Syndrome Challenges in the School Suzanne Bousquet PhD Executive Director School of Psychology Kean University Turner syndrome affects approximately 1 out of every 2000 female live births worldwide ID: 710144
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Slide1
Implications of Turner Syndrome: Challenges in the School
Suzanne
Bousquet
,
Ph.D.
Executive Director, School of Psychology
Kean UniversitySlide2
Turner syndrome affects approximately 1 out of every 2,000 female live births worldwide. Each year 800 new cases are diagnosed in the United States alone.
At least 60,000 American females have Turner Syndrome.
If the average classroom size is 24, and if 12 of the 24 students are girls, then there will be one girl with Turner Syndrome (diagnosed or undiagnosed) in every 160 classrooms at any one time.
Turner Syndrome StatisticsSlide3
Just as there is great diversity in the physical implications of Turner Syndrome (e.g., the presence of cardiac problems), there is great diversity in the cognitive issues related to TS.
Due to varied cognitive issues, girls with Turner Syndrome easily “fly beneath the radar” of educators.
Diverse Cognitive ProfilesSlide4
The hallmark cognitive phenotype of TS is significantly higher Verbal IQ scores than Performance IQ scores (aka Nonverbal Learning Disorder).
However, not all girls with TS show this cognitive
phenotype.Moreover, not all girls with TS who have this profile have comparable degrees of verbal strengths and non-verbal weaknesses.
Turner Syndrome Cognitive PhenotypeSlide5
Neuroimaging studies have reported both anatomical and functional differences between girls with TS and age-matched controls in the following areas: Parietal Cortices
Dorsolateral Prefrontal Cortices
Superior Temporal GyrusHippocampal FormationAmygdalaTemporal Lobes
Turner Syndrome: Brain Development Slide6
Specific brain involvement is further supported by the distinct neurocognitive profile found in girls with TS:Unlike many chromosomal disorders, Turner Syndrome is not associated with mental retardation.
Normal Verbal Abilities
Impaired Visual Spatial AbilitiesImpaired Working MemoryImpaired Executive FunctionsImpaired Attentional Focus
Turner Syndrome: Cognitive Functioning Slide7
The Verbal-Performance IQ split does not provide sufficient specific information about the cognitive strengths and weaknesses.Many of which researchers still do not fully understand.
Interestingly, the variation in full scale IQ scores among girls with TS parallels the variation seen in their own mothers (who do not have TS).
Verbal IQ at same level or higher than mothersPerformance IQ always lower than mothersThis means that mother’s IQ partially predict daughter’s IQ scores.
Implications of Cognitive Phenotype and Brain Functions?Slide8
Girls with TS:perform at par or above peers in word recognition, reading and spelling; some girls are described as avid readers.
have well-developed phonological processing skills, word knowledge, verbal comprehension and verbal reasoning.
relative strength in simple arithmetic, number comprehension and productivity, number comparison, and estimation accuracy.
Cognitive Strengths in Turner SyndromeSlide9
Girls with TS are often diagnosed with Math Learning Disorder.Difficulties are not related to poor number sense or calculation accuracy and are independent of spatial skills.Exactly what is the issue with Math?
Processing Speed
Girls with TS are slower on arithmetic fact retrieval and response times on calculation and estimation problems.
Cognitive Weaknesses in Turner
Syndrome: MathematicsSlide10
One Minute Test Name __________________ Test 1 Sums to 106 + 2 = _____ 5 + 2 = _____ 7 + 1 = _____
1 + 9 = _____ 5 + 5 = _____ 2 + 4 = _____
7 + 2 = _____ 9 + 0 = _____ 8 + 2 = _____ 7 + 3 = _____ 4 + 6 = _____ 1 + 5 = _____ 2 + 3 = _____ 3 + 3 = _____ 5 + 3 = _____ 4 + 4 = _____ 4 + 2 = _____ 5 + 4 = _____
3 + 4 = _____ 8 + 1 = _____ 9 + 8 = _____
Speeded Math
Test ExampleSlide11
Girls with TS have difficulty with:visuoperceptual, and
visuoconstructional
tasks visual-motor integrationcopying from the chalkboardVisuoperception
deficits are apparent on object identification and location identification tasks.
Visuoconstructional
deficits are apparent on tasks that involve visual working memory, as well as processing speed.
Cognitive Weaknesses in Turner Syndrome:
Visuospatial
DeficitsSlide12
Mental Rotation TaskSlide13
Girls with TS have difficulty with:Both working memory and long-term visual memory.
Difficulty recognizing and recalling shapes and sequences.
Girls with TS perform better on tasks involving visual working memory when they “talk themselves” through the task.Cognitive Weaknesses in Turner Syndrome: MemorySlide14
Types of stimuli used to test
visual working memorySlide15
Presentation 1Slide16
Presentation 2Slide17Slide18Slide19
Girls with TS have weaknesses in:Their ability to plan, organize, monitor and execute multi-step problem-solving
Managing materials
Following directionsInitiating tasksCognitive flexibilityThey do have the ability to plan and organize steps toward a goal, however, their poor search strategies, speed of responding, and cognitive inflexibility interfere with reaching the goal.
Cognitive Weaknesses in Turner Syndrome: Executive FunctionSlide20
In one minute, name as many animals as you can think of?In one minute, state as many words you can think of that start with the letter “S”?
Note: Girls with TS are noted to have difficult with verbal fluency tasks
Quick and Easy Executive Function TestSlide21
There is an increased incidence of attention-deficit/hyperactivity disorder (ADHD) among girls with TS versus children in the general population.Remains to be seen if cognitive profiles and treatment response patterns in TS are similar to typical ADHD
(i.e. treatment implications are complicated in TS by growth disturbances).
Some researchers propose that increased incidence of ADHD Combined Type, which is mostly reported in boys versus girls in the general population, may be attributed to both girls with TS and boys only having one X chromosome. Cognitive Weaknesses in Turner Syndrome:
Attentional
FocusSlide22
Trail Making Test ExampleSlide23
Girls with TS may show difficulty interacting with their peers. Some research has shown that there are specific deficits in immediate memory for faces. It may take longer to encode a face and interpret facial emotions. This can impact everyday social communication.Some researchers argue that girls with TS have difficulty reading facial expressions that connote emotions, especially fear.
Because of deficits in visual attention, subtle social cues may escape the notice of a girl with TS.
Cognitive Weaknesses in Turner Syndrome: Social CognitionSlide24
Girls with TS appear physically younger than their age mates. A contemporary phenomenon, called “Grow Older Younger,” as exemplified in fashion, and cultural icons, may mean that girls with TS are seen as less desirable for friend selection. Adolescent girls with TS begin dating later than other adolescents. Sexual activity also begins later for girls with TS.
Some may struggle with low self-esteem and higher rate of lifetime depression.
Possible physical differences associated with TS may compound these issues.Social Cognition ContinuedSlide25
Prepare student for the daily routine and changes immediately when entering the school. Give parents the next day’s routine prior to coming to school.Place in small group math class or provide support for large classroom instruction and assignments.
Introduce new activities one step at a time to minimize frustration.
Provide opportunities for cooperative work with good role models.Allow student to work on the floor when appropriate.Classroom Implications and Recommendations for Younger ChildrenSlide26
Provide assignment notebook and assist with completion.Instruct with specific verbal cues and written reminders.Provide a copy of material and notes from the chalkboard.
Provide binder with dividers or multi-colored folders for each subject.
Verbally point out individual objects and how they relate to the whole.Limit amount of problems or questions on a page and use consistent format. Classroom Implications and Recommendations for Older ChildrenSlide27
Teach memory and comprehension strategies.Reinforce verbal strengths with class discussion and cooperative group activities.Encourage use of word processor for writing.
Allow extra time for work completion and test taking.
Placement in more functional academic programs instead of higher level courses.Be sensitive to the tendency of feeling “overloaded”.
Classroom Implications and Recommendations for Older ChildrenSlide28
Provide opportunities to “shine” in strength areas in the classroom.
Verbally explain humor and commonly misinterpreted sayings.
Provide non-threatening interaction with peers.Involve school counselor or social worker to provide direct instruction in social skills and problem solving.
Encourage positive peer interaction in and outside of school.
Overall, focus on the child’s strengths, but acknowledge areas of growth needed.
Classroom Implications and Recommendations for SocializationSlide29
Girls with Turner Syndrome have:normal intelligence.
Impairments in motor skills, visual-motor coordination, and visualizing objects in relation to each other. This type of impairment is termed nonverbal learning disability (NVLD), which is manifested by a discrepancy between verbal and performance IQ scores with verbal scores being higher.
difficulty with mathematics, tasks requiring manual dexterity, and poor directional sense (i.e., difficulty with left and right). understanding social and nonverbal cues which may lead to social immaturity.
Summary of Cognitive FunctioningSlide30
There is need for a COLLABORATIVE INTERDISCIPLINARY approach TO identify TS girls and to PROVIDE EFFECTIVE INTERVENTIONS
Teachers – General Ed and Special Ed
School NursesSchool PsychologistsSpeech Pathologists, Audiologists
Playground Aids, Girl Scout Leaders, and Others Who Interact Regularly with GirlsSlide31
These patterns of cognitive strengths and weaknesses are seen in a high percentage of Turner Syndrome. Consequently, it may be beneficial for girls to have academic and psychological testing early on so parents and teachers can help girls compensate.
Clearly these young women face many challenges in living with such a disorder. Though they may have increased health risks and face some learning difficulties, with proper treatments and counseling, all evidence suggests these girls can live full, healthy lives.
Early diagnosis is critical for these girls to develop to their full potential.
Conclusion