• Wed, May 24 2006

Chiari Malformation

In Chiari Malformation (also known as Arnold Chiari Malformation, ACM), the cerebellum protrudes into the spinal canal. According to the Pediatric Neurosurgery unit of the Columbia-Presbyterian Medical Center’s Neurological Institute:


Essentially there is extra cerebellum crowding the outlet of the brainstem/spinal cord from the skull on its way to the spinal canal. This crowding will commonly lead to headaches, neck pain, funny feelings in the arms and/or legs, stiffness, and less often will cause difficulties with swallowing or gagging. Often the symptoms are made worse with straining. When the diagnosis is suspected the study of choice is an MRI scan.

Mary Beth Palo’s autistic son was found to have Chiari 1 Malformation. Since surgery on April 28, 2004,

Brett is a changed child. His personality has blossomed, his language has exploded, his sleep issues are all gone, his stims and tantrums have disappeared, his OCD and anxiety are almost non-existent. While we still see some hyperactivity and some small issues, we feel that this was a miracle for us. The surgery truly was our answer to Brett’s autism. He continues to make daily improvements. He is no longer on any medication or supplements.

(A full account by can be read at Brett’s Story-Not Just About Autism.)

Ms. Palo notes that she has met more than a few parents of autistic children who have the Chiari 1 Malformation. (Charlie, for the record, does not have it.) Ms. Palo is also the creator of the Watch Me Learn series of videos. According to her website, her videos have “already helped thousands of autistic children learn everyday life skills and build the foundation for continuing development.” In describing How It Began, she notes that, in 2005, four years after she had made her first video for her son,

Brett is talking up a storm, socializing and just being a kid. He is now one of the happiest children I have ever seen and is in a mainstream class in the local school district.

Though her account of Brett’s Story-Not Just About Autism attributes the changes in her son to the surgery for Chiari 1 Malformation, she does not mention this additional neurological condition and its major significance for her son on the Watch Me Learn website. Ms. Palo presented on Video: A powerful teaching tool for the visual learner at the 2005 AutismOne conference and will be presenting again at the 2006 conference on May 27.

I hope she will clarify whether it was the surgery or her videos that have been the “answer” to her son’s autism.

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  • http://comautworld.blogspot.com Jannalou

    I have a friend – older than I am by a year or two – who recently had surgery to correct her Chiari Malformation. She didn’t have a lot of problems from it growing up, but surgery was deemed necessary at this point because she’d started doing things like blacking out while driving.

    But she is a very high-maintenance person. I have to limit my contact with her because of the mood swings and negativity that seems to pervade her every thought. And I wonder how much of that is due to her not having had the surgery when she was small. I wonder, now, what her life might have been like.

    More of the “What If…?” game…

  • http://www.autismvox.com Kristina Chew, PhD

    Thanks for sharing about your friend—had she known she had Chiari Malformation as a child?

    What if is an endless game, indeed.

  • http://comautworld.blogspot.com Jannalou

    I don’t know if she knew when she was small or not. She only told me about it when she started having trouble and the surgery was scheduled.

    Her surgery was on May 4; she went back to work yesterday. (She’s a preschool teacher and has her own school.) I haven’t seen her in months, but every once in a while we get together for a movie and stuff.

  • Mary Beth Palo

    My son Brett has had many answers…. chronologically video was the first answer, biomed treatments were the 2nd, education being the 3rd, chiari surgery being the 4th and again education following through every day.

    There is not one answer to his remarkable improvements. No question that the Chiari surgery was the major player to Brett’s physical improvements. Without the other components, Brett would not be at the level he is today. He is not cured. Some people may say he is recovered… but he still struggles with issues and we continue to address them with education and video.

    Watch me learn is a tool that is in constant development. The website will be undergoing many changes within the next few months and will be updated accordingly. We have recently focused my efforts on local educational events and continuing to support parents in need. Unfortunately, all of these efforts require a great deal of time which in turn takes time away from not only Watch Me Learn, but family also.

    There is information on the site regarding the chiari surgery along with contact information for WML. Anyone who does contact us will receive the information and help they need. Current and accurate site information will be posted when ready for publication.

  • http://www.autismvox.com Kristina Chew, PhD

    Ms. Palo, Thanks so much for the update about Watch Me Learn and, even more, about Brett. Very good to hear about how well he is doing.

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  • http://club166blogspot.com Club 166

    Chiari malformation is a relatively rare, and potentially fatal malady (my cousin had her two year old die unexpectedly from a non-diagnosed Chiari malformation).

    The types of impairment seen in patients with this are usually things like vomitting, vision changes, and passing out, rather than things like ticcing, OCD behaviors, and problems with social behaviors.

    Since it affects the brain their may be some overlap in symptoms (speech difficulties, muscle weakness, uncoordination), but generally speaking autism and Chiari malformation have little in common.

  • Zaecus

    My personal hypothesis, backed up by a lot of reading, is that we exhibit behavior under stress that can often be described as ‘getting more autistic,’ and the most fundamental forms of stress are those having to do with health.

    If true, this would mean that a good portion of those parents (to avoid an overstatement) who claim their child ‘recovered from’ or was ‘cured of’ autism might have–in addition to any natural development by the child–dealt with the completely separate problem that was causing their child to behave in more identifiably autistic ways. After that, looking at there ‘new and improved’ child, they say the autism is what’s gone, even if someone who isn’t as familiar with the history might look at the child and identify him or her as autistic.

  • http://daedalus2u.blogspot.com/ daedalus2u

    Zaecus, I think you are exactly right. I agree that stress makes everyone exhibit behaviors that can be described as “getting more autistic”.

    The opposite of that is raising NO levels as is mediated by the placebo effect.

  • Becky

    I believe that there can be several issues causing problems that show up on the autism spectrum, not just one. The CNS is a system that has input and ‘loops’ that are so intricately interconnected that it is possible that there is more than one place where the ‘connection’ can be broken causing symptoms and problems, be it chemical, circuitry, or physical malformation.
    I have chiari malformation as do both of my children. When I look at myself and my extended family, I think, “gee, it seems like we’re on the spectrum, but not enough to diagnose.”
    I talk to many people with chiari (and conditions that are coming to light as comorbid in a subset of persons with chiari, including -Hereditary Disorders of Connective Tissue, such as Ehlers Danlos Syndrome (EDS), Marfan’s syndrome, scoliosis and tethered cord or occult tethered cord syndrome (both related to the connective tissue disorders) see below link for info. on this connection-) http://www.spineuniversity.com/new_link_between_chiari_malformation_and_connective_tissue_disorders
    When I talk to others in the same boat, many talk about -the sensory problems they have, lights and sounds in stores, clothing, tag and sock ‘issues’, food textures, etc.; -then there are those who have word find problems and other cognitive issues, memory, etc; -near clinical OCD behavior, but not quite. I have described myself as ‘socially inept’, even before realizing that was a spectrum trait, and I see the same traits in my daughter and other family members. I have a nephew with tics and another for whom they are considering an Aspergers diagnosis.
    I believe the medical community should look more into the connection between chiari and autism that has been found in this subset of children. The connection between the cerebellum and motor issues is fairly well understood, but the effect of cerebellar issues on executive function and affect/emotions is one that needs to be studied more. If there are morphometric(?) studies out there showing cerebellar differences in autisitic children, then why is it so hard to conceive that maybe chiari could be related to autistic traits in some children?

  • Beth

    Hi,
    I just saw this post and it caught my eye since I was not following Autism Vox when this post was active.
    I have a Chiari malformation and a son with AS and ADHD. However, I was completely asymptomatic until I was 32 years old and my symptoms now are very mild (headaches, mainly.) I don’t know if my son has a Chiari malformation but my impression from what I know about Chiari is that it impairs gross motor function for the most part (the areas controlled by the cerebellum.) My son has no gross motor problems or headaches. His official developmental diagnosis says “Cerebral Dysfunction manifesting as Asperger’s Disorder; Attention Deficit Hyperactivity Disorder, Combined Type.” This is a totally seperate area of the brain. How could the two be related?

  • http://www.autismvox.com Kristina Chew, PhD

    @Beth,

    thanks for sharing this—-my husband has ADHD; in high school he was told that he had “minimal brain damage.”

  • kelly

    hi there: I have been in an ongoing battle recently with my sons health. 2 years ago he was diagnosed with tourettes syndrome. he is not classic tourettes but more of the ts plus emotional stuff. he has had a swollen ankle joint for 7 mos which is almost deemed as juvenile arthritis. he recently has had headaches and has been in hospital with neck and spinal problems.he has to take muscle relaxers and codeine to ease the discomfort of pain. he was also born with a hematoma on the back of his head when born. he has a supposed mild form of kiari malformation and his neurologist stated months back it is nothing of great concern. now that his situation has changed I wonder if his kiari has worsened. c/o shooting pains in the back of his head with noted muscular tension and some days he cannot move his neck at all. I think his docs are on the wrong track.
    any suggestions? he is getting a bone scan in a week. will this show what exactly is happening back there?

    any responses greatly appreciated.

    kelly

  • Don

    I am actually looking for some help and information. I was diagnosed with Arnold-Chiari when I was 35. I had surgery at the base of my brain to help relieve the pressure. My issue is that my symtoms have not gone away or diminished. I am now 53 and I have all the standard symtoms that I read about in these blogs. I have total weakness in my left side, my left hand is deformed and my index finger stays curled under all the time. I cannot sense hot/cold on my left side and my left arm has the burning numbness that I have read other people talk about. My sense of balance is terrible, I often get the dizzy spells and recently my short term memory has been suspect. The weakness in my left side is such that I cannot turn a standard door handle with my left hand. The thing is, I work out and exercise about every day and try really hard to stay fit. I have started getting a serious curvature of my back and I get the fatigue that everybody talks about. I am not overweight, as a matter of fact with my muscle loss on my upper body, my wife says I look like someone being starved to death. My question and where I would like some help and advise is, is it to late for me? I had my local neurologist tell me that I was just getting old! Are there examples where people have overcome some of this stuff? My biggest complaint is that my back is starting to curve badly and I am starting to look funny, I also have a constant pain in my left side that I know is related to the curvature. Got any ideas?

  • Becky

    Don,
    go to the link in my post above:
    http://www.spineuniversity.com/new_link_between_chiari_malformation_and_connective_tissue_disorders
    the journal article they discuss is pioneering. it shows a definitive link between connective tissue disorders and chiari malformations. The doctors who discovered this are the doctors at The Chiari Institute on Long Island (NY) and doctors at the National Institutes of Health. TCI is where many people go who had decompressions and either had no relief or only experienced temporary relief before their problems came back.
    what you’re describing to me sounds like familiar to a lot of what I read on the WACMA chat group and some of what I deal with. If I were to venture any guesses in your situation based only on what you’ve written, I would suspect you have a Tethered Spinal Cord (possibly Occult/hidden that ends where a normal cord should but is none-the-less tethered). This often causes scoliosis (the spine is trying to shorten the distance between the top and bottom in order to reduce the tension on the spinal cord, etc). This can often also cause syringomyelia or syrinx which is a CSF filled cavity in the spinal cord. A syrinx can also cause a myriad of problems including dystonia (abnormal muscle posturing), burning sensations, inability to sense temperatures, and so on. Just because you had a decompression, even if it was a ‘good one’ does not mean that you cannot still have chiari. that would likely explain your balance issues and possibly the weakness too (could be syrinx as well).
    do not give up hope at such a young age, there’s no reason. go to the yahoo wacma group, get on there and the people there will help you understand more and find some of the few doctors (neurosurgeons) out there that fully understand the complexity of the whole syndrome, not just the base of the brain and that’s it.
    best of luck and maybe i’ll catch you over at WACMA.

  • Ms. Clark

    Don, if I were you I’d see another neurologist and then another one to get a third opinion if the second one didn’t have any ideas.

    You also should see an orthopedic doctor, a spine specialist about your back.

    My kid had the Arnold-chiari decompression years ago. I totally don’t know if this could be right or not, but I would wonder if there is some kind of damage related to the decompression surgery that you are experiencing now. Not that you shouldn’t have had the surgery, but maybe there are bad side-effects of the surgery long term?? Again, I’m not a doctor, don’t take my word on anything medical, but I’d talk to specialists until I found one who seemed to be willing to help you more and give you a better explanation than “you are getting old.”

  • Ms. Allen

    My 13 dd had decompression surgery 9 mths ago. She is not autistic however has been diagnoised with ADD and a learning disability. Since surgery, her teachers can not believe that she is the same child. No ADD meds and she is catching up like wild fire.

    Her Chiari symptoms were headaches, body aches, extreme fatique, numbness and ringing in the ears. Memory issues started about age 8. Her CM was 7mm.

    Can’t tell you how it all works or adds up….just know that it changed her life in every sense of the word. For once, she is loving school.

  • Becky

    Ms. Allen,
    I would be very interested in hearing more about the difficulties your daughter had, the type of learning disability, if she had problems with activation/’motivation’ as well as distractability and memory and if she had sensory issues that improved after decompression.
    thank you,
    -Becky

  • http://daisymayfattypants.blogspot.com Emily

    My youngest son has a Chiari I malformation, but it is only 4mm, which according to the literature is “borderline” in clinical significance. His ped neuro dismissed it out of hand, although the same doctor had sent my son for the MRI in the first place for unilateral hyperreflexia, delayed motor development, retained reflexes, etc., and that’s how they found the Chiari. The literature indicates that as a person ages (my son was about one year old at the time), the protusion into the foramen magnum can become shorter, so I’m hoping that’s what will happen in his case. We’re probably due for a follow-up MRI, but he responded SO poorly to the sedation that we’re hesitant.

  • Yvette

    My son has been diagnosed with ASD at the age of 4. My mother has Chiari 1 Malformation I was diagnosed with a slight case of Chiari Malformation. My mother had the surgery at the age of 54.
    I am concerned that my son has inherited this my question is how do I request an MRI without sounding like a crazy mom?

  • http://www.autismvox.com Kristina Chew, PhD

    @Yvette,

    Hi—-what have your son’s doctors said when you’ve brought this up? Are there other reasons that an MRI might be helpful?

    I am going to be sure that the mom named Emily who wrote the comment before yours sees your comment—she is a scientist and I think she’ll have some helpful feedback. Very best—

  • Yvette

    Hi
    Yes I did bring this up during his evaluation and In the evaluation summary it was suggested that a pediatric neurological consultation should be considered. I remember what the MRI was like i can’t imagine how terrible it would be for him. One of his sensory issues is sounds.
    I don’t want to leave any stone unturned but what could be the chances that he has what my mom and I have?

  • http://www.rettdevil.org Kassiane

    Yvette,

    I bet they can put him to sleep for the MRI. Since you have a strong family history looking for Chiari is flat out sensible, IMHO.

    Now they can play music through headphones during MRIs, too. I’ve had several and that’s helped me loads.

  • http://www.autismvox.com Kristina Chew, PhD

    @Yvette, our son has had two MRIs and been sedated for both. There was no other way he could lie still for that long!

  • http://daisymayfattypants.blogspot.com Emily

    Yvette, our son had an MRI when he was <1 year old and they found a Chiari I (4 mm). The pediatric neurologist referred him for the MRI because of delayed motor skills, lopsided gait, etc. He had to have two MRIs, actually, because they didn’t complete the orders the first time. He didn’t respond well to the sedation (it’s hard to reason with an 11 month old who thinks he can crawl when he can’t), so we chose not to do the sedation the second time. We made him very comfortable and quiet and…he went through all 20 minutes of the study just fine, even though he is very sensitive to noise. They do have earphones, too, to tune in music or whatever, and some offer videos for kids to watch. If you have a pediatric center in your area, be sure to get your MRI done with them because they’re VERY experienced and know the tricks.

    The Chiaris can run in families (but I don’t have one), and there is at least one doc at MD Anderson who has discussed a link between Chiari I and ASDs. I would definitely pursue this avenue. The literature indicates that these tend to shorten with age; my son’s is only “borderline” at 4 mm (3-5 is considered the edge of clinically significant), and we never did anything about it…I expect it eventually to regress upward through the foramen magnum as he grows. But some people have very long extensions that later result in complications such as syrinxes, etc., so you definitely want to get his brain/c-spine imaged, especially given the family hx and the ASD. The sx of these can include language delays and motor problems.

    Please let me know if you have any questions.

    E

  • Donna

    Hi I have a 13 year old DD. She was diagnosed at age 4 with absance seizures. She was diagnosed 4 months ago with a Chiari I malformation after having an MRI due to headaches. We are having a mri of her spine done next week. My question is can she be involved in sports? We have not been able to get a direct answer from the neurosurgeon.

  • anon

    Contact sports are a no-no with chiari. Any sort of whiplash, large G-forces, or head trauma can potentially worsen the condition.

  • rochelle

    My son has recently been diagnosed with ADHD, OCD and ASD, hypotonia, and has recently found that his reflexes never integrated into his nervous system. some of this runs in my family (low muscle tone and asd symptoms in the boys of this generation). He just turned 7 and honestly didn’t notice anything physical until age 5, in fact we had always been told that physically he was well above average. After extensive testing this year, we finally had an MRI as a way to rule out anything else so we could finally get an asd diagnosis and get some help for school. Unfortunately, the MRI came back indicating Arnold Chiari Malformation. They said it was 1.4cm. What I can’t find is where does that put him on the types? Is that small or significant? We have multiple neurosurgeon consultations scheduled this month, but it is very stressful to wait. I am looking for any advice or info. Could this be what’s affecting my nephew’s, too?

  • Cindy

    My two youngest sons have been diagnosed with Chiari I Malformation and have had the decompression surgery. However, they both had complications and had a second surgery days later. The middle child has had a quick recovery and has no side effects. My youngest however has not made a full recovery and still has alot of symptoms 2 years later. My question is does the decompression surgery work after a second time?
    Also, both my children have epilepsy and we have been told the seizures are not contributed to the Chiari so I’m interested in how many diagnosed with Chiari also have seizure disorders. My child also has a very hard time gaining weight do you have any suggestions. As a mother of three with two kids diagnosed with epilepsy and Chiari Malformation what is the chance my oldest child has Chiari Malformation. We have asked for him to be screened however, the pyhsican says if he is not having any symptoms leave it alone ( but we have also been told not to have any more children).

  • Donna

    Cindy,
    My daughter just had the decompression surgery. She also has epilepsy that is not related to the chiari. She had no symptons of the chiari. It was found in an MRI due to headaches that supposedly are also not related to the chiari. With that said she also had a syrinx that was significant. The surgery will hopefully correct this. My point is that even though your oldest son is not showing symptons you can’t possibly know what is going on.

  • http://www.autismvox.com Kristina Chew, PhD

    Thanks to everyone for posting their stories about this—I’d also suggest contacting Emily with questions as she is a great and knowledgeable resource.

  • maryanne

    My daughter Lauren is 9yrs. old diagnosed with a chiari 1 malformation with a very small syrinx…while she has no physical symptoms she is being evaluated for ADD,has been diagnosed with absence seizures..she also has short term memory problems. The neurosurgeons do not think any of these are related to the chiari..They do not think surgery is necessary at this point. I struggle with what is causing all of these symptoms and i always seem to go back to the chiari..Anyone else feel this way. Not sure what to do next.

  • Faith

    My son is 7 years old and was diagnosed with Autism several years ago. He also has Chiari that was found around one year of age, but the dr. did not feel that surgery was necessary at that time. We had an MRI last week (which we were doing routinely, but haven’t in about 3 years) and it has not gotten any better, but no worse either. He doesn’t seem to have any headaches, dizziness, numbness, etc and his motor skills are fine. His autism mostly involves severe language delays and OCD like behaviors. The dr. is suggesting we may want to go ahead and have the surgery as there could be a slight chance it may improve his “autistic behavior”. Obviously he said there is no way to tell if it will help or not, but his Chiari is 12mm which he says is pretty significant. It is all so scary, but I think we are leaning towards having it. Any thoughts?

  • Shelly

    My son now 11 was treated for allergies for several years before being diagnosed with tourettes about two years ago. Now they are wanting to start him on medication for ADD along with his tourettes medication due to difficulty learning and retaining lessons in school. I was diagnosed with chiari malformation at the age of 29 after 5 years of being treated for miagraines. I had surgery on 2002 and still ahve symptoms but my questions his could his tourettes and ADD be caused by chiari malformation? They know that I had this wouldn’t they want to test him also!!

  • http://www.autismvox.com Kristina Chew, PhD

    I’m just struck by the number of comments here—-I will alert readers to your questions.

  • D. Evans, RN

    I am an active. “healthy” 38 y.o. female recently dx’d with Chiari. I have a 14 year old son who is moderately-severely impaired secondary to Autism. Thanks to all of those who took time to comment here. Now I am going to have our Pediatrician refer us out for a MRI for my son. I know how miserable I have been with the headaches, et al. I have even experienced several episodes of SVT(Supraventricular Tachycardia) and was put on antihypertensive agent prior to the dx.! It is a very dangerous condition that very often is misdiagnosed or simply never found. Finally a MRI for headaches revealed the source of many of my problems and may have saved my life! I would hate to think my son, or any other person who cannot communicate effectively, is or will ever have to endure sx of Chiari in silence! Thanks again!

  • Rhonda

    I just ran into this thread on Christina’s site, as I was searching for a link between autism and Chiari. My daughter has autism, and I was found to have a Chiari in 2005, and just found out two days ago that it is causing a posterior blockage of the csf flow and that I have to have brain surgery in December. I also have a brother with Tourette’s. As I started reseraching the Chiari, I became aware of a number of people with Chiari who also have children with autism. I myself had many characteristics that could place me somewhere on the spectrum. Now I think I’m going to look into getting my daughter an MRI, which we’ve been hesitant to do as she will require sedation.

    Thanks again for your work, Christina.

  • Jenny

    I first read this site 2 years ago… My autistic son has Chiari –mild… but also seizures, grand mals and absence and its probably from Juvenile Myoclonic Epilepsy… and I have just been Dx’d with JME and a mild Chiari as well…

    My “Hmmmmmmmm” meter is pegged right now. Lot’s to think about and consider.

  • http://www.autismvox.com Kristina Chew, PhD

    @Jenny,

    Lots seems an understatement—hope things are all right. Thank you for posting about your family—very very best—-

  • Lori

    I was diagnosed with a syringomelia and a mild case of Chiari in my twenties after experiencing ongoing headaches and sudden complete numbness in both arms. The numbness and headaches thankfully went away. Now 15 years later, my 10 year old has been exhibiting escalating signs of Tourette’s over the last few months. After reading the comments on this website I am going to request her pediatrician order an MRI.

  • Diane

    I have a friend whose son needs to have the surgery. She is a single mom with Multiple sclerosis and diabetes. she had to close her business because the schools are no longer able to handle her son. He is about 13 and he has autism and has been diagnosed with Chiari malformation. This single mom, also has 3 other children. She needs financial help to get the surgery as they have no insurance. Does any one know who I can send her to for help.
    Her son is getting very violent and unmanageable. At a younger age the single mother had to institutionalize him because she was a single mom with twin babies and he was going to hurt them and she was going to lose all of them, if he stayed in the home. She put him in a state owned institute. He was malested and she was told she could not get him out- that he was the property of the state- she has been through so much and her son deserves a chance to recover from his painful existence. I pray someone who has some contacts will contact me, so that I can help this beautiful person in dire need of assistance.