Questions and Answers
1. Is there a specific neuropsychological profile associated with DMD/BMD?
Yes. All boys with DMD/BMD have a similar cognitive profile, regardless
of overall level of intellectual function or degree of physical impairment.
This does NOT mean that all boys with DMD/BMD are impaired.
Rather, across the subjects there are characteristic patterns of relative
strengths and weaknesses.
2. What is the profile?
Boys with DMD/BMD are generally weak in attending to and comprehending complex verbal information. That is, when presented with complicated “strings” of verbal information, many children just don’t “get it.” A possible real life example: You say, “Go upstairs, brush your teeth, put your pajamas on and get into bed.” You then go upstairs and find he’s put the pajamas on the bed. You may feel frustrated that he didn’t do as he was told, but he feels he has, because he could only “get” part of what was said and knew it had something to do with pajamas and bed. He can understand the parts of this statement, it’s when it’s all put together that it is just too much.
Moreover, boys with DMD/BMD have difficulty discriminating speech sounds, or phonological processing. Youngsters may make sound substitutions in their speech, have difficulty repeating verbal sounds accurately, and then find learning to “sound out” words in beginning reading particularly problematic. In general, the children with DMD/BMD show reading difficulties similar to those shown in children with dyslexia or reading disabilities, and often cannot tell some sounds apart from others. It’s a subtle, but real, finding that may have big implications for early language development and later reading acquisition.
3. Are there areas that they are strong in?
Yes! Boys with DMD/BMD have numerous strengths. They are strong in learning and rote memory. They also have good visual perceptual skills (that is, they can discriminate visual patterns, recognize incomplete pictures and construct puzzles well). They have excellent vocabularies. They are good at problem solving and abstract thinking.
It’s very important to keep these strengths in mind when thinking about ways to help in school. Building on these may help alleviate lots of learning-related frustrations that these children may have.
4. Will the learning problems get worse over time?
No. Unlike the physical aspects of DMD, the cognitive issues are not progressive. That’s good news. Further, if anything, the cognitive issues improve over time. Language problems that the young children struggle with may be very limiting at age three or four. At ages five and six, children may have difficulty attending to the level of verbal information that their peers do, and that can result in “acting out” or problem behaviors. They also may struggle with learning the basic early reading skills. Once they are seven and eight, they seem to have a much better command of their verbal comprehension. And by nine or ten, the difficulties are much less problematic.
The difficulty comprehending complex language appears to be a delay rather than a deficit, so boys may “catch up” over time. Overall, boys with DMD/BMD appear to be about two years behind where they should be on this skill, yet they are age-appropriate on most other verbal skills.
Be aware, however, that children who do not grasp the basic phonetic aspects of reading when they are first taught it may do progressively worse as school demands increase. Reviewing phonic skills when the child with DMD/BMD is a little older and ensuring the child masters decoding will prevent this.
5. Are there specific behaviors associated with DMD/BMD?
Parents rate DMD/BMD boys as being more immature and having more social problems than their unaffected siblings. Overall, these areas are weakest for DMD/BMD subjects. For some children with DMD/BMD (but not most) these problems are severe enough to fall on the autism spectrum of behavioral disorders, as they impact both on language and reciprocal social skills. More children with DMD/BMD have autism spectrum disorders than expected in the general population, yet most have only mild behavior issues that do not warrant diagnosis. Some researchers have suggested the children have more attention deficits than their peers. Some have also suggested the boys tend to be more depressed than other children, yet more recent findings have indicated that boys with DMD/BMD do NOT rate themselves as being more depressed than their siblings.
6. If there is an UNDERLYING difficulty in comprehending complex language, how does everything fit together?
This is evidence that the missing dystrophin products in the brain likely contribute to the development and performance of specific cognitive tasks.
Language skills in general may be slow to develop. Parents are more likely to report delayed speech in boys with DMD/BMD than their siblings.
Overall on verbal tests, DMD/BMD subjects tend to do more poorly. Previous studies have reported lower verbal IQ than nonverbal IQ. Yet, their single word comprehension is strong. Tests that DMD/BMD children do poorly on require attending to long strings of serially presented information.
Parents rate their children as “immature” or having “attention problems.” Maybe these children are immature in that they don’t understand as much as other children their age. Maybe they’re inattentive in that they can’t follow complicated instructions.
School instruction generally relies on the ability to listen to and understand complex instructions, so poor comprehension of directions could impact on all areas of academic achievement.
DMD/BMD kids are better at sight reading than “sounding out.” The mechanics or “sounding out” requires many steps - associating the sound with the letters and then stringing the sounds together to derive the word meaning. Maybe the skills necessary for this are similar to those necessary for understanding complex instructions.
What can we do?
1. Be AWARE that your child may have trouble
understanding complex language. Although at times it may
feel as if he is not “minding” you, he may just not understand.
This has potential to lead to irritation on your part and frustration on his. Watch out
2. Try to present information in short, concise statements. Take things in a step-by-step approach. Try not to be complicated.
3. Use contextual information, like gestures and pictures, to get your points across.
4. If he seems frustrated or withdrawn, try to determine if it’s because he just doesn’t follow. Encourage him to speak when he’s unsure. Many kids act out or withdraw into themselves once they realize they don’t “get” everything. Try to stop that before it happens
5. Use visual aides in all aspects of schooling. Some parents report that index cards with names of objects written on them placed on those objects (like “lamp” stuck on a lamp, and “door” on the door) help kids learn to sight read. They visually associate what the word looks like with the object.
6. Don’t push learning to read if your son is not ready. Consider keeping him back a year in school to allow him more time to develop the underlying abilities that he may need to avoid making him overly frustrated. It may be helpful to review or teach phonics at a later age when he will be more able to learn the necessary skills.
7. Try to find things that he enjoys doing and emphasize those Art classes, computers,
One way we feel the work has already benefited is in the completion of the Parent Project sponsored publication An introduction to education matters for parents and Learning and Behavior in Duchenne Muscular Dystrophy for parents and educators.
Pat Furlong and James Poysky of PPMD organized a workshop to focus on behavioral concerns in children with DMD. This workshop resulted in a document beneficial to all children and families affected with DMD. This is a compendium of information about the nature of the learning problems in DMD and suggested ways to intervene and help children who have these difficulties. They brought together an impressive group of specialist in reading and behavioral interventions and introduced them to our work and the problems commonly observed in boys with DMD. The result is a clear guide that may be useful to you. Click on the link above and you will be able to download the toolkit for personal use.
Dr. Hinton along with Dr. Edward Goldstein of Children’s Hospital of Atlanta have recently published a chapter on Duchenne Muscular Dystrophy in the book Neurogenetic Developmental Disorders: Variation of Manifestation in Childhood edited by Michele M.M. Mazzocco and Judith L. Ross.
Dr. Hinton also published two chapters on the Dystrophinopathies in the Handbook of Pediatric Neuropsychology edited by Andrew Davis, and Cognitive and Behavioral Abnormalities of Pediatric Diseases edited by Ruth D. Nass and Yitzchak Frank.
Feel free to click on the book title to review the chapter and find out more information about DMD.
DMD Project at Columbia University