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Behavior, Learning, and Emotions in DMD SMDF Conference 2014 Stockholm, Sweden. James Poysky, PhD Clinical Assistant Professor Baylor College of Medicine. Overview. How common are behavior and learning concerns in DMD? Causes of behavior and learning problems in DMD Coping with DMD

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Behavior learning and emotions in dmd smdf conference 2014 stockholm sweden

Behavior, Learning, and Emotions in DMDSMDF Conference 2014Stockholm, Sweden

James Poysky, PhD

Clinical Assistant Professor

Baylor College of Medicine


  • How common are behavior and learning concerns in DMD?

  • Causes of behavior and learning problems in DMD

  • Coping with DMD

  • DMD and brain functioning

    • Cognitive skills and learning problems

    • Neurobehavioral disorders

  • Treatment recommendations

Behavior concerns in dmd
Behavior Concerns in DMD

DuchenneConnect (preliminary data that may change before publication)

Learning concerns in dmd
Learning Concerns in DMD

DuchenneConnect (preliminary data that may change before publication)

Potential causes
Potential Causes

  • Psychological

    • Coping with DMD

  • Psychosocial Factors

    • Family stress/conflict

    • Peer interactions

    • Teachers/adults

  • DMD impact on brain functioning

  • Medical factors

    • Steroids

    • Fatigue/sleep

    • Medical procedures

    • Blood sugar

Coping with dmd

How are the boys coping?

  • Same as boys with other chronic medical conditions

    • Being sad and frustrated at times due to DMD is a normal reaction

    • Coping gets better with age

    • Ages 8-10 and adolescence might be extra difficult

    • Some boys may become depressed/distressed

Hendriksen, Poysky, Schrans, Shouten, Aldenkamp, Vles, 2008; Fitzpatrick et al 1986; Liebowitz et al 1981

Coping with dmd1

  • Some boys not as “independent” as they could be

  • Big focus on transition to adulthood

    • Living independently

    • Making decisions in medical care

    • Employment

    • Romantic relationships

  • Peer interactions in dmd
    Peer Interactions in DMD

    Social Problems: 34%

    • Immaturity

    • Overly dependent

    • Social skills deficits

    • Social anxiety

    • Teasing/bullying

    • Peer inclusion

    Hinton, Nereo, Fee, Cyrulnik, 2006

    Families and dmd
    Families and DMD

    • Family Adjustment

      • Increased rates of parental depression and isolation

      • Behavior problems can be as stressful for parents as physical aspects of DMD

      • Sibling adjustment

        Abi Daoud, Dooley, Gordon 2004; Bothwell , Dooley , Gordon , MacAuley, Camfield 2002; Poysky & Kinnett , 2009; Nereo, Fee, Hinton, 2003

    Dystrophin in the brain
    Dystrophin in the Brain

    • Full-length dystrophin

      • cerebral cortex

      • sub-cortical structures

      • Cerebellum

    • Smaller isoforms

    Dystrophin in the brain1
    Dystrophin in the Brain

    • Possible effects of absent or dysfunctional dystrophin:

      • Makes neurons less efficient in sending signals to each other

      • Makes neurons less ready for new signals

      • Reduced formation of new “connections” between neurons

    Knuesel et al. Eur J Neurosci., 11:4457-62 (1999); Vaillend & Billard, Hippocampus, 12:713-717 (2002); Kueh, Head, Morley, Clin Exp Pharmacol Physiol. 2008 Feb;35(2):207-10

    Neurocognitive skills
    Neurocognitive Skills

    • Increased risk for weaknesses in:

      • Language development

      • Short-term memory

      • Social reasoning (perspective-taking, social judgment, “reciprocity”)

      • Executive functioning (flexibility, planning, organization, insight)

      • Attention/Impulse Control

      • Fine Motor Skills and Motor Planning?

        Cotton, Voudouris, Greenwood 2001; Hinton, De Vivo, Nereo, Goldstein, Stern 2000; Hinton, De Vivo, Nereo, Goldstein, Stern 2001;Cyrulnik, Fee, De Vivo, Goldstein, Hinton 2007; Hendriksen, Vles 2006; Hinton, Nereo, Fee, Cyrulnik 2006; Cotton, Crowe, Voudouris 1998; Wicksell, Kihlgren, Melin, Eeg-Olofsson 2004; Donders, Taneja 2009

    Learning disorders
    Learning Disorders

    40% may have a learning disorder despite normal intelligence.

    Dyslexia: Difficulty learning to read

    Dyscalculia: Difficulty learning mathematics

    Dysgraphia: Difficulty with writing

    Signs of dyslexia
    Signs of Dyslexia


    • Difficulty with:

    • Rhyming

    • starting/ending sounds

    • letter names/sounds

    Elementary + Beyond

    • Difficulty with :

    • “Sounding-out” words

    • Spelling

      •Guessing at words

      • Dropping word endings (“slow” instead of “slowly”)

      • Slow, laborious, or “dysfluent” reading

    Signs of dyscalculia
    Signs of Dyscalculia

    • Difficulty understanding

      • Math concepts

      • Math procedures

      • Memorizing basic math facts

    Signs of dysgraphia
    Signs of Dysgraphia

    • Fine Motor

      • Writing is hard to read, sloppy

      • Difficulty staying within space/lines

      • Slow writing

    • Mechanics

      • Spelling problems

      • Errors in grammar (sentence structure) or syntax (meaning)

      • Forgets to capitalize, punctuation errors

    Signs of dysgraphia1
    Signs of Dysgraphia

    • Sequencing/Organization

      • Difficulty with:

        • thinking of what to write

        • knowing where to start

        • sequencing thoughts

      • Rambling statements that don’t fit together

      • Misses main point

    Neurobehavioral disorders in dmd1
    Neurobehavioral Disorders in DMD

    Signs to look for:

    Attention-deficit disorder: 12% – 30% in DMD

    (with or without hyperactivity-impulsivity)


    Blurts things out




    Easily frustrated

    Too loud

    Hendriksen & Vles 2008; Poysky & Lotze, 2008; Hinton et al. 2006

    Avoids work

    Overly focused on fun

    Easily distracted

    Messy and disorganized



    Difficulty following directions

    Neurobehavioral disorders in dmd2
    Neurobehavioral Disorders in DMD

    Oppositional, argumentative, & explosive behavior:

    52% of boys with DMD?

    Hard-headed, gets stuck on things

    Rigid expectations

    Difficulty adjusting to unexpected outcomes

    Difficulty controlling anger

    Blames others

    Difficulty anticipating consequences

    Doesn’t learn from mistakes

    Punishment escalates behavior

    (Poysky, Hodges, Lotze – unpublished data)

    Neurobehavioral disorders in dmd3
    Neurobehavioral Disorders in DMD

    “HANGRY” = Hungry + Angry

    • Angry

    • Irrational

    • Mean/aggressive

    • Emotionally sensitive/labile

    • Don’t feel hungry

    • Rapid return to happy/normal mood

      after eating food

    Neurobehavioral disorders in dmd4
    Neurobehavioral Disorders in DMD

    Increased risk of:

    • Anxiety

      • Worries

      • Excessive fears

      • Avoids new situations or people

      • Fearful of being alone

    • OCD

      • Rituals and excessive routines

      • Very particular about things being even, lined up, etc.

      • Repetitive behaviors

      • Intrusive thoughts/images

        Hendriksen & Vles 2007

    Neurobehavioral disorders in dmd5
    Neurobehavioral Disorders in DMD

    Autism: 3-19%

    Signs to look for:

    • Delayed language development

    • Excessive and unusual interests/obsessions and routines

    • Impaired understanding of social interactions

    Wu et al. 2005; Hendriksen & Vles, 2008; Darke, Bushby, Le Couteur,

    McConachie, 2006; Hinton et al 2006

    Treatment recommendations

    Treatment Recommendations

    Bushby K, Finkel R, Birnkrant DJ, Case L, Clemens P, Cripe L, Kaul A, Kinnett K, McDonald C, Pandya S, Poysky J, Shapiro F, Tomezsko J, Constantin C, DMD Care Considerations Working Group. The diagnosis and management of Duchenne muscular dystrophy – part 1. Diagnosis, pharmacological and psychosocial management. Lancet Neurology 2010;9(1):77-93.

    Treatment recommendations1
    Treatment Recommendations

    • Effective treatment options!

    • Same interventions as non-DMD kids.

    • Early interventions work best.

    Treatment recommendations2
    Treatment Recommendations

    Recommended testing/assessments

    • Neuropsychological/developmental

      • All kids with DMD at diagnosis or prior to starting school

    • Language and autism

      • If concerns arise

    • Emotional/coping screening

      • Annually, in clinic

    Treatment recommendations3
    Treatment Recommendations


    • Parental behavior management training

      • Noncompliance, disruptive behavior, temper meltdowns

    • Individual therapy

      • Low self-esteem and depression, anxiety, obsessive-compulsive disorder, coping

    • Group therapy

      • Social skills deficits

    • Applied Behavior Analysis

      • Autism

    Treatment recommendations4
    Treatment Recommendations

    Social interventions

    • Promoting patient independence and self-advocacy

    • Talking about DMD: child, peers, teachers, etc.

    • Developing interests and staying involved!

      • Modified/adapted sports, summer camps, and youth groups/programs

      • Art groups, equestrian, and aqua therapies, use of service dogs, nature programs, and internet/chat rooms, etc.

    Treatment recommendations5
    Treatment Recommendations

    Educational interventions

    • Learning needs

    • Behavior needs

    • Modifying potentially harmful activities (physical education)

    • Saving energy

    • Safety (e.g., climbing on playground)

  • Private tutoring

  • Treatment recommendations6
    Treatment Recommendations

    Psychiatric Medication

    • For moderate to severe problems

      • Stimulants for ADHD

      • SSRI’s for anxiety/depression

    Treatment recommendations7
    Treatment Recommendations

    Family interventions

    • Parent mental health

      • Counseling

      • Involvement in DMD community

      • Support network

      • Marital support

    • Sibling mental health

      • Individual attention

      • Connect with other siblings

      • Promote own identity

    Treatment recommendations8
    Treatment Recommendations

    Other therapies

    • Speech/Language Therapy

      • Developmental language delays, articulation problems

    • Occupational Therapy

      • Independent living skills, assistive technology, writing problems


    • More frequent, smaller meals (need to be healthy)

    • Consult with doctor/dietician


    • Increased risk of behavior problems in DMD

    • Multiple factors may contribute to behavior problems

      • Coping/adjusting to DMD

      • Brain functioning

      • Family stress/adjustment

      • Peer/social issues

    • Interventions can help

      • Multiple interventions may be needed

      • Earlier is better


    • Topics for discussion

      • Behavior problems.

      • Talking to kids about DMD.

      • Learning problems.

      • Parent relationships

      • Other?

    • Private conversations OK

    Thank youSMDF and boys/young men with DMD!treat-nmd.euTREAT NMD: Family Care Guidelines Education Matters: Learning and Behavior Guide (Poysky) Psychology of Duchenne (Hendriksen)