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Welcome to the Ask the Professional Corner where each month a professional in the medical, educational or therapeutic field will answer questions relative to their area of expertise as they relate to individuals diagnosed with a disorder of the corpus callosum. Questions must be submitted one month in advance of each topic. We encourage you to submit your questions to info@nodcc.org.
January 2007
Cognition & Neuropsychology
Lynn K. Paul PhD is currently serving as Visiting Associate Faculty at California Institute of Technology, where she is developing an ACC research program. Among her 9 research publications are 7 on the neuropsychology of ACC, particularly focused on cognition, non-literal language, and psychosocial functioning.
Warren S. Brown PhD is Professor of Psychology at the Graduate School of Psychology at Fuller Theological Seminary and Director of the Lee Travis Research Institute.He is actively involved in neuropsychological research, particularly related to the consequences of agenesis of the corpus callosum for intellectual and social abilities.
1. What is your opinion on stem cell replacement relative to the corpus callosum?
It is our belief at this time that stem cell treatment holds very little promise for treating corpus callosum disorders. Stem cell replacement is effective in conditions where the cells were originally intact and thus can be “replaced.” If corpus callosum cells are absent, as in complete or partial agenesis, the brain has organized itself without those connections and there isn’t anything to replace. The brain wouldn’t know what to do with those connections even if they could be introduced.
2. What is neuropsychological testing, what type of results should we expect to receive, and how do we go about getting this testing done for our son?
Neuropsychological assessment is an evaluation of an individual’s cognitive skills across many domains (verbal, visual, learning and memory, organization, etc.) for the purpose of better understanding his or her strengths and weaknesses. It involves multiple standardized tests, most of which involve paper-and-pencil, manipulating puzzles, or giving verbal responses.
Performance is evaluated in three ways: comparison with age-matched peers, comparison of one’s own performance across tests, and through observation of the approach to testing. Although the tests may overlap with those given by in an educational evaluation, a neuropsychological assessment includes a broader range of domains and involves analysis of how performance relates to brain structure and function. A neuropsychological evaluation may include a learning evaluation and academic recommendations if requested. Neuropsychological evaluations must be conducted by a board certified neuropsychologists (ABPP-ABCN) or by a clinical psychologist with post-doctoral training in neuropsychology. Neuropsychologists are Ph.D.’s, whereas a neurologist is a M.D.
A neuropsychological assessment is strongly recommended for children with DCC who are of school age. Prior to school age, developmental assessments by speech-pathologists, occupational therapists and developmental specialists may be preferable. Once at school-age, a neuropsychological evaluation can provide a realistic understanding of the child’s intellectual strengths and weaknesses, as well as current comparison with peers. The neuropsychological evaluation also serves as a baseline for comparison if the child has any later cognitive changes (for example following onset of seizures, following head-trauma, or a change with unknown causes). A neuropsychological assessment may also be helpful for adults with DCC who are facing vocational difficulties or problems coping with daily life.
3. I have a patient with ACC who has many symptoms related to autism. Are there any related articles, review or otherwise describing this relationship?
Autism is a behavioral diagnosis which may involve multiple brain systems. In contrast, corpus callosum disorders are anatomical diagnoses which may involve a broad range of symptoms. Research into the behavior symptoms common in complete ACC show many similarities to those seen in autism, particularly regarding impaired social interaction and social communication. Currently, there is no published literature exploring this comparison. However, we do have a paper under review on this subject.
4. I am a parent of a child with ACC diagnosed prenatal and confirmed with CT after birth. I was advised at the time that it would probably never affect her. She is now 8 and the school has identified that there are certain social skills that they are concerned about. They say she is fine intelligence wise. They are in the process of having her observed and assessed. What can we expect in the future?
The behavioral consequences of corpus callosum disorders often become more evident during later childhood and adolescent years. The corpus callosum in typical children completes development during those years, thus the absence of the corpus callosum becomes more evident as their peers gain new abilities. Individuals with ACC often have difficulty reading social cues such as facial expressions, vocal inflection, and other non-verbal communication. They may also have difficulty interpreting second-order meanings of information, such as the non-literal meaning of a proverb or idiom, or the humorous component of a sophisticated joke.
They tend to take others very literally and may have difficulty determining the importance of a particular interaction (taking a small slight too seriously on one hand and not taking in negative feedback from peers on the other hand). Due to these difficulties, as well as cognitive impairments in abstract thinking and problem solving, individuals with ACC require very concrete training about appropriate social behaviors. They are not likely to learn from subtle peer pressure but instead require direct feedback about their behavior. They may however be very gullible, due to poor understanding of other’s thoughts (impaired theory of mind) making them vulnerable to bullies and direct peer pressure.
There is no systematic research regarding social skills interventions for people with ACC, so the following suggestions are based solely on anecdotes and parent insights. If you have not already educated your child about her condition, it is important to do so now. It may be helpful to educate her peers and teachers as well, so that they may be more understanding and watch out for her. Peers are present at key times when your child may need help, so they are a great resource. It is also wise to establish a pattern of openly discussing social interactions with your child, highlighting cause-and-effect sequences and pointing out cues about what other people are feeling and thinking.
5. My grandson was born with Agenesis Corpus Callosum and is five years old now. I am curious to find out if there is any information on disciplining a child with ACC. He gets spanked often, but it does not seem to help. Are there any studies on this?
Unfortunately, there are no research studies on this issue.
6. My 16 month old was born with mild hydrocephalic and agenesis of the corpus callosum. One of my concerns is that my daughter does not speak but MAMA and DADA. Should I have her checked and who would I see. Her doctor seemed concerned at her checkup and said we would watch it. What is your opinion? We are walking now (14-1/2month) but she does seem very wobbly and has already received stitches. She did not sit up until 11 months. We were in physical therapy but insurance refused to pay any longer since she started to walk. Is there something I can do to fight insurance on this?
Since you know that she has a brain abnormality, it is typically best to get intervention early instead of waiting to see if the delays get worse. It is wise to find out about the developmental evaluation and treatment programs available in your state. Early childhood intervention programs may involve physical therapy, occupational therapy and speech therapy. Your local school system should be able to direct you to the state-funded programs for early childhood/developmental evaluation.
February 2007
Speech & Language
JoAnneTully CCC-SLP and her husband Tim are the parents of a 17-year-old daughter who has complete agenesis of the corpus callosom. She is employed as a speech-language pathologist in the Delavan-Darien school district in Wisconsin.
1. I am a speech-language pathologist working with a first-grader with partial agenesis of the corpus callosum. He speaks in complete sentences and produces most sounds correctly. However, I have just begun working on his prosody. In our first session addressing voicing issues, he was unable to imitate/approximate exaggerated high and low pitch. His mother reports that he does not hum or sing tunes but he does make voices when playing independently with action figures. I have searched our ASHA professional website but did not find any therapy techniques or suggestions. Do you have any resources that might help me?
I don't know of any articles or discussions that have directly talked about prosody in children with DCC. I do know, however, that a lot of children with DCC have some degree of apraxia, and prosody disorders often accompany apraxia. The Apraxia-Kids website has a good article by Shelley Velleman about prosody. The link to that article is http://www.apraxia-kids.org/site/c.chKMI0PIIsE/b.980831/apps/s/content.asp?ct=464243
I think some of the rhythm and other activities sound like they would be fun and possibly successful in working with a child like your student. One thing to keep in mind is that children with ACC require much more repetition than other disabled children when they learn new material. If he seems to be "getting" something you teach him, he really is getting it! But it will take repetition, repetition, and MORE repetition to make sure that skill is established. That will apply to prosody and any other thing you teach him. I will keep your email address handy in case I come across more information about prosody. Good luck with your little student.
2. My daughter just turned 21 years old. At about 12 months, a neurologist stated her corpus callosum seemed narrower than normal and she might have agenesis of the corpus callosum. Another neurologist felt that we should not focus on this label because it would possibly interfere with an appropriate education. Her main diagnosis is spastic-athetoid cp. She is wheelchair bound. Her nonverbal IQ was determined to be normal at age 7 as assessed by a public school psychologist. She is now inconsistently verbal, uses a toughbook computer that is preprogrammed with phrases that she accesses. The phrases consist of recorded speech. She has a history of seizures (from Grand mal to petite mal) that have come and gone. She is now being reassessed for possibly re-occurring seizures. Despite the severity of her disability she picks up info quickly and is very active. What info do you have on young adults with this condition?
I am not too experienced in the area of cerebral palsy, but I have worked as a speech pathologist on a team that treated children with CP who used voice-output augmentative communication devices. My 19-year-old daughter has complete agenesis of the corpus callosum as well as a chromosome microdeletion (1q44). She does not have CP, but she has low muscle tone and uses a wheelchair for mobility. She can crawl from place to place and stand holding onto something, but she is not able to walk -- her balance just has never been good enough. Her cognitive development is quite delayed, although she seems to know a lot more than she is able to express. She can speak in short sentences and communicates her basic wants, needs, and observations pretty well, but her speech is still very delayed. She has a delightful and sometimes-sophisticated sense of humor.
I think our daughters have some similarity in their seizure histories. My daughter had severe seizures (grand mal and other) until she was 5, when a combination of medicines brought them under control. Two years ago she had a 48 hour EEG (with the original intent of possibly weaning her from seizure meds) that disclosed unusual brain activity, even though no seizures were observable in her behavior. The suspected seizure activity mostly occurred during sleep. The doctor put her on Depakote and she is still on it at the present time; it has not caused any
problems for her.
Is your daughter satisfied with the level of communication she is able to achieve with the Toughbook device? Does she get frustrated by it? I am guessing you have been working with an SLP or other professional to get this working for your daughter. The biggest advice I would give you about this is to continually monitor how easy/hard it is for her to use, and not to settle for less than satisfaction. There are SO many alternative devices available and new technology is being developed all the time; there are solutions to almost every problem that comes up. If you have any concerns about her device and its use, please let me know
and I'll try to help you find resources in your area.
I think the biggest way in which your daughter's agenesis of the corpus callosum might affect her communication (and other) abilities will be in the amount of repetition she may need to learn new skills. This is the thing I've seen most frequently in talking to SLPs who work with clients who have ACC. The typical client with ACC requires much more repetition than other clients who have speech and language disorders. A person with ACC has to forge new brain pathways when learning something new, and that requires repetition, repetition, and more repetition.
Our biiggest frustrations with therapists (PT, OT, and speech) who worked
with our daughter over the years were that they expected her to retain what
they had taught her after only a couple of practices. Sometimes she really DOES perform a brand new skill beautifully -- even a couple of times -- but that does not mean it is "established" in her brain yet. When they try to have her do it the next time, she appears to have lost the skill. It's not really lost -- it is just not fully
established yet.
One other area in which ACC may affect your daughter may be in her pragmatic language development -- the skills of knowing when to ask questions (or stop asking questions), answer questions, "read" social cues. With a voice-output device, the timing of social responses is necessarily different, but the basic social/pragmatic skills are still important. I don't know if your daughter has trouble reading social cues, timing her comments and questions, or understanding body language, but if she does this may be a characteristic of her ACC. An SLP may be able to help her work on skills in these areas.
March 2007
Neurology
Elliott Sherr MD, PhD is an Assistant Professor in Neurology and Pediatrics at UCSF. He directs the Brain Development Research Program, a group that studies the genetics and biology epilepsy and disorders of cognition, more specifically with a focus on disorders of the corpus callosum, including Aicardi syndrome.
April 2007
Early Intervention PT & OT
Professional TBD
May 2007
Social Security & Government Services
Stuart Gilkison
June 2007
IEPs
Richard Chamovitz
July 2007
Inappropriate Behaviors - Teens and Adults
Lynn K. Paul PhD and Stacy Everson PhD
August 2007
Feeding, Toileting & other Milestones
Professional TDB
September 2007
Social Skills & Behavior Modification
Terri Naramor PhD
October 2007
Seizures, Shunts & other Neurological Issues
Elliott Sherr MD and John Bodensteiner MD
November 2007
Educational Strategies
Donna Rosenbery PhD
December 2007
Transitions to Adulthood
Professional TBD
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