The corpus callosum — the largest white-matter structure in the brain — connects the left and right hemispheres. Agenesis of the corpus callosum (AgCC) is a condition in which the structure is partly or completely missing, severely limiting integration of left-brain and right-brain function.
AgCC is rare, occurring in just one in 4,000 individuals. It may occur alone or with other cerebral abnormalities. Approximately three to five percent of children diagnosed with neurodevelopmental disabilities have AgCC. Up to 75 percent are male; however, AgCC is also a feature of Aicardi’s syndrome, a severely disabling genetic disorder that occurs almost exclusively in females.
Symptoms of AgCC may range from mild to severe. Children may demonstrate normal intelligence with some compromise in specific skills, or they may be hydrocephalic, have substantial retardation, and suffer seizures and spastic episodes. AgCC is also associated with physical malformations, including midline facial defects.
Neuroanatomically, AgCC will neither improve nor worsen, and it is rarely life threatening. However, it is common to see an increase in symptoms as an individual with AgCC moves from childhood into adolescence. This change is due to their peers’ improvements in skills such as abstract thought and social insight, and their own relative impairments in these areas. Treatment usually consists of learning to deal with specific symptoms and managing seizures if they occur. Although the challenges of living with AgCC are often significant and lifelong, patients are frequently able to lead meaningful and productive lives.
Because AgCC is rare and is associated with a wide and varying range of symptoms, patients frequently are not diagnosed, or they may be incorrectly diagnosed with ADHD, Asperger’s Syndrome, psychiatric disorders, or other disorders. Accurate diagnosis of abnormalities of the corpus callosum, including AgCC, requires a brain scan (preferably an MRI).
Developmental Problems in AgCC
Developmental milestones such as walking, talking, and reading are typically delayed to a varying extent in children with AgCC. Motor skills, especially those involving left-right coordination (for example, swimming or tying shoes) may be a particular challenge. People with AgCC may have heightened sensitivity to some sensory cues such as certain types of touch or food textures, yet they typically have a high tolerance for pain.
Development of young children with AgCC may be similar to that of other children; however, social or problem-solving skills typically do not improve as they go through elementary school. Normally, the corpus callosum matures and becomes more efficient as children approach adolescence, allowing abstract reasoning, problem solving, and socialization to develop rapidly. This does not occur in children with AgCC; consequently, they fall behind their peers mentally and socially as they proceed through their teens and into adulthood.
People with AgCC may be oblivious to the social consequences of their own behavior and typically do not pick up on the social cues of others. They may fail to comprehend jokes or figures of speech, tending instead to interpret communication literally. They are also not as effective as their peers in recognizing emotion in people’s faces, and they are often extremely gullible.
Caring for People with AgCC
Even patients in whom the corpus callosum is totally missing develop a rudimentary compensatory system, providing them with some interaction between the left and right brain hemispheres. Starting a child early with behavioral and cognitive training can help to maximize his or her abilities.
Neuropsychological testing is a necessary tool for evaluating a patient’s cognitive skills. Each patient has unique needs, so it is important to work with teachers, specialists in learning disabilities, and other professionals to develop an Individualized Education Program (IEP). Because of their deficits in social and cognitive skills, patients must be educated about their condition and given consistent direct feedback. Constant repetition is essential for them to learn new material.
It is also very important for caregivers to network with each other. Networking provides a means of sharing ideas and experience, and also establishes a way to coordinate efforts to raise awareness of AgCC throughout the community and health and educational systems.
The National Organization for Disorders of the Corpus Callosum is a nonprofit corporation established in 2002 by professionals, parents, and individuals with corpus callosum disorders. Their mission is to enhance the quality of life and promote opportunities for individuals with disorders of the corpus callosum and raise the profile, understanding, and acceptance of these disorders through research, education, advocacy, and networking.
For more information on AgCC, visit the National Organization for Disorders of the Corpus Callosum Web site at www.nodcc.org.
October 4th, 2007 at 6:03 pm
Wow, this is an amazing story! I can’t imaging what it is like to know you have a missing part of your brain, but you can;t do anything about it!
October 9th, 2007 at 10:23 pm
My little boy boy was diagnosed at 5 yrs. old with c-ACC. He is doing amazingly well. He is in 5th grade this year. Keeping up with curriculum. Reading above grade
level.
Thank you to all of the people involved in this research and getting the word out.
October 10th, 2007 at 7:54 pm
My son is 6 and was diagnosed with c-ACC at one day old! I am so psyched that this is going to be addressed on TV!!!
We have an ACC awareness website on myspace for anyone who interested in learning more about ACC:
http://www.myspace.com/ACC_awareness
Thank you to PBS for including ACC in your show!
October 11th, 2007 at 1:33 am
Yes reitition is vita for those of us with C-ACC! I feel theatre was my saving grace in life. I reccomend it to any of you parents whose child shoes deficits in memory! It worked for me and I have C-ACC!
October 11th, 2007 at 1:11 pm
I have a precious grandson that was diagnosed with ACC before he was born. At 6mos gestation, my daughter had an ultrasound and that is when it was detected.
He is a sweet beautiful baby, and is doing so well at 14 mos.
Keep up the wonderful job in your research.
October 13th, 2007 at 8:04 am
I have a 6 yr old son who was diagnosed at 1 day old. At my 37 week ultra sound was the first time anyone found anything wrong. We were told we were having a hrydocephalus baby, and went thru hell the last 10 days of pregnancy,with Dr appts, traveling 2 hours to IU med center,etc. I am so glad to see this brought into a public forum. Society in general has never heard of it, so you have to explain it every time. People unknowingly can ask some questions that can be very hurtful to the parents and child. But I know it is purely do to the fact of ignorance about acc. As parents we learn to be our childs biggest advocate.
Thank you for bringing into the light so to speak!
October 13th, 2007 at 6:55 pm
I am a mom of a 2 year old son with ACC and I want to thank you all for sharing your stories.
October 13th, 2007 at 9:17 pm
On behalf of our family and our ACC child, I thank you for this work. Will it be shown soon outside New York? This link is great for sending to friends and relatives who would like to know more.
October 17th, 2007 at 12:12 pm
I couldn’t thank you enough for the research done, this web site, and the program! My 8 year old son was diagnosed with P-ACC at age 3. I am passing this site on to friends and family and educators. Is there a way to get a copy of the program that aired in NY? I am very excited that people will become more aware of this dysfunctional brain disorder. Thanks again!
November 19th, 2007 at 9:10 am
when will this show air? does anybody know? I don’t live in NY, so I want to know when in January is the actual show?
November 24th, 2007 at 6:04 am
[...] of online TV episodes have been launched by a site called Curious and one of them caught my eye: What is: Agenesis of the Corpus Callosum? On the site you can see a ‘taster’ video which seems to be relevant to Sperry. The [...]
December 11th, 2007 at 1:25 am
Just found this. Looking forward to people being able to watch and learn. At 32 weeks, my son was also thought to be born w/ hydrocephalus. The day he was born, C-acc was discovered instead. He is almost 8 now!
December 26th, 2007 at 10:06 pm
My daughter was diagnosed with hydrocehpalus at 28wks, after she was born it was determined she had ACC. She’ll be 7 in 2 days and there’s still so much to learn. My husband and I started seeing some mild facial twitching which we think are seizures so we’re heading back to the docs to get all the necessary check up MRI etc. I’m looking forward to this PBS special.
January 4th, 2008 at 7:16 am
Caught only last part of program. Describes my son. When will it air again in Visalia, California? Please email response at dschurman@sbcglobal.net.
January 11th, 2008 at 4:59 pm
Is it possible to get a copy of the
channel 13 special onagenesis of
the corpus colleseum?
January 25th, 2008 at 4:46 pm
How do people with AGCC respond when asked to describe some unseen object placed in their left hand?
January 31st, 2008 at 6:42 pm
Thanks for sharing
February 2nd, 2008 at 8:43 am
lqgkzpr gjkvrw coyigsmxk nemzfqx ynxktr qxprzt otdkefxlu
February 9th, 2008 at 1:17 pm
I’d prefer reading in my native language, because my knowledge of your languange is no so well. But it was interesting! Look for some my links:
February 11th, 2008 at 4:01 pm
Dear Joe (comment #16) - people with AgCC do not exhibit a disconnection syndrome as seen by the split-brain patients. Thus, a person with AgCC can name an object in their left hand. Best regards, Lynn Paul
February 26th, 2008 at 9:27 pm
Missed the special, is it possible to get a copy of it? My son has C-ACC. Please email response to kkimhen1@aol.com
February 27th, 2008 at 1:42 am
Hello Dr. Paul,
I live in Toronto, Canada. My son is almost 3 yrs old and he’s quite active. He was diagnosed with ACC. There’s not many resources in my area, not that i know of. I myself am a 34 yrl old individual who had also some learning milestones throughout my life:I dislike math immensely, I dislike changes, I’m a bit isolated and shy. I’m raising my son alone though. I’ve been told i am a great mother. Now…i’m scared of the next stage up, putting him in school. For the time being i take him to a drop-in centre and he goes to speech and language. I know more less what ACC is. The community where i live is not aware of what ACC is though.
I don’t know how i will confront the obstacles ahead of us. I even have the fear of not being able to protect my son once he’s not under my wing…
I watched the little video here. I wish there was more i could follow, learn from, and share my experience.
Thank You for your attention,
Loretta
February 29th, 2008 at 7:27 pm
Dear Loretta and others who have been impacted by a corpus callosum disorder. I recommend that you contact the National Organization for Disorders of the Corpus Callosum (www.nodcc.org). They have an annual conference for families, as well as online discussion boards and a family networking program. This is a great way to learn about AgCC and get support.
Best regards, Lynn
March 14th, 2008 at 5:19 pm
I have a daughter who is 9. She has C-ACC and FAS. I homeschool her. Just looking for anyone out there who is doing the same. Alot of things she does, are hard to put into words. For example, she does alot of talking to herself or actually it sounds as if she is talking to someone who is not there. She does alot of oral stimulation with nonverbal words and laughing. In the last year she has regressed in development. So glad I found this sight. Would love to hear from others.
March 23rd, 2008 at 9:51 pm
I’d prefer reading in my native language, because my knowledge of your languange is no so well.
March 26th, 2008 at 4:28 pm
Hi,
My daughter was diagnosed with p-ACC. The lining was thinner and the back portion of her CC was missing. She is 4 years old, and has significant delay in some of her gross motor skills and fine motor skills, but she is at least progressing
She has been in school now for almost a full year and I see so much progress in her social skills as well. I’m so proud of her, and I know that this is not a “death sentence” for her to have a normal meaningful life.
April 11th, 2008 at 6:41 pm
To parents and individuals with CC disorders I’d like to recommend the ACC listserv, operated by the University of Maine. It has many members with a wide variety of experiences, and is a great source of information and support.
June 13th, 2008 at 11:13 pm
Fascinating. I homeschool my AgCC son also and we have managed to minimize a lot of the socialization problems and maximize learning (compared to what was happening in school), but yes, the constant attending to learning is taxing. I agree that one side of the brain probably recognizes fear, surprise, etc. in faces but that the other side of the brain has no knowledge of this and so the child tells you the wrong answer. My son also seems oblivious to mean-ness in a teacher, for example, and tells me it is okay, even when I have seen the teacher (in this case a music tutor) be significantly rude with him. I think that the part of the brain that controls speech is not aware of the rudeness, but that another part of the brain is fully aware but unable to vocalize it. This would explain why some children with AgCC can gradually slide into depression while vocalizing that everything is alright. Please continue to investigate the possibility that these children are not oblivious to slights and insults but that maybe only the side of the brain that is able to vocalize is oblivious. This would make these children in need of a lot more empathetic treatment than they would outwardly indicate is necessary. Keep up the great work, Dr. Paul. We so need this research.
January 25th, 2009 at 7:26 pm
My son is 28 yrs old. He has hydrocephalus, a shunt implanted at age 6. He just had an “episode” and they found his shunt fractured in the c3 area. and revised it. The neurosurgeon told us he had ACC. This is the first time in 23 yrs anyone has every mentioned ACC, or that part of his brain was missing! With all the MRI’s taken over the years, no previous neurosurgeons pointed this out to us. We were saddened but after reading your site information, so many of his behaviors makes sense now.
May 14th, 2009 at 6:57 pm
My grandson is almost 6 with thin corpus callosum. He is walking with a walker, but does not speak. He puts everything in his mouth. Teachers and therapists report that he understands and knows what is going on. My question is has anyone had a child who begins to speak at a much later time.
June 19th, 2009 at 6:05 am
I am 48 and recently diagnosed with ACC. What adult resources are there for me? I see a lot of resources for young children and adolescents, but not for adults.
July 7th, 2009 at 6:04 pm
Mary Sweet,
If you join the ACC-Listserv you will find other adults with ACC on the e-mail support group. There are also many parents of kids who have ACC as well involved on the Listserv. You can join the ACC-Listserv at the site below:
http://lists.maine.edu/cgi/wa?SUBED1=acc-l&A=1
You can also contact the National Organization for Disorders of the Corpus Callosum (NODCC):
http://www.nodcc.org/
Lynnea is an adult who has ACC who recently began ACC-Awareness to help create more awareness about ACC:
http://agenesiscorpuscallosum.blogspot.com/search/label/Adults%20with%20ACC
Sandie *Mom to 15 year old Matthew with complete ACC*
July 9th, 2009 at 1:38 am
My son is almost 12 and my wife and I are very curious on what awaits him in middle school, high school and adulthood. He was complete ACC. He has had an IEP since age 3 and is starting middle school in 2 months. He plays sports and is a really nice boy but really struggles socially and has a tough time making friends. Can you tell me how life was through school and now way into adulthood. What were you diagnosed with?
July 11th, 2009 at 6:21 pm
I just learned my friend’s 14-y-o son has been diagnosed with having an undeveloped CC. He just started having epileptic fits. It makes so much sense now because he has a hard time picking up nonverbal cues, e.g. when others are in a bad mood. At 14, he’s also socially “innocent” e.g. He can be very “touchy-feely” even when it’s inappropriate. But we love him for his innocence and his trusting approach to life and relationships! He hasn’t done tremendously well in school, but his performance is comparable to that of his peers. Thanks for this forum!
July 13th, 2009 at 10:39 pm
I was diagnosed with P-ACC when I was 42 years old. I had a mid line encephalocele removed when I was 7.5 months old. I had trouble with math in school. I have trouble understanding some movies and keeping up with some t.v. programs make me mentally tired and confused. I suffer from migraines that effect my thinking and functioning. The radiologist asked my doctor how I got to the hospital when I was admitted for a migraine. My Neurologist did an MRI during that visit and the radiologist saw the P-ACC. The Neurologist wasn’t intrigued or forth coming about what it was and I had to go home and google it! I later asked the Neuro why the Radiologist asked how I got to the hospital and he said most of the time you have mental retardation and don’t learn to sit up or walk. I feel I suffer daily as I have depression and feel I’m socially different.
July 20th, 2009 at 10:24 am
dgilbert…
For you to wait until 42 to be diagnosed must have been hard with all the questions you might have had, and only now getting part of an answer.
The radiologis that told you “most of the time you have metal retardation and don’t learn to sit up or walk” I believe to have been mistaken. I know there is still a lot to learn about the ACC condition, and MRI’s have changed it’s ability to be discovered and how people are studying and understanding it.
My daughter is 7 going on 8, and is a wonderful, happy, and very loving young girl. She has speech, motor, and social delays. She also likes to “hide” just how much she knows and understands. She’s shy and in public, she’s very reserved and withdrawn. We are so blessed to have her in our lives and are excited and apprehensive about the future. She will have challenges ahead, but knowing there are others out there with the same condition that are adults who might have had struggles, but are making it by… that’s inspiring. It sounds (dgilbert and others) like you’ve been misunderstood for far too long and I’m glad this research is being done, because you have so much to offer to this world. Thanks for writing in and like other’s have said, look at http://www.nodcc.org to see all the activities / groups that are happening to help bring awareness and understanding for this condition.
July 29th, 2009 at 4:06 pm
I have a 4&1/2 yr old grandson who was just diagnosed with this disorder. He cannot speak, but babbles all day long. He knows all objects and the people in his life and is very happy! He received Early Intervention up until 3 which assisted him in walking etc. He has hypotonia of his muscles and fine motor skills are difficult. He is also not toilet trained. Pediatric neurologist advised my daughter that he will never speak and she was devestated-is this true?
August 26th, 2009 at 12:23 pm
My brother did the same thing but is 36 now and talks non-stop. He holds a part time job and lives on his own although his house is next door to me and he has lots of family support. I have a 19 year old step-son with spastic quad cerebral palsy and he uses a communication device to speak. It is a computer made by Prentke Romich called an ECHO-14. There are many different types availble.
August 29th, 2009 at 10:17 am
My sister has ACC as well as aicardi. I am currently looking for a neurosurgeon in the Akron Ohio area to review her and her case. Recently she was diagnosed with a 6cm mass on the midline of her brain, then released from the hospital without her neurolgist comparing her past scans to determine if this was pre- existing or not. I am in desperate need of someone close and competent. PLEASE HELP US!!!!
August 31st, 2009 at 9:56 am
My Grandson is 2 1/2 months old, diagnoised with ACC. He seems fine so far, he is smiling, cooing and trying to stand up in our lap. Is this normal for this type of problem.
October 11th, 2009 at 5:48 pm
I have C-AgCC and I didn’t find out until I was 26 yrs old ( I am now 40), but boy, do I now understand why I couldn’t figure out how you could hold the handle bars of a bike and peddle at the same time or hold the handles of a skipping rope and lift those legs, or even tie my shoes. I can also use both hands for everything! My world is so much different to everyone else’s ( I see things so much differently) and I believe I have more caring emotion and empathy for the human race and peoples feelings than the average, normal person out there. Yer school was a huge challenge, I found I made heaps of friends though. Learning for me was hard….math..what a nightmare! I work and I admit I do find it hard at times. The sight in my left eye isn’t so good and neither is the hearing in my left ear……and repitition, fantastic! Go you AgCCers, we’re unique, embrace it and take it on!
October 25th, 2009 at 1:10 am
my son was diagonosed with complete agcc at age 3 …..mabey all of us would be better off without this part of our brain …i wish i could be more like my son. He is a complete opptimist. I come from a very small town…Jaydn has softend the hearts of the most hurt souls, from a very young age he has reached out to strangers young and old …without fear of rejection…He knows no evil….scary for mom but also very inlightning. He is struggling in math but learning to read now. My worst fear is the bully factor..Jaydns lack of social skills, mainly in self defense, are a target to those children who feel the need to hurt back…I worrie about his future in junior high…..any suggestions…please talk to me.
November 2nd, 2009 at 5:00 am
Hi Dena,
my name is Deone. I am the 1 who left the last message before you. Welcome to the world of Agcc. Just like your son I have complete Agcc, and I too am an optimist. I find too that people have always been comforted by my ways. We have a quiet loving peacefulness about us, and a smile for everyone.Do you find he forgets things? We are who we are and we can’t change that, and I believe god has blessed us with something special. I was bullied to some extent at school and learning was so hard, but I also found I had lots of friends, mind you slightly younger ones. It was hard at school but i hope like me your son can find away to cope with any of the bullying that may occur, it made me into a stronger person by learning to deal with it. I’ve gone on to raise 3 kids of my own and I even go to work. Do his teachers know of his Agcc? Perhaps they can help in some way to keep away the bullies. I went through school thinking I was just dumb and stupid, and my report cards were always, she daydreams, she doesn’t pay attention. I’m there with you and I know the feelings you are going through for your son, just be there for him if and when he needs you. I grew up with the most loving and supportive parents and that nurturing really helps us.God bless you.