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
 DMD and brain functioning
 Cognitive skills and learning problems
 Neurobehavioral disorders
 Treatment recommendations
Behavior Concerns in DMD
DuchenneConnect (preliminary data that may change before publication)
Learning Concerns in DMD
DuchenneConnect (preliminary data that may change before publication)
Potential Causes
 Psychological
 Coping with DMD
 Psychosocial Factors
 Family stress/conflict
 Peer interactions
 Teachers/adults
 DMD impact on brain
functioning
 Medical factors
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Steroids
Fatigue/sleep
Medical procedures
Blood sugar
COPING WITH DMD
How are the boys coping?
 Same as boys with other chronic medical conditions
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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 DMD
 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
Social Problems: 34%
•Immaturity
•Overly dependent
•Social skills deficits
•Social anxiety
•Teasing/bullying
•Peer inclusion
Hinton, Nereo, Fee, Cyrulnik, 2006
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
 Full-length dystrophin
 cerebral cortex
 sub-cortical structures
 Cerebellum
 Smaller isoforms
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
 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
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
Preschool
Elementary + Beyond
• Difficulty with:
• Difficulty with :
 Rhyming
 starting/ending
sounds
 letter names/sounds
 “Sounding-out” words
 Spelling
•Guessing at words
• Dropping word endings (“slow”
instead of “slowly”)
• Slow, laborious, or “dysfluent”
reading
Signs of Dyscalculia
 Difficulty understanding
 Math concepts
 Math procedures
 Memorizing basic math facts
Signs of Dysgraphia
 Fine Motor
 Mechanics
 Writing is hard to read,
 Spelling problems
sloppy
 Difficulty staying within
space/lines
 Slow writing
 Errors in grammar
(sentence structure) or
syntax (meaning)
 Forgets to capitalize,
punctuation errors
Signs of Dysgraphia
 Sequencing/Organization
 Difficulty with:
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thinking of what to write
knowing where to start
sequencing thoughts
 Rambling statements that don’t fit together
 Misses main point
Neurobehavioral Disorders in DMD
Attention-deficit disorder: 12% – 30% in DMD
(with or without hyperactivity-impulsivity)
Signs to look for:
Impulsive
Blurts things out
Interrupts
Impatient
Fidgets
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
Forgetful
Daydreams
Difficulty following directions
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 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 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 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
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 Recommendations
 Effective treatment options!
 Same interventions as non-DMD kids.
 Early interventions work best.
Treatment Recommendations
Recommended testing/assessments
 Neuropsychological/developmental
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All kids with DMD at diagnosis or prior to starting school
 Language and autism
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If concerns arise
 Emotional/coping screening
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Annually, in clinic
Treatment Recommendations
Psychotherapy
 Parental behavior management training
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Noncompliance, disruptive behavior, temper meltdowns
 Individual therapy
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Low self-esteem and depression, anxiety, obsessive-compulsive
disorder, coping
 Group therapy
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Social skills deficits
 Applied Behavior Analysis
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Autism
Treatment Recommendations
Social interventions
 Promoting patient independence and self-advocacy
 Talking about DMD: child, peers, teachers, etc.
 Developing interests and staying involved!
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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 Recommendations
Educational interventions
 Learning needs
 Behavior needs
 Modifying potentially harmful activities (physical
education)
 Saving energy
 Safety (e.g., climbing on playground)
 Private tutoring
Treatment Recommendations
Psychiatric Medication
 For moderate to severe problems
 Stimulants for ADHD
 SSRI’s for anxiety/depression
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 Recommendations
Other therapies
 Speech/Language Therapy
 Developmental language delays, articulation problems
 Occupational Therapy
 Independent living skills, assistive technology, writing problems
Diet
 More frequent, smaller meals (need to be healthy)
 Consult with doctor/dietician
SUMMARY
 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
TOMORROW
 Topics for discussion
 Behavior problems.
 Talking to kids about
DMD.
 Learning problems.
 Parent relationships
 Other?
 Private conversations OK