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Arnold Keari Type 1
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Arnold Keari Type 1

My son, one of triplets, just had a brain MRI done on Wednesday. My husband and I met with our pediatrician who said that J. has a rare brain abnormaility called Arnold Keari Type 1.  She was unable to give us much information since she said that after looking through medical books and on line that she was not able to find much to inform us on.  We will be meeting with a neuro-surgeon on Wed. May 17 and I am very interested in asking the most appropriate questions.  I am also interested in any sites, journals and/or resources that are available to further educate myself on this disorder.  Please help!!!! thank you - lori
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Dear Lori:

The Arnold Chiari malformation is extremely serious as it includes a spina bifida (hole in the spinal column that communicates to the skin, the depression of the cerebellum and brainstem into the spinal column, and usually hydrocephalus.  Is this being diagnosed in utero?  There are currently some centers doing in utero surgeries for this conditions and this may lead to a better prognosis.

If you triplets are already born, and there is no spina bifida, then likely you misunderstood and this is a Chiari I malformation.  This is not that uncommon, and when it is mild, there are usually no, NO, NO, NO neurological consequences.  Sometimes when it is large, it can cause problems and surgery is warranted (respiration problems, intermittent hydrocephalus).  I would wait and see the neurosurgeon or your pediatric neurologist before worrying.  Most likely, in the face of a normal triplet baby, I would think it will turn out to be a minor Chiari I.

Sincerely,

CCF Neuro MD
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thank you for your reply regarding Arnold Chiari malformation.  we will be seeing a neuro surgeon on this coming wednesday.  can you advise me of any specific questions i should ask about.  my first question was in regards to a thinning cerebral cortex which is totally unrelated to this malformation.  could you give me some additional information on what i could possibly expect from this .  thank you - lori
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Dear Lori:

As previously mentioned, there is no such entity as an Arnold Chiari I malformation.  So, you need to clarify the diagnosis.  Second, if your son has a Arnold Chiari, you need to ask questions concerning the meningomyelocele in his back.  Since your diagnosis is uncertain, I would have him/her have the neuroradiologist review the MRI scan.

CCF Neuro MD
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I am sorry that I have been giving you the wrong information.  J. has Arnold Chiari Type 1, not Arnold Chiari 1 as I have been mistakingly typing.  Does this change any thing now as far as your responses go? thank you for all of your help. It really appreciate your quick responses.
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Dear Lori:

Again, there is not such thing as an Arnold Chiari I, it is either Chiari I or Chiari II or Chiari III malformation.  An Arnold Chiari malformation is a Chiari II malformation.

CCF Neuro MD
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this proves even doctors can be anal. they knew what the mother saying but put her in hell to answer it. then the doctor say the chiari malformation type 1 does not cause problem. how would they know they have never had chiari type 1.

thank you, thank you
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Hope you have have had some positive results since your initial
questions regarding your child.  Although I am not a medical
expert, I could be called a well-traveled patient.  The good
news is that a surgical procedure may be available for your child's condition.  However, it would be advisable to find a
neurosurgeon who is very familiar with Chiari Malformation
surgical procedures.  The down side to this is that, too often,
a neurologist's ego will not permit them to tell you that this is not their "area of specialization".
For more info on Chiari Malformation (otherwise known as Arnold-
Chiari}, Type I & II see http://cpmcnet.columbia.edu/dept/nsg/
PNS/ChiariMalformation.html
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