Guest guest Posted September 12, 2002 Report Share Posted September 12, 2002 Hello everyone. I've been somewhat of a lurker on this forum for a few weeks. In fact, I've been a member since about the time Deborah went to LIJH for the PET scans. I had seen an old post of Deborah's (from 2001 I think) at the MGH forums some 2 months ago or so. It talked about PD, MSA, DBS and .... Chiari. I emailed her then got in touch with and told him not to rule Chiari I malformation out as a possibility for Deborah's problems, even though some doctors told them it probably wasn't causing her problems. I told that if Dr Levesque and PET scans didn't show anything conclusive, to visit probably the world's top Chiari I expert at North Shore in Manhasset, NY. This doctor spoke with Dr Levesque, and perhaps a few others, and and Deborah were rushed to Manhasset for potential emergency Chiari surgery. As it turned out, they didn't think the Chiari per se was causing her problems, although it is still a bit fuzzy since she has been deemed too toxic to even try surgery or some tests. I believe Deborah is to return in 6 months or so after her body has cleaned itself out for retesting. told me that this doctor had a hunch of what was causing her problems. They were waiting for her to have severe enough headaches in order to perform a spinal tap (lumbar puncture) and monitor if her headaches got better. If so, then she probably had elevated ICP (intracranial pressure) which would explain at least some of her problems. Although I don't know for sure, I suspect that the Chiari may be a secondarily caused by the high ICP, if that indeed is the case. I may have some of these details incorrectly. I've managed to get a member of the shydrager forum to scan MRI pics and send them to me. I saw borderline Chiari and asked this person to email the scans and symptom history to one of the few experts. This was done and Chiari has been suggested and the doctor has asked that a few more tests be done prior to a visit. I won't mention this person's name as I was asked to keep the name confidential until there is a confirmation of the diagnosis. I've also noticed that another member here has a daughter with Chiari I and he mentioned that he's seen several people diagnosed with it over the years. OK, so why then have so many people had multiple MRIs and various doctors tell them that they are normal, you ask?! Well, let me explain... My daughter had several head MRIs for progressive neurological problems over her first 5 years of life. We had several " I don't knows " and a few misdiagnoses. All said the MRIs were normal. At some point down the road, a few nurses suggested her symptoms sounded like Chiari I malformation. We researched, found a Chiari forum and discovered an expert in NY who was diagnosing and treating Chiari I in patients without the age-old classic criteria. After several doctors said she definitely didn't have Chiari I, we got an opinion from this doctor in NY and he diagnosed Chiari I malformation. We made a few enemies in the local medical community because of this so we then got another Chiari expert and he, too, diagnosed it. Since she was deteriorating quickly, we decided to have her undergo surgery for Chiari and some 20-25 symptoms were alleviated, some almost immediately, including life-threatening respiratory-related problems and occasional bouts of blindness, among others. Chiari I is poorly understood and very often missed or deemed insignificant. Most doctors will only diagnose it if the cerebellar tonsils (2 lobes at the inferior part of the cerebellum) protrude or herniate into the spinal cord, extending below the level of the foramen magnum by at least 5mm. In fact, many doctors, perhaps most, think 5mm descent is " mild " or " borderline " and cannot cause problems. A few specialists in the U.S. now realize that the age-old criteria is inaccurate and incomplete. That is, not only are there other factors which must be analyzed on imaging, in addition to tonsillar herniation, but symptomatic patients with less than 5mm tonsillar herniation should not be excluded from the diagnosis....this includes people who demonstrate NO tonsillar herniation....like my daughter! Thus, that is the reason why so many specialists said she did NOT have Chiari I. The 2 experts know there are other factors required to make the diagnosis. Surgery outcome proved them right!! Chiari I can cause a multitude of possible symptoms. The experts are now finding that Chiari I patients often have cervical spine problems, elevated ICP, and other anomalies. Most doctors don't know about this, don't understand it, or are taking a very cynical view of it....I would guess most belong to the last category. You see, it would mean doctors have been wrong for so long - I mean, you and I know they are not infallible - it's just that many don't want us to know that. I can go on and on about the number of people I've seen on the Chiari forum who've been told for years that they have no Chiari, or have mild Chiari, or find out years later from reading an old radiology report that they have Chiari but that the doctor didn't mention it because it was deemed insignificant, and then end up seeing an expert, getting treatment and suddenly feeling better! My child is one of those. How does this relate to MSA? Well, MSA is one of those syndromes which is diagnosed primarily or solely on the basis of symptoms. Chiari I can cause all sorts of symptoms - everyone is different. It can mask many syndromes. You can have one, two, 10, 30, 70 symptoms and various combinations and severity of symptoms. What is Chiari? Well, there's more and more evidence to suggest that it is one of mesodermal origins - that is, it is not a brain tissue/neural problem per se. It's a bony problem (underdeveloped occipital bone and possibly cervical spine anomalies) which in turn creates a smaller compartment to contain neural tissues, arteries and CSF cisterns. It thus secondarily causes compression of neural tissue and arteries as well as CSF pathways....and often, but not always, compresses the cerebellum such that the tonsils protrude down into the spinal canal, past the craniovertebral junction (where the cranium and spinal canal meet). Milhorat et al published " Chiari I malformation redefined " in the medical journal, Neurosurgery, in May 1998. They defined other criteria which should be used to diagnose Chiari I and also found that 9% of their 364 symptomatic patients had less than 5mm tonsillar herniation. This is the controversial part. Many doctors are taking a cynical view of these few experts suggesting they are decompressing too many people. What little they know. Bottom line - I would have no doubt that there are alot of people out there with neurological and autonomic problems who have less than 5mm tonsillar herniation (and even many with more) who are being misdiagnosed and told their Chiari is insignificant or they don't have it, but they do match the new criteria. Some of these experts have fueled the controversy in suggesting that many people with cervical spinal stenosis (CSS), often found with Chiari I, also need surgery - their criteria for CSS is also controversial, although I don't understand it as well. This is mostly because at the end of 1999, a Wall Street Journal article came out talking about all this and the fact that a few doctors were performing surgery on patients diagnosed with Fibromyalgia and Chronic Fatigue Syndrome. Of course, the media put a twist on it suggesting that there was now a surgical solution to FMS and CFS patients - this was not the case - the reality is that some percentage of FMS/CFS patients are actually misdiagnosed and have Chiari and/or CSS. Then came a Dateline show on it, followed by 20/20 and various other smaller newspaper articles and TV shows on it. I saw all this unfold on the Chiari forum. The old guard in the neurosurgical field has taken a dim view of all this, but then again, how many thought the world was once flat and insisted on it even after people suggested it wasn't! So, is it possible that some MSA patients really have Chiari I? I don't know for sure, but I wouldn't be surprised if at least some do. Like I said, Chiari I patients are used to being told they don't have Chiari even though many specialists review their MRIs and say there's nothing abnormal or told it's mild. It happened to us. I've seen it happen to many people on the Chiari forum over the last 5 years. Even the 5mm tonsillar herniation criteria is not agreed upon by many doctors. Some think you need substantially more herniation to be symptomatic. Some think it can't cause many symptoms and some think you need an associated syrinx (spinal cord cyst) to bother with it, regardless of how large the herniaton amount is. There's alot of disagreement. There's only about a handfull of doctors who understand the new criteria. The elite in the neurosurgical field are taking a cynical view of it all and refuse to believe it. It'll take them a long time to rid themselves of the ego and try to understand it. Hope this of use to you. Regards. ES Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 12, 2002 Report Share Posted September 12, 2002 ES, MRI's are NOT the only tests run on most people here. Diagnoses of MSA is far more complex than that. Remember that my daughter saw most of the same doctors that my wife Charlotte saw and my daughter was diagnosed as Chiari, while my wife was diagnosed as " probable MSA " . My wife had many, many tests run from 1989-1995. None of them showed Chiari. On the other hand my daughter showed different symptoms such as a hand that " fell asleep " that Charlotte never exhibited. Over the years here on the list I have suggested to at least 3 people that they had symptoms that sounded more like Chiari than MSA as I have seen each (and my daughter's syrnix is 15 mm). None of us are neurologists and they use a battery of tests to determine what is (and isn't) going on in your brain. To me and Charlotte, they waited and logically waited for more to develope before moving on with a diagnoses. In Deborah's case, I feel they decided on a drastic operation in the early stages when they should have adopted a " wait and see " stance. That is only my opinion and I still do not understand why all those things were done as I never saw symptoms listed in the order that she got them compared to the treatment she received. Charlotte asked about fetal tissue transplant in 1993 and was told that she did not have typical PD so an experimental proceedure for a condition she might not have, was too risky and the doctor would not recommend it for one of her family members. The Chiari operation is risky also. If your symptoms are bad enough that you want to risk permanent paralysis, then it is worth the risk. However my daughter's neuro-surgeon told her that he would NOT recommend it for his wife if she needed it. Instead, he suggested that she get physical therapy and refrain from any violent exercise or amusment park rides. To date this has worked for her (almost 5 years). She may need the operation in the future, but the " wait and see on doctor's advice " has seemed to work for us. Note too that is what Deborah is now doing as well. I hope it works for her as well as it worked for us. Charlotte did live almost 12 years with MSA and that seems to be a fairly good record. Take care, Bill Werre sousaed wrote: >Hello everyone. > >I've been somewhat of a lurker on this forum for a few weeks. In fact, >I've been a member since about the time Deborah went to LIJH for the >PET scans. > >I had seen an old post of Deborah's (from 2001 I think) at the MGH >forums some 2 months ago or so. It talked about PD, MSA, DBS and .... >Chiari. I emailed her then got in touch with and told him not >to rule Chiari I malformation out as a possibility for Deborah's >problems, even though some doctors told them it probably wasn't >causing her problems. I told that if Dr Levesque and PET scans >didn't show anything conclusive, to visit probably the world's top >Chiari I expert at North Shore in Manhasset, NY. This doctor spoke >with Dr Levesque, and perhaps a few others, and and Deborah >were rushed to Manhasset for potential emergency Chiari surgery. > >As it turned out, they didn't think the Chiari per se was causing her >problems, although it is still a bit fuzzy since she has been deemed >too toxic to even try surgery or some tests. I believe Deborah is to >return in 6 months or so after her body has cleaned itself out for >retesting. told me that this doctor had a hunch of what was >causing her problems. They were waiting for her to have severe enough >headaches in order to perform a spinal tap (lumbar puncture) and >monitor if her headaches got better. If so, then she probably had >elevated ICP (intracranial pressure) which would explain at least some >of her problems. Although I don't know for sure, I suspect that the >Chiari may be a secondarily caused by the high ICP, if that indeed is >the case. I may have some of these details incorrectly. > >I've managed to get a member of the shydrager forum to scan MRI pics >and send them to me. I saw borderline Chiari and asked this person to >email the scans and symptom history to one of the few experts. This >was done and Chiari has been suggested and the doctor has asked that a >few more tests be done prior to a visit. I won't mention this person's >name as I was asked to keep the name confidential until there is a >confirmation of the diagnosis. > >I've also noticed that another member here has a daughter with Chiari >I and he mentioned that he's seen several people diagnosed with it >over the years. OK, so why then have so many people had multiple MRIs >and various doctors tell them that they are normal, you ask?! Well, >let me explain... > >My daughter had several head MRIs for progressive neurological >problems over her first 5 years of life. We had several " I don't >knows " and a few misdiagnoses. All said the MRIs were normal. At some >point down the road, a few nurses suggested her symptoms sounded like >Chiari I malformation. We researched, found a Chiari forum and >discovered an expert in NY who was diagnosing and treating >Chiari I in patients without the age-old classic criteria. After >several doctors said she definitely didn't have Chiari I, we got an >opinion from this doctor in NY and he diagnosed Chiari I malformation. >We made a few enemies in the local medical community because of this >so we then got another Chiari expert and he, too, diagnosed it. Since >she was deteriorating quickly, we decided to have her undergo surgery >for Chiari and some 20-25 symptoms were alleviated, some almost >immediately, including life-threatening respiratory-related problems >and occasional bouts of blindness, among others. > >Chiari I is poorly understood and very often missed or deemed >insignificant. Most doctors will only diagnose it if the cerebellar >tonsils (2 lobes at the inferior part of the cerebellum) protrude or >herniate into the spinal cord, extending below the level of the >foramen magnum by at least 5mm. In fact, many doctors, perhaps most, >think 5mm descent is " mild " or " borderline " and cannot cause problems. > >A few specialists in the U.S. now realize that the age-old criteria is >inaccurate and incomplete. That is, not only are there other factors >which must be analyzed on imaging, in addition to tonsillar >herniation, but symptomatic patients with less than 5mm tonsillar >herniation should not be excluded from the diagnosis....this includes >people who demonstrate NO tonsillar herniation....like my daughter! >Thus, that is the reason why so many specialists said she did NOT have >Chiari I. The 2 experts know there are other factors required to make >the diagnosis. Surgery outcome proved them right!! > >Chiari I can cause a multitude of possible symptoms. The experts are >now finding that Chiari I patients often have cervical spine problems, >elevated ICP, and other anomalies. Most doctors don't know about this, >don't understand it, or are taking a very cynical view of it....I >would guess most belong to the last category. You see, it would mean >doctors have been wrong for so long - I mean, you and I know they are >not infallible - it's just that many don't want us to know that. > >I can go on and on about the number of people I've seen on the Chiari >forum who've been told for years that they have no Chiari, or have >mild Chiari, or find out years later from reading an old radiology >report that they have Chiari but that the doctor didn't mention it >because it was deemed insignificant, and then end up seeing an expert, >getting treatment and suddenly feeling better! My child is one of >those. > >How does this relate to MSA? Well, MSA is one of those syndromes which >is diagnosed primarily or solely on the basis of symptoms. Chiari I >can cause all sorts of symptoms - everyone is different. It can mask >many syndromes. You can have one, two, 10, 30, 70 symptoms and various >combinations and severity of symptoms. What is Chiari? Well, there's >more and more evidence to suggest that it is one of mesodermal origins >- that is, it is not a brain tissue/neural problem per se. It's a bony >problem (underdeveloped occipital bone and possibly cervical spine >anomalies) which in turn creates a smaller compartment to contain >neural tissues, arteries and CSF cisterns. It thus secondarily causes >compression of neural tissue and arteries as well as CSF >pathways....and often, but not always, compresses the cerebellum such >that the tonsils protrude down into the spinal canal, past the >craniovertebral junction (where the cranium and spinal canal meet). > >Milhorat et al published " Chiari I malformation redefined " in the >medical journal, Neurosurgery, in May 1998. They defined other >criteria which should be used to diagnose Chiari I and also found that >9% of their 364 symptomatic patients had less than 5mm tonsillar >herniation. This is the controversial part. Many doctors are taking a >cynical view of these few experts suggesting they are decompressing >too many people. What little they know. > >Bottom line - I would have no doubt that there are alot of people out >there with neurological and autonomic problems who have less than 5mm >tonsillar herniation (and even many with more) who are being >misdiagnosed and told their Chiari is insignificant or they don't have >it, but they do match the new criteria. Some of these experts have >fueled the controversy in suggesting that many people with cervical >spinal stenosis (CSS), often found with Chiari I, also need surgery - >their criteria for CSS is also controversial, although I don't >understand it as well. This is mostly because at the end of 1999, a >Wall Street Journal article came out talking about all this and the >fact that a few doctors were performing surgery on patients diagnosed >with Fibromyalgia and Chronic Fatigue Syndrome. Of course, the media >put a twist on it suggesting that there was now a surgical solution to >FMS and CFS patients - this was not the case - the reality is that >some percentage of FMS/CFS patients are actually misdiagnosed and have >Chiari and/or CSS. Then came a Dateline show on it, followed by 20/20 >and various other smaller newspaper articles and TV shows on it. I saw >all this unfold on the Chiari forum. The old guard in the >neurosurgical field has taken a dim view of all this, but then again, >how many thought the world was once flat and insisted on it even after >people suggested it wasn't! > >So, is it possible that some MSA patients really have Chiari I? I >don't know for sure, but I wouldn't be surprised if at least some do. > >Like I said, Chiari I patients are used to being told they don't have >Chiari even though many specialists review their MRIs and say >there's nothing abnormal or told it's mild. It happened to us. I've >seen it happen to many people on the Chiari forum over the last 5 >years. Even the 5mm tonsillar herniation criteria is not agreed upon >by many doctors. Some think you need substantially more herniation to >be symptomatic. Some think it can't cause many symptoms and some think >you need an associated syrinx (spinal cord cyst) to bother with it, >regardless of how large the herniaton amount is. There's alot of >disagreement. There's only about a handfull of doctors who understand >the new criteria. The elite in the neurosurgical field are taking a >cynical view of it all and refuse to believe it. It'll take them a >long time to rid themselves of the ego and try to understand it. > >Hope this of use to you. Regards. > >ES > > > >If you do not wish to belong to shydrager, you may >unsubscribe by sending a blank email to > >shydrager-unsubscribe > > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 14, 2002 Report Share Posted September 14, 2002 ES and all, I am sorry that your daughter took so long and had so many tests with wrong diagnoses. My daughter was in her thirties and I had nothing to do with her decision, she did it all on her own. In 1998 she consulted two of the top doctors in the field and BOTH told her that the Chiari operation was very risky at that time - one advised that she have the operation and one advised that she not have the operation so she went to a third who had done the operation many times - he told her, he would not advise his wife to have the operation if it was her. So she went with the majority and gets checked yearly. Since the symptoms are not getting worse, are not life threatening and not extreme, they are still not advising the operation. Since my daughter still has some symptoms, I took a look at your sites and found that the one at Duke University had a doctor with a familiar sound (Dr. Milhorat). So I went back and found his site which you did not include: http://www.asap4sm.com/docscorner/milhorat02.html Please note the following from his site: Dr. Milhorat has strict requirements for surgery. He does not recommend surgery for patients unless they meet one of the following criteria. First, evidence of progressive clinical deterioration. Two, the current symptoms, while not progressive, have reached a point of being unbearable or disabling. It is the patient who is in the best position to make that determination, put into the context of their own lives. Dr. Milhorat also uses a scale of 0 to 100. 100 means you're asymptomatic, 0 would mean you're dead. He does not operate on patients unless they are 70 or below. He does not recommend surgery for nuisance symptoms. These are complaints you wish you didn't have but you do; nevertheless you get through the day all right and they do not really affect your way of life. At the present time, he does not recommend surgery to prevent problems from occurring in the future because the natural history of Chiari I and SM is not understood. With the advent of MRI we are able to see vast numbers of patients with a wide spectrum of complaints ranging from nothing at all, to a little something, to moderate, to severe. There are no longer any rules about progression. If a patient is getting worse after you've followed it for a while, it's likely it will continue. But if a patient is stable and remains so month after month and year after year, there is no way to determine whether it's going to get worse, and probably as time passes it is increasingly likely it will remain the same forever. With one exception. Trauma is a big problem. Patients with Chiari with or without a syrinx are much more likely to have symptoms begin to progress after an accident. Patients with a diagnosis of Chiari or SM that is not producing much in the way of symptoms should be on notice to keep themselves out of harm's way. Avoid activities that have a high risk. Dr. Milhorat believes that the Duke Study may identify many people who are asymptomatic. He believes these people can become symptomatic if they have an accident, fall, trauma or so forth. Several of your sites (at least the medical ones - not the patient ones) suggest that the operation IS dangerous. You MUST look at the medical sites and NOT sites written by patients who were helped greatly by the operation to get the whole picture. There IS a possibility of complete paralysis from the operation. You are correct in saying that if a patient has progressive MSA symptoms, and has a Chiari, they should be evaluated for the operation. As I said, over the past three years I have told at least three people that I can remember that they should ask about it. I can not speak for your experience, but I do NOT believe that after researching the doctors near you for an expert on Chiari, you would come up with three doctors who would be wrong in most cases - unless you live in a remote area possibly. Yet you imply that you went to many, many doctors who were wrong. Could you actually mean that you went to many, many doctors before you found one who understood the problem? We took Charlotte to many doctors before getting a diagnoses of Parkinson's and she had an MRI as early as 1985 for suspected problems of the upper spinal column and brain stem which showed no evidence of a syrnix. While she did not have complete closure of the spine around the spinal cord - there was no evidence of any problem and that was again looked at by experts at NIH in 1995. It DOES pay to get more than one opinion on a diagnoses of MSA - I agree there completely. If you have an obvious Chiari, especially one with a syrnix you should be evaluated by an expert also. But there are many and Dr. Milhorat was the one who recommended 's doctors (My cousin was a doctor at Duke and knew him at the time). However, we should all learn from Deborah's experience that jumping into brain surgery is NOT always the best option and sometimes more opinions are better. I have always relied on my family doctor to help me out in times like this. In 1980, I was told I needed a kidney operation (85% success rate) and my doctor said it was worth the risk - I got the operation and was among the 85% rather than the 15%. In 1996 they said I needed a heart operation as I had a 90% blockage in the main artery of my heart, my family docotr agreed and the surgeon said that with my physical condition, I had a 99% chance of playing softball within three months, I played softball in 87 days. But in ANY operation there is some chance of infection such as Deborah's .. In a Chiari operation, this is complicated by the fact that they are cutting bone within a hair's width of the spinal cord itself. I personally will not question my daughter's or your decision to have or not have the operation, that is a personal decision which should be based on as many facts as you can get. My concern is that these "facts" you are presenting cause confusion and concern here on the list. I certainly do not know if many on this list have Chiari, BUT neither do you. I have seen a Chiari MRI, but I am NOT an expert and do not know what symptoms it will cause. I have seen several MRI's of two people with syrnix cysts, but I still could not tell you if they are causing problems. Doctor's go through many years of training and experience to learn to see these problems and at some point, you must trust them to guide you to your decision. By bringing up these horror stories of bad doctor's you create mistrust in list members of their doctors. Now remember that you finally trusted a specific doctor or group of doctors before you authorized an operation. Would you want someone second guessing you if the operation had been a complete failure? I have had questions from people on the list asking if they should have had more tests done on their loved one to prevent them from dying - NO! You trusted your doctor, you did the best you could for the patient given the knowledge you had at the time and the patient died. It was in God's hands, you did the best you could. As a lay person goes, I am as familiar as almost any other lay person on MSA and some related brain disorders, yet I do NOT know for certain that Charlotte died of MSA as she did not want an autopsy. She was luckier than most as she did get an evaluation at NIH which was presented to a team of about 15 experts in NINDS and the prevailing thought was MSA probably OPCA related. As time went on after that she developed more of the other symptoms of other parts of the neopolitan ice cream analogy. She also had severe dystonia of her right arm which is not exactly typical for MSA, but I feel it was late MSA which many patients do not see. Personally, I feel it is better to focus on what can be done for the patients. 1. If you are unsure ask you family doctor who has known you the longest about a second opinion, that way you will get the opinion of someone who knows you AND has the training to help you make an informed decision. Don't "doctor shop" on your own. If you have not known any doctor long, check with a support group on your primary diagnoses. 2. Exercise! Even with Chiari there are exercises (not violent exercise) which can help. Get a prescription for physical therapy from your doctor. 3. Keep hydrated! This will not hurt a person with Chiari and will help MSA patients. 4. Eat a proper diet - this helps you fight any disease/disorder. 5. Don't panic! Panic and rash decisions can harm you more than wait and see, unless your family doctor agrees you need an immediate operation .. They are trained to make this sort of decision, you are not. As for the idea that only some doctors know how to diagnose Chiari, my daughter saw the same doctors that Charlotte saw in roughly the same order and she was diagnosed okay with standard tests. Yes, it took a year or more! I do not know how so many doctors missed a diagnoses in anyone else's case, but I feel from my experience on this list that a misdiagnoses in matters that count such as treatment of symptoms is rare and getting rarer. I do not believe that a diagnoses of MSA when it is really CBGD or something of that nature really affects treatment at this time. I do believe that it pays to find a family doctor early in life and stick with them so they learn you as an individual. We have trusted our family doctor since 1976 to help us choose the right medical path. And he had the records from our two previous family doctors - we did dump a family doctor because of personality problems in 1976 after less than two years. You need a doctor you can trust! Everyone here should try to evaluate information they receive on this list logically against their doctors and not assume their doctor is wrong. If in doubt, get a second opinion from a doctor - NOT one of us (including me). Take care, Bill Werre Quote Link to comment Share on other sites More sharing options...
Guest guest Posted September 14, 2002 Report Share Posted September 14, 2002 Bill (and all), First, let me clear something up. I'm not saying that just because someone is diagnosed with Chiari I that they should jump into surgery. If I made gave that impression, let me assure you that was not my intention. As I said, if your daughter can manage with the problems she has without surgery, then that's great. I agree it's preferrable over surgery. I know about Dr Milhorat's strict criteria for surgery and I agree with him. I know ... he was the first doctor to diagnose my child with Chiari I malformation after 5 years of searching for an answer to the progressive problems, with several " I don't knows " and misdiagnoses along the way. Dr Milhorat's Chiari I diagnosis got 3 local neurosurgeons in a fit. My child had life-threatening respiratory problems and beginning to go blind. That's why we chose to have the surgery done. Months later, we diplomatically notified the local neurosurgeons of the alleviation of symptoms and they never replied - just goes to show the lack of class and concern! These doctors knew our child was worsening, had no definite diagnosis, no treatment and they kept mum and have refused to attempt to learn to this day, almost 4 years later! I'm not advocating people have surgery for nuisance symptoms - that would be irresponsible. You posted one of Dr Milhorat's articles from the ASAP site and made references to it. I'm glad you did. Here's another one of his: http://www.asap4sm.com/docscorner/milhorat01.html Here's what it says: " The current understanding of the Chiari I is a condition that involves herniation of cerebellar tonsils into the spinal canal. The condition can be associated with Syringomyelia and malformations at the base of the skull. Currently it has a narrow definition by radiologist- the herniation must be at least 3 millimeters beneath the opening of the skull; otherwise it is not regarded as a Chiari. Radiologists further disagree whether it should be 3 or 5 millimeters. This radiographic criteria is considered the beginning and the end of diagnosis. " Dr Milhorat knows even at least 3mm tonsillar herniation is not required for the diagnosis. I know as my child had 0mm herniation (i.e., no herniation) and he diagnosed Chiari I anyway - he knew of the other criteria. Here's another passage ... and the point to my posts: " We have come to understand the Chiari Malformation in the following way. It is a volumetrically too small posterior compartment of the skull. The posterior compartment is formed by the occipital bone in the back; this particular part of the bone is called the supraocciput. It's formed by a roof, which is made up of a membrane called the tentorium. It has a front, which is the clivus. Then there is a mystical opening at the top of the tentorium and there's a mystical opening at the bottom known as the foramen magnum. Now in the Chiari I malformation, the volume of this compartment is too small. The tentorium is sloped like the steeple of a church, not the roof of a house, where the normal tentorium comes out like this, the Chiari tentorium tends to be high rising. The back bone, as a consequence, tends to be short. In many patients with Chiari the clivus is short. Some patients with Chiari have basilar impression where the tip of the second vertebral body, the odontoid, tilts back. We refer to that as retroflexion of the odontoid. These make a small posterior compartment. Oh, and did he leave it out? There can also be herniation of the cerebellar tonsils through the foramen magnum. Milhorat has come to understand this as one of the consequences of a too small posterior fossa- not the actual phenomena. This is not the whole problem, but just a reflection of the too small posterior compartment. In Milhorat's study population, 34 patients or 9% had typical Chiari symptoms- typical headaches, visual disturbances, Meniere's like syndrome, lower cranial nerve disturbances, 2/3 had syrinxes. They had a small posterior fossa, they had a high steeped tentorium, they had a short supraocciput, and they had a small clivus. But you know what? Their tonsils were not down enough to be called a Chiari Malformation so these patients were kicked out of the group. But if you look here at the T-2 weighted images where spinal fluid is white behind the cerebellum you can see this is a compressed posterior fossa with an absence of CSF posteriorally and lateral to the cerebellum. This is a Chiari Malformation with the tonsils at the level of the foramen magnum, and not grossly below it. So we are redefining the Chiari Malformation as not simply how far the tonsils come down, but it is a diagnosis, which includes 7 specific signs. They include: the volume of the posterior fossa being small, CSF volume being reduced, compression owing to smallness of the bones in the back and most of them have varying degrees of tonsillar herniation. The failure to have greater than 3 mm to 5 mm herniation does not exclude the diagnosis of Chiari I in our new definition. " You must realize that these findings are relativelty new (published May, 1999) - like it took people a long time to recognize that the earth was not flat, the medical community will also take some time to realize that Chiari I's age-old definition is inacurrate and incomplete. Promoting surgery for Chiari I was and is not the point to my posts. There are people out there, Bill, that have serious problems. Some debilitating, some life-threatening - yes, there are also people who have " nuisance " symptoms. It's the crowd which belongs to the debilitating and life-threatening category which I mostly target my posts....and to those who seek answers to their condition, regardless of how bad it is. If some people find out they have Chiari I, whether they choose to have surgery is their business, not mine or yours. They can, however, listen to stories from others, just as they do on the shydrager list. I'm sure you post your opinions to give others your experiences and I'm sure you'd prefer that they listen and believe you, even though I'm sure you know not all do. I post for the same reason. People can choose to believe me or not - I can live with that. That's the nature of medical forums/mailing lists. You can choose to learn from others or not. Once again, the point to my posts was to make people aware that there have been relatively new findings regarding Chiari I - there are new criteria used to diagnose Chiari I malformation that most doctors don't yet know about it, don't understand it or are letting cynicism take hold of rational thinking. Case in point - my child did not fit the old or classic criteria for the diagnosis, thus why more than a dozen neurosurgeons, neuroradiologists and neurologists said there was no Chiari I. Dr Milhorat and a neurosurgeon from Chicago diagnosed Chiari I because they are one of the very few who have a grasp on overcroweded posterior fossa without significant tonsillar herniation (the latter, the only criteria used by most doctors today) and realize it can cause problems. In fact, many doctors even consider " obvious " Chiari I (based solely on significant tonsillar herniation) insignificant to symptoms - Chiari I is poorly understood and seeing a specialist who sees this alot is definitely recommended. Because doctors didn't agree with my child's Chiari I diagnosis doesn't necessarily make them " bad doctors " , as you seemed to imply what I was saying. I'm sure many of them are excellent doctors. In fact, I know some of them are very highly reputed specialists in their field. However, they don't know everything. I'm a computer specialist and I don't know everything there is to know about every computer's hardware, operating systems, development methodologies, etc. I have my specialty and doctors have their expertise within their domain. Even within my specialty, I don't know everything. Dr Milhorat will be the first person to admit that to you. Regardless of what the medical community likes lay-people to think, they only understand a small amount of how the human body works. In the end, they really don't know much (I've been told that by doctors). You suggesting that I'm fear-mongering people about " bad doctors " is a misinterpretation on your part. Some people on this list may not be comfortable with their diagnosis - what's wrong if they seek out other possibilities or doctors? You have no right to tell them NO, that's wrong. It's their business....and I'm not telling people you MUST see other doctors or seek other diagnoses. I'm telling people what I know and anyone can follow up on it if they wish - that's their business. If they do, I'll gladly do the best to answer their questions. You are right that neither you or I are Chiari experts. However, I've learnt quite a bit about it over the last 5 years, reading many people's stories and reading tons of Chiari I full-text articles published in medical journals, including consulting doctors for their opinions and I'm trying to share that. When people tell me they have Chiari, or think they have Chiari, I tell them what I know and always refer them to one or more doctors who I feel are knowledgable and experienced with it if that's what they want to do. I never tell people " yep, you have Chiari - go have surgery " . I give the some guidance and it's up to them whether they want to see any of the doctors I recommend. Even if they do, they are the ones who have to make the final decision of whether they should go ahead with surgery or not and if they're comfortable with their doctor. Trust me, I don't want that responsibility hanging over my head! You bring up the topic of people who've lost loved ones and question should they have had more tests done? Whether the answer is yes or no is irrelevant, unfortunately. These people did what they could under the circumstances and that can't be changed. The important thing is that people who are with us today and potentially deteriorating should have the option to learn about other medical conditions, experiences, new findings, etc, in order that they can decide for themselves whether they want to pursue it or not. You cannot answer for all the people on this list and discourage them from pursuing Chiari I or anything else. That's simply selfish. There are misdiagnoses all the time in the medical field - I'm sure you know that as much as the next person here. I know that as our child was misdiagnosed on several occasions. If people are content with their MSA diagnosis, then that's fine - that's their business. I just hope that the information I provide might give some people who are not comfortable with their diagnosis or doctor(s) something to consider. If they want to pursue it, that's their business. If not, that's their business. Your comment, 'Don't " doctor shop " on your own'. I know where I've heard that ... from doctors. I say to people with medical problems ... doctor shop to your heart's content. If you are capable of learning and being resourceful, you can find better doctors than they themselves will. Why? Doctors don't like other doctors to contradict their opinions. Who do they often refer you to for second opinions? Doctor buddies. If you don't think some people should shop for doctors, then that's your opinion. Had we taken your attitude regarding this issue, my child would be dead now...on more than one occasion too! My child had life-threatening liver tumors at birth - local doctors prescribed some medication and we were told life would be very short. We the parents of the patient (not the doctors) researched and found a clinical trial in Boston - our child was put on the experimental treatment and a life was saved. We researched alot and found the best doctors - it was the best thing we ever did! Patients, in my opinion, sometimes, perhaps often, have to take matters into their own hands because doctors are far from being infallible and often don't have the time to research on your behalf. It doesn't mean you will find answers to your problems by doing so, but you might. If you go buy a car, you'll shop around. Big deal, it's only a car! When it comes to your health, there's alot more at stake - shopping around for the right doctors is sometimes/often imperative. Best regards. Quote Link to comment Share on other sites More sharing options...
Recommended Posts
Join the conversation
You are posting as a guest. If you have an account, sign in now to post with your account.
Note: Your post will require moderator approval before it will be visible.