenberg, J. R. (1999).
  Bridging cognition, the brain and molecular
  genetics evidence from Williams syndrome. Trends
  in Neurosciences, 225, 197-207.

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Abstract

• Williams syndrome (WMS) is a rare sporadic
  disorder that yields a distinctive profile of
  medical, cognitive, neurophysiological,
  neuroanatomical and genetic characteristics.
• The cognitive hallmark of WMS is a dissociation
  between language and face processing (relative
  strengths) and spatial cognition (profound
  impairment).
• Individuals with WMS also tend to be overly
  social, behavior that is opposite to that seen in
  autism.
• A genetic hallmark of WMS is a deletion on
  chromosome band 7q11.23. Williams syndrome is
  also associated with specific neuromorphological
  and neurophysiological profiles proportional
  sparing of frontal, limbic and neocerebellar
  structures is seen using MRI and abnormal
  functional organization of the neural systems
  that underlie both language and face processing
  is revealed through studies using event-related
  potentials.
• The non-uniformity in the cognitive,
  neuromorphological and neurophysiological domains
  of WMS make it a compelling model for elucidating
  the relationships between cognition, the brain
  and, ultimately, the genes.

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WMS symptoms

• 1 in 20,000 to 30,000 live births.
• specific facial and physical features
• a constellation of cardiovascular difficulties,
  e.g. supravalvular aortic stenosis (SVAS)
• failure to thrive in infancy transient neonatal
  hypercalcemia
• delayed language and motor milestones
• abnormal sensitivities to classes of sounds
  (hyperacusis)
• standard Full Scale IQ scores range from 40100,
  with means of around 60
• difficulty with general problem solving
• not able to achieve independent living
• difficulty dealing with money or balancing a
  checkbook

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Drawing vs. description of an elephant by a
teenager with WMS (1A)

• And what an elephant is, it is one of the
  animals. And what the elephant does, it lives in
  the jungle. It can also live in the zoo. And what
  it has, 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 ... If
  they're in a bad mood it can be terrible ... If
  the elephant gets mad it could stomp it could
  charge. Sometimes elephants can charge. They have
  long tusks. You don't want an elephant as a pet.
  You want a cat or a dog or a bird...
• (Full Scale IQ of 49, Verbal IQ of 52 and
  Performance IQ of 54)

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Word fluency Name all the animals you can in
60 sec (1C)

• WMS
• brontosaurus
• tryrandon
• dinosaurs
• ibex
• brontosaurus rex
• elephant
• dog
• cat
• lion
• baby hippopotamus
• whale
• bull
• yak
• zebra, puppy, kitten, tiger, koala, dragon

• DNS
• dogs
• cats
• fish
• bird
• fish

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What if you were a bird? (1E)

• WMS 1 You could fly, you could have babies, fly
  north or south, east or west.
• WMS 2 Good question. I'd fly through the air
  being free.
• WMS 3 I would fly through the air and soar like
  an airplane and dive through trees like a bird
  and land like a bird.
• WMS 4 I would fly through the air where my
  parents would never find me. Birds want to be
  independent.
• WMS 5 I would fly and if I liked a boy, I would
  land on his head and start chirping.

• DNS 1 Bird seeds.
• DNS 2 You'd be strong.
• DNS 3 I don't fly.
• DNS 4 I not a bird, you have wing.
• DNS 5 Fly in the air.

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Sample story-telling task by age-matched and full
scale IQ-matched individuals with WMS and DNS (1F)

• WMS age 17, Full Scale IQ 50
• Once upon a time when it was dark at night ...
  the boy had a frog. The boy was looking at the
  frog ... sitting on the chair, on the table, and
  the dog was looking through ... looking up to the
  frog in a jar. That night he sleeped and slept
  for a long time, the dog did. But, the frog was
  not gonna go to sleep. And when the frog went out
  ... the boy and the dog were still sleeping. The
  next morning it was beautiful in the morning. It
  was bright and the sun was nice and warm. Then
  suddenly when he opened his eyes ... he looked at
  the jar and then suddenly the frog was not there.
  The jar was empty. There was no frog to found
  (whispered).

• DNS age 18, Full Scale IQ 55
• The frog is in the jar. The jar is on the floor.
  The jar on the floor. That's it. The stool is
  broke. The clothes is laying there.

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Localglobal task (2C)

• WMS

• DNS

Model
Y Y Y Y Y Y Y Y Y Y
Y Y Y Y Y Y
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Dissociation between language and (spatial)
cognition

• DNS
• weak complex expressive language (NA)
• weak receptive word knowledge (1C)
• weak verbal memory, strong spatial memory 1D
• weak grammar (1E)
• low linguistic affect (1F)
• preserved spatial cognition (2C)
• weak face-processing abilities
• deficient in theory of mind(?)

• WMS
• strong complex expressive language (1A)
• strong receptive word knowledge (1C)
• strong verbal memory, weak spatial memory 1D
• strong grammar (1E)
• high linguistic affect (1F)
• impaired spatial cognition (2C)
• strong face-processing abilities
• theory of mind(?)

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Neurophysiological profile

• WMS have different organization of ERPs

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Neuroanatomical characteristics

• DNS
• anterior cortex reduced wrt posterior
• limbic structures (e.g. amygdala) reduced
• cerebellum reduced
• subcortical structures (lenticular nuclei) normal

• WMS
• anterior cortex normal wrt posterior
• limbic structures normal (but one had abnormal
  amygdala)
• cerebellum normal
• subcortical structures reduced but these
  prominent in general motor control 11 reduction
  of the left lenticular nuclei in TS subjects
• indentation of temporo-parietal region
• central sulcus ends before midline

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Molecular-genetic profile

• Deleted region in chromosome 7

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Next class

• Some presentations