Arnold-Chiari Malformation Support Group
Arnold-Chiari malformation, sometimes referred to as 'Chiari malformation' or ACM, is a congenital anomaly of the brain. Arnold-Chiari Malformation II occurs in almost all children born with both spina bifida and hydrocephalus, but ACM I is typically seen in children and adults without spina bifida.
I have been all over multiple forums. My chief complaints are distorted vision and balance issues. This is been continuous since April of this year, but I started noticing vision problems last fall, with tracking and adjusting to distances. Below is a brief history:
2008 diagnosed with Myasthenia Gravis primarily bulbar. MG can, effect vision, however this has been ruled out by many docs. I'll explain a bit further down. I saw an optician in January of 2013 for my vision problems, stated 20/25 with irregular astigmatism in right eye.
April of 2013, woke up and felt off balance and as if the world was slanted. Noticed distorted wavy/moving vision. Shimmering vision, and significant tinnitus with hyperacusis. I have had tinnitus for a long time, this was much louder, especially on left side and I could not stand ringing from tube television. No double vision (which would have been convenient for MG). I went to the local ER, ct scan was performed. I told them the best I could describe it was oscillopsia, I asked if it could be from MG, they said I would know more about that then them. wow. This was on a Saturday.
I call my neurologists office. Doctor gets back to me 2 days later after the problem started, and says to come in that Monday morning. He evaluates me, orders MRI w/wo contrast to rule out MS, stroke, vasiospasms. Evoked potentials are ordered with routine blood work to check for autoimmunce diseases, b12 deficiency, and other things. Only MG came back high. Everything else was clear, MRIs and evoked potentials.
May of 2013 I see a neuro-opthalmologist at the Mittleman Eye Center in West Palm. I have full exam, everything is fine. OCT, glaucoma, and visual field testing is normal. Said maybe stress. (I asked my neuro if anxiety could cause this when I first saw him and he said the symptoms were causing my anxiety. I would have to agree now...) Referred to retina specialist to rule out other conditions.
May of 2013 see an ENT to rule out ear issues that could cause problem. VENG comes back mildly abnormal. Referred to University of Florida.
June of 2013, see Retina specialist and have a special dye injected, retina is perfect. Says my vision problem is odd.
June 2013, neuro says we may want to run MRIs again in 6 months or have a spinal tap to rule out MS 99%. Spinal tap is done, it's good. He thinks MG, refers me to Mayo Clinic to see another neuro-opthalmologist and a neuro muscular specialist.
July of 2013, I have a second set of MRIs done on a Tesla 3 because my neuros MRI was a 1.5. Complete MRI of spine and brain, w/wo contrast, normal.
August of 2013, see Dr. O'Malley at UF for Ent consult. Reuqests CT scan for Superior Canal Dehisence. This comes back clean.
August of 2013, go to Mayo Clinic. Another eye exam is performed, they said eyes look good. Not from MG but don't know what.
August of 2013, I make an appt. to Bascom and Palmer which is the #1 eye hospital in the U.S to see neuro-opthalmologist. This appt. is 9/17/13.
September of 2013 this week. See doctor I was referred to for VENG back in May just to pick his brain. Doctor confirms that I have an eye movement problem evident on the VENG. He does not specialize in eye care, but the test picked up abnormal saccades in right eye, and abnormal optokinetics at 40 degrees in both eyes. I cannot believe the other eye doctors didn't even think of offering testing for this.
September of 2013 this week, I followed up with my neuro. He now thinks I had a stroke that effected the visual cortex bilaterally, it's just too small to see on 2 MRIs and 2 ct scans. I don't think this is likely.
What I have concluded. I think there is an issue in the area of the brain that is responsible for piecing my vision together. I think it's something high in the neck because I have constant pain there. I also notice when I lay on my stomach and tilt my head back, my hearing cuts out about 50% and I can hear my pulse beat in my ears. I get dizzy oftenT and sometimes feel like I am going to pass out. I have never passed out, yet. I've heard about Arnold Chiari formation for a long time now, but I can't imagine this would have been missed. I don't think I have it, but I think I have a problem in the cerebellum. I attached some pics to see what some of you think. The other thing I am thinking, is some kind of atrophy or MSA. The reason why, is I had an abnormal sleep study where I moved way too much in my sleep. The eye movement issue indicates a neurological issue, not an eye health issue. And also the light headedness when standing. I will bring this up in my next appt. Thanks for hearing me out. 30,000 worth of testing has been done easily and honestly, it's pretty scary that a lot of my doctors aren't even aware of the test results they order. I have better records then most of them and have to remind them of things that have been performed or even suggest testing. I had to bring up to neuro, after thanking him for everything he has done for me, that not one person has thought of performing a brain function test such as an EEG. He said I was right and ordered one. Why the heck do I HAVE TO BRING THAT UP? I would think doctors would think of this. Anyways, if anyone has anything to offer, please let me know
Additional info: Just to add, I'm 27 years old as well. I think age is a factor and I noticed when I looked at some of the symptoms of this condition, some of them apply. I notice too, that when I sneeze, especially when I have a headache, I get a sharp ice pick pain at the base of my neck. I do have a c3-c4 bulge that I have know about since 2008 as well. I am also showing early signs of cervical disc disease which runs in my family. I have no other family history of anything neurological, including myasthenia gravis. I know bulge discs can cause neck pain, but this pain is high up, and my scalp tingles too. I have experience numbness and tingling in hands and feet for a couple of years right now. Mayo Clinic has performed multiple EMGs and Nerve Conductive study tests and they were good. I have also been tested for Lyme, twice. Below are two pics of my MRI:
http://neurotalk.psychcentral.com/attachment.php?attachmentid=7681&stc=1&thumb=1&d=1379214086
http://neurotalk.psychcentral.com/attachment.php?attachmentid=7682&stc=1&thumb=1&d=1379214095
2008 diagnosed with Myasthenia Gravis primarily bulbar. MG can, effect vision, however this has been ruled out by many docs. I'll explain a bit further down. I saw an optician in January of 2013 for my vision problems, stated 20/25 with irregular astigmatism in right eye.
April of 2013, woke up and felt off balance and as if the world was slanted. Noticed distorted wavy/moving vision. Shimmering vision, and significant tinnitus with hyperacusis. I have had tinnitus for a long time, this was much louder, especially on left side and I could not stand ringing from tube television. No double vision (which would have been convenient for MG). I went to the local ER, ct scan was performed. I told them the best I could describe it was oscillopsia, I asked if it could be from MG, they said I would know more about that then them. wow. This was on a Saturday.
I call my neurologists office. Doctor gets back to me 2 days later after the problem started, and says to come in that Monday morning. He evaluates me, orders MRI w/wo contrast to rule out MS, stroke, vasiospasms. Evoked potentials are ordered with routine blood work to check for autoimmunce diseases, b12 deficiency, and other things. Only MG came back high. Everything else was clear, MRIs and evoked potentials.
May of 2013 I see a neuro-opthalmologist at the Mittleman Eye Center in West Palm. I have full exam, everything is fine. OCT, glaucoma, and visual field testing is normal. Said maybe stress. (I asked my neuro if anxiety could cause this when I first saw him and he said the symptoms were causing my anxiety. I would have to agree now...) Referred to retina specialist to rule out other conditions.
May of 2013 see an ENT to rule out ear issues that could cause problem. VENG comes back mildly abnormal. Referred to University of Florida.
June of 2013, see Retina specialist and have a special dye injected, retina is perfect. Says my vision problem is odd.
June 2013, neuro says we may want to run MRIs again in 6 months or have a spinal tap to rule out MS 99%. Spinal tap is done, it's good. He thinks MG, refers me to Mayo Clinic to see another neuro-opthalmologist and a neuro muscular specialist.
July of 2013, I have a second set of MRIs done on a Tesla 3 because my neuros MRI was a 1.5. Complete MRI of spine and brain, w/wo contrast, normal.
August of 2013, see Dr. O'Malley at UF for Ent consult. Reuqests CT scan for Superior Canal Dehisence. This comes back clean.
August of 2013, go to Mayo Clinic. Another eye exam is performed, they said eyes look good. Not from MG but don't know what.
August of 2013, I make an appt. to Bascom and Palmer which is the #1 eye hospital in the U.S to see neuro-opthalmologist. This appt. is 9/17/13.
September of 2013 this week. See doctor I was referred to for VENG back in May just to pick his brain. Doctor confirms that I have an eye movement problem evident on the VENG. He does not specialize in eye care, but the test picked up abnormal saccades in right eye, and abnormal optokinetics at 40 degrees in both eyes. I cannot believe the other eye doctors didn't even think of offering testing for this.
September of 2013 this week, I followed up with my neuro. He now thinks I had a stroke that effected the visual cortex bilaterally, it's just too small to see on 2 MRIs and 2 ct scans. I don't think this is likely.
What I have concluded. I think there is an issue in the area of the brain that is responsible for piecing my vision together. I think it's something high in the neck because I have constant pain there. I also notice when I lay on my stomach and tilt my head back, my hearing cuts out about 50% and I can hear my pulse beat in my ears. I get dizzy oftenT and sometimes feel like I am going to pass out. I have never passed out, yet. I've heard about Arnold Chiari formation for a long time now, but I can't imagine this would have been missed. I don't think I have it, but I think I have a problem in the cerebellum. I attached some pics to see what some of you think. The other thing I am thinking, is some kind of atrophy or MSA. The reason why, is I had an abnormal sleep study where I moved way too much in my sleep. The eye movement issue indicates a neurological issue, not an eye health issue. And also the light headedness when standing. I will bring this up in my next appt. Thanks for hearing me out. 30,000 worth of testing has been done easily and honestly, it's pretty scary that a lot of my doctors aren't even aware of the test results they order. I have better records then most of them and have to remind them of things that have been performed or even suggest testing. I had to bring up to neuro, after thanking him for everything he has done for me, that not one person has thought of performing a brain function test such as an EEG. He said I was right and ordered one. Why the heck do I HAVE TO BRING THAT UP? I would think doctors would think of this. Anyways, if anyone has anything to offer, please let me know
Additional info: Just to add, I'm 27 years old as well. I think age is a factor and I noticed when I looked at some of the symptoms of this condition, some of them apply. I notice too, that when I sneeze, especially when I have a headache, I get a sharp ice pick pain at the base of my neck. I do have a c3-c4 bulge that I have know about since 2008 as well. I am also showing early signs of cervical disc disease which runs in my family. I have no other family history of anything neurological, including myasthenia gravis. I know bulge discs can cause neck pain, but this pain is high up, and my scalp tingles too. I have experience numbness and tingling in hands and feet for a couple of years right now. Mayo Clinic has performed multiple EMGs and Nerve Conductive study tests and they were good. I have also been tested for Lyme, twice. Below are two pics of my MRI:
http://neurotalk.psychcentral.com/attachment.php?attachmentid=7681&stc=1&thumb=1&d=1379214086
http://neurotalk.psychcentral.com/attachment.php?attachmentid=7682&stc=1&thumb=1&d=1379214095
http://neurotalk.psychcentral.com/attachment.php?attachmentid=7682&d=1379214095
These images are bigger.
I'll try to be brief but wanted to share since seem to have some medical similarities.
Starting last year I had intermittent double vision, just in the mornings and evening and a droopy eyelid for years.
By May it was constant double vision, lot of blurriness and began having some difficulty in getting a full breath. Not a lot of headaches. Neuro suspected MG and also wanted to do a MRI to rule out MS. The MRI showed a 20 mm Chiari Malformation and limited flow. I was told that was the cause of my vision problems.
Soon afterwards I began feeling strange in my throat, like I have to think to swallow and strange sensations on my face, like it's being pulled down. Again attributed to Chiari. I was decompressed just about a year ago by a specialist and shown to be successful. I did better for about six weeks afterwards, than as I returned to work my symptoms came back with a vengeance along with some new ones. I also developed a limp that comes and goes and at times my tongue feels burnt.
January 2013 I saw a neuro opthomologist who determined that I do have MG and my regular neuro started me on Mestinon and Prednisone. Mestinon helps a little, prednisone does not.
By June 2013 I had to stop working due to symptoms getting worse and had one hospitalization for symptoms exasperation from what i thought was MG. IVIG worked very well but unfortunately I had an allergic reaction and cannot continue. Now my neuro's and consults say I do not have MG since I have no test that shows it and that the Chiari is most likely unrelated since I have been decompressed and yet new symptoms have arised. I have also had eye muscle surgery to straighten the eyes back up in July 2013 , but still have intermentant double vision.
I feel for you - it's so so frustrating to know something is wrong with you and to not have a clear answer or diagnosis. Or in both our cases, that there is something clear (you the Myasthenia and me the Chiari) but there is something more and yet we are left with Google. I'm not a radiologist by any means, but it does look like a clear droop in your MRI. But my understanding is if so it would be pretty clear to the radiologist and neurologist and would be relayed to you. I know you have a copy of your MRI's but do you have the radiologist reports as well? I don't know where you are located, but if it is Chiari you can send it to a specialist in regards to a consult. Again strange that if there is Chiari no one has mentioned it.
Good luck to you - keep us posted. = )
Angie
The neurologist only spotted the bulging disc in my lower back. He had the neurosurgeon look at the MRI to determine if I needed surgery on the disc. The neurosurgeon spotted the SM and did further testing which revealed the Chiari and another bulging disc in my neck. He was not concerned about the discs whatsoever, but he was actually alarmed by the SM size, the Chiari, and my reflexes. He said that he was concerned about the "Giant". Sometimes, doctors could care less about pain issues unless they have actual tests which prove your body is in distress. What a shame, your body has to become dilapidated to that point.
God bless you in your search for an answer. It took almost 30 years for me to find an answer and being misdiagnosed because of problems arising as a result of the SM and Chiari.