Chiari Malformation support

[Markthehatter]

Hi all,

I hope this finds you as well as you can be.

This is a long post so for those that don't have the energy or want to read a long post, I will get to the main points first.

• I have been suffering with significant long covid since March 2022 (symptoms below) and have been predominantly housebound in this time. I have however experienced bouts of fatigue usually lasting up to a week throughout my adult life. Mild CFS/ME? I cannot be sure.
• Last week following a brain and spinal MRI, I received the following results 'There is minor cerebellar ectopia with approximately 7 mm caudal descent of the tonsils at the foramen magnum. There is however no significant crowding of the neural structures demonstrated. Differential diagnosis is between a Chiari I malformation or minor cerebellar ectopia. Please correlate clinically.'
• I also present craniocervical instability symptoms but understand I would need to send my MRI images to a specialist.
• Whilst at this point in time I do not have a concrete Chiari 1 malformation diagnosis (currently sat on the fence with a differential diagnosis, from what I have researched online this may be because chiari is a tricky diagnosis and the radiographer must be very experienced in identifying chiari malformation. I will of course seek full clarification but at this point in time, so that I can further my research, I am assuming it is a chiari 1 diagnosis.
• My doctor suspects I have a connective tissue disorder, most likely Ehlers Danlos Syndrome.

My questions to anyone in the community that has any experience of a chiari diagnosis or potential treatment are;

• What is your personal experience of surgical treatment? Was it successful in the short term or to date?
• Has anyone decided against surgical treatment rather choosing other forms of symptom's relief, e.g., traction manipulation etc?
• Can anyone that has had successful treatment recommended a surgeon. I note these are two in Spain, Dr Gilete and Dr Salvador, and several in the US with Dr Bolognase receiving the best reviews.
• Could the chiari 1 malformation diagnosis be a red herring? I have suffered symptoms relating to chiari all my adult life and these have been hugely increased since my long covid onset. However, has anyone suffering with long covid and then received a chiari diagnosis attempted treatment for the chiari and their long covid symptoms remained.

Many thanks if you have read this far. I have listed my symptoms below. Those with a diagnosis or knowledge of chiari I would be really interested to know if you think my symptoms present a strong case for chiari influence (I am aware many have chiari malformation but remain asymptomatic all their life. It seems chiari malformation doesn't matter until it very much does). I will also apologise if you have read this message on other forums. I am trying to gather as much information from as many well-informed folk as I can. Thanks in advance to anyone that responds.

Take care every one

Mark

- I have a constant tightness and pain at the read base of my skull. This hurts when sneezing and sometimes completely locks out. Sometimes a very sharp pain in back of skull when sneezing and often accompanied by a strange clicking noise.
-Significant brain fog- Difficulty to process information, think and process the world around me.
-Bobble head (feel like my head is floating on my shoulders or like I am on a boat)
-Significant neck pain for the last 18 months, preventing me from being able to hold my head up for 3 months last year. Pain flares at the slightest provocation, e.g., laying awkwardly on a pillow, and pain remains for weeks and sometimes months. This has improved to the extent that I am now able to hold up my own head for an hour without resting.
-Headaches made worse by straining, e.g., going to the toilet, laughing, coughing
-Unsteady gait and poor coordination- always walking into things now
-Numbness and tingling feeling in hands and feet
-Problems with motor coordination of hand always knocking things over and hand writing is awful
-Constant very loud ringing in ears
-Vision changes- I find it hard to gauge depth of things around me which makes me dizzy
-Orthostatic intolerance with coat hanger pain
-Problems with swallowing- sometimes have difficulty swallowing food and am constantly trying to clear my throat
-Temperature dysregulation- I rarely sweat and frequently over heat
-Frequent neck injury with no apparent cause and does not respond well to stand physio and massage treatment *
-Sleep disturbance- Have not slept more than 1 hour in 18months and sleep apnea. *
-GI dysfunction, diagnosed irritable bowel syndrome *
-Electric pulse/shudder feeling down spine *
-Chronic fatigue *
- Postexertional malaise
-Lower back pain *
-I believe I also suffer from MCAS due to the symptom increase I experience directly after eating certain foods and an increase in symptoms when stopping taking my mast cell stabiliser medication (ketotifen).

The symptoms with a * show symptoms that I have had prior to my long covid journey. I have been admitted to A and E 3 times prior to long covid with horrific pain often caused during sport. Normal x rays have always come up blank. These symptoms have been troubling prior to Long Covid, in particular unexplainable constant neck pain and fatigue. This suggests to me that Covid, as is common with viral illness, has worsened the problems potentially caused by Chiari.

 

[almost]

Greetings @Markthehatter . Welcome!

I have Chiari type 1 as well. I have probably had this most of my life. My tonsils don't descend as much as yours, but I have some of the same symptoms you do, and am also trying to unravel whether some of my new symptoms that have appeared recently are long covid (I had it May of this year) or something else.

I have known about my Chiari for several years. My neurosurgeon, whom I trust, tells me this surgery is the absolute last choice you want to make. It is messy and prone to complications, and there is no guarantee things will improve. For me, he suggests leaving it alone.

My case has some additional complications in that I also have hydrocephalus (quite pronounced) and also a new thing he mentioned at my last visit that I need to get his term for -- essentially my spine and skull meet at an odd angle. I don't think it is CCI but may have similar effects.

It really would take A LOT for me to pull the trigger on Chiari surgery. I'm not sure net-net that it would be a gain for me. You are doing the right thing here -- getting others' experiences, which added to your personal research will help you decide.

 

[lenora]

Hello..... I'm a 76 yr. old female who has been dealing with this for approx. 35 years.

I started off with a diagnosis of Syringomyelia (I believe that Chiari wasn't know about then). I had a shunt (which promptly clogged) implanted in my spinal cord. Truly, the pain was unbearable and it was the worst surgery I've ever had or recovered from.

Fast forward 5 years and a different surgeon. This man was from our medical school and later transferred to another hospital. You're lucky to have a diagnosis of Chiari....so many radiologists miss it and, indeed, my neurologist was the one who found my SM and Chiari.

The surgeon refused to operate until he had perfected what was then a new technique. He knew what he was doing and I did see other Chiari patients in his office.

I had so much pain I thought I would go out of my mined Then we had nothing for nerve pain, gabapentin came along later. I did have the ACM surgery, and it immediately helped with the neck pain and, in addition, the cysts in my spinal cord were deflated. I had hydrocephalus and most of the symptoms you mention.

I also have scoliosis, lordosis and kyphos and was about 3 or 4 when they were discovered (I couldn't sit) and had terrible back pain. The treatment then was casts and braces....oh, how I hated them.

For years after surgery, my pain was better, expecially the neck pain, I could use my arms again and my feet were also better. I don't know exactly when, but most of those symptoms have returned over the years. The neck pain is still better. Straining is a no-no for us, and if I were you I'd give up sports as it can cause further damage. Walking is Ok if you can tolerate it.

I will say the Chiari surgery was easier by far than the spinal cord one. Once my skull had knitted, I did feel that I had some energy and was even able to travel at times. Other times I just had to get off the airplane, especially if it was delayed.

The symptoms you describe are very familiar and I have a lot of them back again. Insomnia never went away and is my constant companion. The arm and hand weakness is especially pronounced. My neurologist has died and my surgeon has retired or I'd be happy to share their names.

I know Dr. Bolognese's name because he was a trainee at the time I had my surgery. I never met him or even went to Long Island, for that matter. I found what I needed here in Dallas, TX.

By the time of my second surgery, I was in good physical condition. Proper meals, walking, use of vitamins, herbs, acupuncture (although acupressure turned out to be best) and I was able to get on with my life. I had an MRI each year.

I understand that Chiari surgery is now out of favor. A surgeon truly has to know what he's doing, there is no doubt of that.

,In my older years, I've developed a lot of problems common to older people. A few are heart disease, high BP, very high cholesterol (familial), two types of epilepsy which caused me to fall and end up with a few broken bones, the worst being a fractured pelvic area which kept me more or less bedridden for a year and definitely stopped my walking. I can walk, no problem, but my balance is off and the epilepsy can be a problem. Just extremely short spells. Still, I don't dwell on these matters and the many others I have because I'm old and what's the point?

I'm fed-up with doctors, and the new replacement neurologists I saw made it very clear that they didn't even know why I was there to begin with. So I stay with my internist (who isn't exactly young) and my cardiologist. Have I give up? No, I think I'm incredibly worn out. It's a lot of effort to have so many things wrong.

I learned about vitamins probably 40-45 years ago. Since then, many new ones have been discovered, enzymes, etc.

I'm afraid this is like everything in our lives: You have to make your own choices and yes, they're often difficult. Your Ehler-Danlos possibility makes everything harder, especially surgery, I would expect.

I hope you receive the names of doctors who can help you in the area you're in (state or province). Fla. used to have some of the top men, but I heard the best has also retired. Still, there are surgeons in the Fla. area.....The Miami Institute being one. At least they can direct you to the head surgeon now. It's a complex associated with a university. Good luck and better health. Yours, Lenora

 

[Markthehatter]

Thanks very much for your response. I certainly hear you when you urge caution when considering the operation. I must say though I have not anecdotally heard of anyone that has had a difficult surgical procedure, only positive reports online. This is course will have a strong selection/feedback bias but I do wonder what the objective success and failure rate is (acknowledging its a simple binary choice like I present here).

I think the choice regarding surgical intervention mostly revolves around quality of life. At this point in time, mine does not and my quality of life has been quite poor for the past 18 months. The sleep issues are fairly confounding. Plus I have a 6 month old baby that I cannot care for. I'm not pulling the heart strings here. Just trying to present my situation and why I may pull the trigger on a surgical procedure.

I am at the early stages regarding comorbidities so will certainly be patient here regarding a final decision. May I ask the route you took to find your hydrocephalus diagnosis?

 

[almost]

You're welcome, @Markthehatter . Everyone's situation is different. I completely understand why your calculus might tip toward moving forward. Family is very important.

I was diagnosed after complaining of pain behind an eye, and an ordered MRI clearly showed it. It too can exhibit similar symptoms to many brain disorders, as the swollen ventricles press the brain against the skull and this pressure, even if it is 'low pressure' hydrocephalus (my type), will over time cause brain damage and some loss of function. A good low-tech way to spot it (of course not fool-proof) is to look at head size. The pressure will expand the brain case as you develop, giving a characteristic large head.

I did not treat it at first diagnosis, as I was largely asymptomatic and I was a younger, prouder lad that didn't want to be bothered with the treatment. As I was having new, CFS like symptoms along with a new sleep disorder, I did have it treated last year with a ventriculoperitoneal (VP) shunt. It is basically a pump that drains ventricular fluid into the abdomen. It was an attempt to address my central apnea and my surgeon also said I was fortunate to have gone this long without larger problems, so waiting further was risking more serious loss of function. It did not solve my sleep problem, unfortunately.

I had accumulated some studies on the procedure and outcomes. I don't know if I still have them available, but I will look when I get a chance.

 

[lenora]

Yes, many shunts are used today and they drain into the abdomen. It usually takes care of the hydrocephalus.. Mine was discovered by an MRI (a new machine at the time),

So many of us suffer from the never-ending interruption of the sleep cycle. Sleep Clinics are useless, unless you have apnea, and you leave with a prescription for trazodone if you don't. Sleeping aids (pills) never helped me in the least, nor did vitamins....anything, really. I'll go for extended periods with no sleep (especially after hospitalization when I'm given IV's for everything). Along with my neurologist,, when he was alive, I decided to forego all sleeping meds. He agreed 100%. They work for a night or even two....if I'm lucky, and then that's it.

I have a better sleep cycle without any interference. If I had my choice, I'd sleep from 4:00 a.m. to about 10:00....but then that's not the way the world works. Age helps with that....retirement. I don't worry about sleep any longer, and haven't for years....I think it just makes it all worse.

I don't regret my Chiari surgery, but won't be undergoing it again with someone new. I know the problems I'm facing, and that helps. I won't know the surgeon in the same way, so would rather not use one. Remember that I'm older and that makes a difference. I also don't want to spend Medicare $$.

Good luck in your decision. I wish more people would write in to help you. @almost is a good source, also.

Yours, Lenora

 

[BaileyBeached]

Hi all,

I hope this finds you as well as you can be.

This is a long post so for those that don't have the energy or want to read a long post, I will get to the main points first.

• I have been suffering with significant long covid since March 2022 (symptoms below) and have been predominantly housebound in this time. I have however experienced bouts of fatigue usually lasting up to a week throughout my adult life. Mild CFS/ME? I cannot be sure.
• Last week following a brain and spinal MRI, I received the following results 'There is minor cerebellar ectopia with approximately 7 mm caudal descent of the tonsils at the foramen magnum. There is however no significant crowding of the neural structures demonstrated. Differential diagnosis is between a Chiari I malformation or minor cerebellar ectopia. Please correlate clinically.'
• I also present craniocervical instability symptoms but understand I would need to send my MRI images to a specialist.
• Whilst at this point in time I do not have a concrete Chiari 1 malformation diagnosis (currently sat on the fence with a differential diagnosis, from what I have researched online this may be because chiari is a tricky diagnosis and the radiographer must be very experienced in identifying chiari malformation. I will of course seek full clarification but at this point in time, so that I can further my research, I am assuming it is a chiari 1 diagnosis.
• My doctor suspects I have a connective tissue disorder, most likely Ehlers Danlos Syndrome.

My questions to anyone in the community that has any experience of a chiari diagnosis or potential treatment are;

• What is your personal experience of surgical treatment? Was it successful in the short term or to date?
• Has anyone decided against surgical treatment rather choosing other forms of symptom's relief, e.g., traction manipulation etc?
• Can anyone that has had successful treatment recommended a surgeon. I note these are two in Spain, Dr Gilete and Dr Salvador, and several in the US with Dr Bolognase receiving the best reviews.
• Could the chiari 1 malformation diagnosis be a red herring? I have suffered symptoms relating to chiari all my adult life and these have been hugely increased since my long covid onset. However, has anyone suffering with long covid and then received a chiari diagnosis attempted treatment for the chiari and their long covid symptoms remained.

Many thanks if you have read this far. I have listed my symptoms below. Those with a diagnosis or knowledge of chiari I would be really interested to know if you think my symptoms present a strong case for chiari influence (I am aware many have chiari malformation but remain asymptomatic all their life. It seems chiari malformation doesn't matter until it very much does). I will also apologise if you have read this message on other forums. I am trying to gather as much information from as many well-informed folk as I can. Thanks in advance to anyone that responds.

Take care every one

Mark

- I have a constant tightness and pain at the read base of my skull. This hurts when sneezing and sometimes completely locks out. Sometimes a very sharp pain in back of skull when sneezing and often accompanied by a strange clicking noise.
-Significant brain fog- Difficulty to process information, think and process the world around me.
-Bobble head (feel like my head is floating on my shoulders or like I am on a boat)
-Significant neck pain for the last 18 months, preventing me from being able to hold my head up for 3 months last year. Pain flares at the slightest provocation, e.g., laying awkwardly on a pillow, and pain remains for weeks and sometimes months. This has improved to the extent that I am now able to hold up my own head for an hour without resting.
-Headaches made worse by straining, e.g., going to the toilet, laughing, coughing
-Unsteady gait and poor coordination- always walking into things now
-Numbness and tingling feeling in hands and feet
-Problems with motor coordination of hand always knocking things over and hand writing is awful
-Constant very loud ringing in ears
-Vision changes- I find it hard to gauge depth of things around me which makes me dizzy
-Orthostatic intolerance with coat hanger pain
-Problems with swallowing- sometimes have difficulty swallowing food and am constantly trying to clear my throat
-Temperature dysregulation- I rarely sweat and frequently over heat
-Frequent neck injury with no apparent cause and does not respond well to stand physio and massage treatment *
-Sleep disturbance- Have not slept more than 1 hour in 18months and sleep apnea. *
-GI dysfunction, diagnosed irritable bowel syndrome *
-Electric pulse/shudder feeling down spine *
-Chronic fatigue *
- Postexertional malaise
-Lower back pain *
-I believe I also suffer from MCAS due to the symptom increase I experience directly after eating certain foods and an increase in symptoms when stopping taking my mast cell stabiliser medication (ketotifen).

The symptoms with a * show symptoms that I have had prior to my long covid journey. I have been admitted to A and E 3 times prior to long covid with horrific pain often caused during sport. Normal x rays have always come up blank. These symptoms have been troubling prior to Long Covid, in particular unexplainable constant neck pain and fatigue. This suggests to me that Covid, as is common with viral illness, has worsened the problems potentially caused by Chiari.

I don't think I have any significant information to add, but I'll share some of my issues more as moral support.

I technically have 5mm chiari but no one seems to think it's a big deal, and I'm not eager to contradict them.

I had squeezing in the back of my neck, but when I took more omega 3 this eased it up and seemed to help my circulation overall. Sadly because of mcas I can no longer take omega 3

My doctors currently think I have some cci and also that my left jugular is being compressed. I did the whole invasive traction thing, and much to everyone's surprise did not get way better, but my right eye vision improved significantly. There's a whole cluster of illnesses, most of which it seems you're aware of, that are often evaluated at the same time because sometimes they're related. As far as I know they are: chiari, craniocervical instability, Eagle's syndrome, ijv compression, tethered cord syndrome. There may be others, but those are the ones I'm aware of.

I *think* sometimes tethered cord can cause chiari, but I'm not sure.

I can't offer you details because I don't have them, but I had a friend of a friend get chiari surgery and it didn't go well. But I think same friend had a relative for which it went well.

A lot of your symptoms sound like mine except you seem to fit more neatly into a chiari/cci/Eagle's syndrome boxes. I'm apparently not in enough pain at the right places to properly qualify. One of my docs said mitochondrial dysfunction (which I have) can cause people to not have as much pain though so.... who knows. I'll take my blessings where I can find them.

From what I understand the docs I'd trust in the US to evaluated cci often are very familiar with chiari and all these other issues as well. So I'd just get evaluated by Dr B, and/or Henderson and/or Patel. I'm less familiar with Patel but I've heard good things. There are likely others with more detailed and accurate info on all of this, so feel free to add to or correct any info I shared.

I'm not exactly sure if this works for chiari, but there are some non surgical treatments for cci related to strengthening tendons. They are of course not covered by insurance. I have a vague recollection of one of my docs saying chiari could be caused by same lax ligaments/tendons. That could be completely wrong though. Like I said chiari is not my primary focus, so I don't pay as much attention to it. Non surgical treatments include prolotherapy, prp, and some stem cell stuff. Some people try bpc 157. Bpc 157 did amazing things for my mdds and brain inflammation, but bad things for my mcas.

Anyways, not sure if any of this helps. But best wishes all the same!

 

[lenora]

Hello again......I did want to tell you that prior to my Chiari Surgery, I had a lot of neck pain that extended into my arms, wrist and hands. I was constantly a medical groupie....a new doctor's office at least once/wk.

It's the arms and hands that finally made the decision. At some point in all of this, I developed epilepsy (not terribly uncommon in older people), two different types though. Anyway, I'd say I was managing OK until a seizure threw me across the room and I fractured my pelvis in a number of places plus vertebrate, of course, and the arm, wrist and hand pain is back. I believe the preferred treatment today is CCI surgery.

Please see a specialist in this country....there are a number of them. There is no need to travel long distances, although copies of your MRI's and any other tests are often read by someone like Dr. Gilet & that's a good route to take.

There are never any guarantees as to how you'll react after surgery. Unfortunately, I also developed Fibromyalgia....wretched illness. The forms for surgery scare everyone....but you aren't going to have surgery without them. I just had to overlook so many of the possible side-effects.

If possible, I would try a cervical collar and see how that will work for you. I know it's what I'd do if necessary. Times change, often within a few years, so ask questions of your surgeon, lots of questions and try to choose someone close to where you live - it makes life a whole lot easier. Also, prepare your body nutrtionally and emotionally for what's ahead. Deep breathing, even meditation help. You want to be as healthy as possible when you go into surgery. Healing is so much faster. I think that's what made my Chiari surgery so much easier. Yours, Lenora