Learning and Behavior Related to Turner Syndrome

The purpose of the following information is to overview general strengths and weaknesses related to learning and behavior in those with Turner syndrome.

It’s difficult to generalize the TS population because there is a high degree of individuality among people, even those with the same karyotype. A minority of those with TS have careers high-status careers such as doctors and a small percentage lack the ability to manage their everyday needs and depend on others for help. The vast majority of those with TS function between the two extremes and are satisfied with their quality of life.  

Being aware of the strengths and challenges related to education, relationships and work are important to support appropriate and timely interventions.

 

The following are common diagnoses for those with TS. Research suggests the same interventions for the general population may be effective for those with TS as well.

 

  • Attention-deficit/hyperactivity disorder (ADHD)

  • Specific learning disorders (SLD)

  • Social communication disorder

  • Autism spectrum disorders

  • Developmental coordination disorder

 Intellectual Functioning 

Most people with Turner syndrome have average intelligence with approximately

10% experiencing intellectual disability. Intelligence consists of verbal and

performance abilities and much overlap exist between the following abilities:

  • verbal reasoning (problem-solving based on words and language)

  • visual-spatial abilities (spatial relationship of objects)

  • perceptual reasoning (using the senses)

  • executive functioning (controlling behaviors)

In TS, verbal reasoning is consistently higher than perceptual reasoning. When there is a large discrepancy between verbal and performance IQ, it generally points to a learning difficulty, overcompensation, and/or impairment of some kind.

Verbal reasoning does not simply mean spoken, it expands to letters, numbers, and ways people understand language.

Most people with TS do well with skills that involve language as the foundation. They often enjoy hobbies like crossword puzzles, reading, painting by numbers, or assembling something with written instructions.

Perceptual reasoning involves perceiving/visualizing something in your mind. Someone with TS may struggle with reading a map or assembling furniture easily, even though they completely understand the words on the map or instructions. Related challenges include comprehending the size of letters or objects such as reading different fonts, forming letters or numbers correctly, and copying information from sight to paper. Perceptual skill deficits may affect handwriting, driving, and organizational tasks. Visual Perception: An Overview for Understanding - YouTube

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Academic and Employment Achievement 

  • The education level of those with TS  is similar to or greater than that of the general population.

  • The employment status of young women with TS is equal to or higher than that of similar groups although retirement occurs much earlier.

  • Research suggests that as adults with TS age their occupational status often declines. The finding relates to their educational level exceeding the job level. Additionally, older women report their current work experiences as less positive and more challenging. Reference: High Levels of Education and Employment Among Women with Turner Syndrome

 

Executive Function and Control Deficits 

People with TS may experience impairments within different areas of the management system of the brain (cognitive) related to planning, flexible thinking, and controlling thoughts and behaviors. It is unclear which impairments may only affect those with ADHD symptoms.

Some professionals use the term nonverbal learning disorder (NLD or NVLD) to describe the unique deficits related to specific executive function deficits in TS such as organization, integration, planning, prioritizing, time management, and self-monitoring. Someone may have challenges with nonverbal skills yet speak effectively and have an outstanding vocabulary.

 

 

 

 

 

 

 

 

 

 

 

 

 

The following cognitive abilities often pose challenges to those with TS:

Working Memory. Working memory is like a temporary sticky note in the brain. It is a skill that allows someone to work with information without losing track of what they are doing.

 

The following impairments are related to working memory deficits.

Big picture processing is often difficult for individuals with TS that are detail-focused. They often struggle to process or describe components as a whole concept, rather than each component separately.​​

 

 Common behaviors resulting from big picture processing impairments:

  • doing more than necessary to reach the desired outcome at the cost of time and energy

  • producing work that misses the main point of the task or demonstrates a lack of understanding

  • becoming overwhelmed and giving up which may be interpreted as a lack of motivation

  • attempting to overcome challenges by redoing work

  • difficult with comparisons and determining how two similar but different objects are alike or different

  • retention: learning and initial retention of information appear normal, but the information is often forgotten very quickly.

Math skills: 50-75% of those with TS have poor performance in math which is related to a learning disorder called dyscalculia. It is a neurological condition that affects school-level mathematics skills. Some children with dyscalculia cannot grasp basic number concepts and work hard to memorize them yet miss the logic behind the concepts. 

 

Recommended interventions for math skills:

  • academic accommodations such as tutoring, allowing for additional time, and using verbal strengths.

  • occupational or physical therapy to support hand-eye coordination and visual-spatial issues at school and in daily life. Examples include using graph paper to align numbers, and activities to strengthen working memory.

  • Research-based games targeting math deficits; The Number Race and Graphogame.

Flexible Thinking is the brain’s ability to control actions and to adapt flexibly to changing environments. Those with TS often exhibit rigid thinking, which is a result of the brain's inability to be flexible and not to be confused with stubbornness or difficulty. It is important to recognize the frustration someone with rigid thinking must feel.

 

Unfortunately, rigid thinkers may try the following ways of adapting to changing environments:

  • Actively resists any form of change

  • Attempt to control all situations

  • Resist following the lead of others – viewed as strongly oppositional behavior (pattern of an angry or cranky mood, defiant or combative, and vindictiveness toward people in authority.)

  • Exhibit repetitive self-stimulation (tapping, biting fingernails, organizing, and reorganizing items, rocking, humming, etc.)

  • Insist on following strict, ritualistic routines

  • Have difficulty moving on from strong negative feelings

 

Attentional Control deficits relate to the ability to concentrate, pay attention, and control impulsive behaviors. Those lacking the ability to maintain necessary attention may have hyperactivity disorder (ADHD) which occurs in 25% of the TS population.

Interventions for attentional control include:

  • neurological testing

  • behavioral therapy, including parent management training

  • classroom modifications to help in-class behavior

  • medications for ADHD when necessary

 

 

 

 

 

 

Motor Skills and Speed

Coordination difficulties include clumsiness and problems with tasks like learning to walk, tying shoes, handwriting, and riding a bike. Some children may be diagnosed with developmental coordination disorder.

Sensory Processing

allows people to physically feel texture and size. Someone with sensory function may be more or less sensitive to sensory inputs and some issues may be related to lymphedema.

Oversensitive people react negatively to

  • clothes that have large seams, tags, or itchy material

  • loud noises and bright lights

  • heights, such as swinging

  • food textures and may gag easily

Under-sensitive function leads to sensory-seeking stimulus and often:

  • Can’t sit still

  • Seek thrills (loves jumping, heights, and spinning)

  • Can spin without getting dizzy

  • Don’t pick up on social cues

  • Don’t recognize personal space

  • Chew on things (including their hands and clothing)

  • Seek visual stimulation (like electronics)

  • Have problems sleeping

  • Don’t recognize when their face is dirty, or nose is running

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Interventions for motor skill deficits include:

  • occupational or physical therapy for coaching and training of the specific motor skill

  • early intervention for preschool-age children

  • academic accommodations as appropriate for school-age children

Perception: Motor and Visuospatial

The perceptual process begins with receiving stimuli from the environment and usually ends with the unconscious interpretation of those stimuli.

 

People with TS often have significantly poorer performance related to:

Visual-spatial processing tasks such as:

  • abstract visual information like infographics or symbols

  • visual-motor integration requires hand-eye coordination and deficits result in problems in test-taking, difficulty copying information from one object to another, and written material may be poorly spaced, and/or unorganized.

  • visual memory allows the recall of information such as situations, objects, places, animals, or people. Examples of deficits include someone getting lost because they don’t remember the surroundings or copying information takes more time.

  • visual tracking is the ability to control eye movements. There are two types of tracking: maintaining your focus on a moving object and switching your focus between two objects. Those who have poor visual tracking have more difficulty driving, may confuse one word for another while reading, and playing sports that require following a ball.

  • figure-ground perception deficits cause trouble seeing an image within a competing background like focusing on one line of print while reading a book, focusing on one piece of information on a busy computer screen, recognizing individual sounds within crowded scenes, and understanding speech in noise.

  • facial processing abilities in TS are generally average to low average.

    • Researchers believe those with TS visual-spatial challenges have the most difficulty recognizing expressions of anger and fear in others. Possibly because they focus attention on the mouth and not the whole face. It is suggested that deficits are related to the way the TS brain codes and memorizes faces. Resulting problems include poor recognition of nonverbal cues such as eye-rolling, frustration, and disappointment, which affects relationships and potentially safety.

    • Eye gaze such as looking, staring, and blinking is an important nonverbal behavior. Looking at another person can reveal a range of emotions including hostility, interest, and attraction as well as the ability to determine if someone is being honest.

    • Compared with peers, women with TS are significantly less accurate in assigning intentional emotional labels (such as “shy,” “hostile,” and “flirtatious”) to visual cues within the upper half of someone’s face. The resulting problems include difficulty starting or maintaining peer relationships.

  • space-form perception is being aware of the relative positions of your own body and objects around you. Space perception provides cues, such as depth and distance, that are important for movement and orientation to the environment. Impairments make driving difficult, such as not knowing if an object is further in distance or just appears smaller.

  • spatial orientation is the ability to identify the position or direction of objects or points in space. Weaknesses in spatial orientation may lead to misplacing or losing items, being late to places, difficulty reading out loud, being unorganized, difficulty judging time, and difficulty with mathematics calculations.

Interventions for visuospatial processing include:

  • academic support if struggling educationally

  • occupational therapy to learn strategies to help with challenges

  • Receiving an evaluation from a psychologist/psychiatrist and possible treatment of poor social skills and issues with anxiety, depression, low self-esteem, social communication disorder, and an autism spectrum disorder.

  • Social skills group therapy through school or community provider

  • Using verbal strengths in educational curriculum

  • Describing materials aloud and using verbal mnemonics such as “Roy G. Biv” to remember the colors of the rainbow

 

Language

Most people with TS have average or above-average verbal reasoning which measures acquired knowledge, verbal reasoning, and attention to verbal materials. Language or verbal-based reasoning involves talking/conversing, hearing/listening, writing, reading, and thinking. Language can be negatively affected if someone has hearing loss or certain executive function skill deficits.

People with TS generally are average to above average in the following:

  • spoken and written communication

  • the ability to hear quickly and correctly, store, recall and make different speech sounds. (phonological processing)

  • understanding words, phrases, and sentences; including naming, categorizing, understanding, and recognizing different words, defining words, synonyms, and antonyms, and understanding multiple meanings (semantic skills)

  • reading and pronouncing longer and unfamiliar words when compared with age-matched peers.

  • reading comprehension

  • understanding spoken and written words such as “put on your coat”

  • expressive vocabulary in speech or writing: speaking, gesturing (example- waving), writing (example- texting) expressions (example- smiling), and vocalizations (example-yelling)

  • comprehending and responding to words, even if the person cannot produce those words.

  • processing sounds to develop or understand words, babbling /ma-ma/ attaches to mama/momma, becoming the word mommy (phenomics processing)

  • having long-term memory for verbal information

People with TS generally have below-average skillsets for:

  • fluency tasks such as the ability to express oneself easily and articulately such as self-talk, listening to and recalling spoken information.

  • verbal recall and the ability to remember specific information after a period of rest or distraction from that information. Those with TS often have delayed verbal recall.

  • expressive language is related to the use of words, sentences, gestures, and writing to convey meaning and messages to others. Those without good expressive language skills have difficulty sharing their thoughts or ideas or showing they understand what others are saying. They may use vague words like “thing” or “stuff”, use simple sentences, or have long pauses before speaking.

Interventions for language deficits include:

  • Using verbal strengths to enhance academic and work performance such as oral testing or verbal presentation to assess acquired skill or knowledge.

  • Considering occupations that rely on verbal strengths.

Specific recommendations related to intellectual functioning and those with Turner Syndrome

  • Be aware that TS is associated with a cognitive profile (brain pattern) that may negatively impact educational/work success.

  • Integrate the following health providers into the care of individuals with TS

    • a neuropsychologist (focuses on how the brain works) to perform a neuropsychological evaluation at preschool age, at school entry, at the transition to high school and higher education, or at any time that difficulties arise. It’s important the evaluator have knowledge of TS- specific cognitive deficits.

    • allied behavioral health services (services provided by social workers, counselors, and psychiatrists) to support the cognitive and psychosocial challenges associated with TS such as:

      • Specific learning disorders

      • Social communication disorder

      • Autism spectrum disorders

      • Developmental coordination disorder

    • refer children for occupational and/or physical therapy in early life or at school entry

      • helpful for spatial deficits interfering with academic/employment functioning

    • refer children for speech therapy in early life or at school entry

  • Interventions developed for those without TS may be adapted with similar positive effects.

  • When learning or work issues are present, academic or employment accommodations should be made, including tutoring/training, an extension of time demands, and utilizing learning/teaching/training strategies that take advantage of verbal strengths.

  • Utilize learning disability resources for help with intervention and strategy ideas to overcome or minimize deficits.

The information summarized within the article was substantially gathered from the following resources:

 
 
 
 

Resources for learning and behavioral needs

"Every Student Succeeds" Act;

education law for public schools

What You Need to Know (link)

Driving and TS

Livescribe Smart Pen- helpful tool for taking notes in class (link)

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Beckham Linton,M.A., CCC-SLP

Heartland Social Learning Center

Social Coaching, Language and Communication Coaching

beckham.hslc@gmail.com

www.heartlandsociallearning.com

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Diane McLean,MEd, PCC 

Odyssey Learning, LLC

Life Coaching for ADHD and Executive Functions

Odyssey Learning can help you develop important executive functions to accomplish your goals. 

www.execskills.com

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Barbara Bissonnette,CPC

Forward Motion Coaching

Career and Employment Coaching

In-person coaching available in Massachusetts; Skype and telephone coaching available nationwide and in Europe.

barbara@forwardmotion.info

www.forwardmotion.info

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Dean Mooney,PhD, LCP,LSP

Maple Leaf Clinic

Neuropsychology, Nonverbal Learning

Neuropsychological, educational, psychological, speech and language, and social thinking assessments and therapy  for children, adolescents, and adults.  

info@mapleleafclinic.com

www.mapleleafclinic.com