Need Advice Chiari Malfromation

Brain and nerves
1

Hi all, a little back story. For years I have had pain at the base of my skull. It would come and go, never gave it much thought, just put it down to bad posture at work. I could not lie flat with my head on a pillow as I know I would have pain the next day. To be honest it never impacted my life that much. The pain level was not too bad, most times a five, at times an eight.

This went on for years, on and off until recently. I play tennis and started to get dizzy turns, the room did not spin it more felt like I would fall over. Legs felt weak, at times I had to leave during games. The dizziness is now constant. The pressure and pain came back in the head, this time it wont leave, it's been months. It's a constant dull pain, does not increase with coughing etc. The pressure comes and goes, seems to hit most after I stand up from desk at work. I also have pains between my shoulder blades, which can be intense at times.

I have seen an ENT, they did VNG tests and found an upbeating torsional nystagmus on moving me down to flat position which stopped when flat. They diagnosed Bppv, did all the Epley maneuvers, saw Physio, none of it helped. Went to see Neurologist, had MRI done, they found a borderline Chiari Malformation that they feel may be incidental. Waiting for follow up appointment so I can try and request a Cine Phase MRI.

Went back to the ENT, he now says nystagmus could be Chiari related, very confusing. Sorry for the long winded back story, now for the questions.

- I thought nystagmus associated with Chiari was down beating?

- Do my symptoms sound like Chiari?

- If I have a Cine Phase MRI and it shows CSF flow is normal and no syrinx, does that mean my borderline Chiari is not causing the symptoms?.

- When I wake up in the morning the dizziness is there but the head pressure and pain is barely noticeable. The head pressure and pain seems to increase as the day goes on. The worst days are when I am inactive sitting at a desk. Going for a long walk seems to help a bit, does this fit with Chiari?. I thought exercise would make it worse.

- I live in Maine, are there any good Chiari specialist in the State?.

Thanks in advance for all of your help and advice.

2

Even if you get diagnosed with a borderline Chiari, that doesn't mean the symptoms aren't significant.  The Cine MRI should show if there is any CSF flow blockage. Nystagmus is one of the symptoms of Chiari. 

- I thought nystagmus associated with Chiari was down beating?  Don't know what you mean by down beating. 

- Do my symptoms sound like Chiari?  Yes

- If I have a Cine Phase MRI and it shows CSF flow is normal and no syrinx, does that mean my borderline Chiari is not causing the symptoms?.  

No. If you have borderline Chiari and there isn't a herniation of tissue, you can still have all of the symptoms. Remember- your cerebellum is being pushed up against the brainstem. Even if the tonsils aren't pushed through the foramen magnum, the fact that they are being pushed at all is indicative of a lack of space in your skull. 

- When I wake up in the morning the dizziness is there but the head pressure and pain is barely noticeable. The head pressure and pain seems to increase as the day goes on. The worst days are when I am inactive sitting at a desk. Going for a long walk seems to help a bit, does this fit with Chiari?. I thought exercise would make it worse. 

Dizziness varies, but some days it can be worse than others. I think a walk and fresh air can be helpful with any sickness.  Stretching your neck or doing some kind of exercise can certainly help.

- I live in Maine, are there any good Chiari specialist in the State? Don't know. Check into your local is a universary neurology/neuroscience program. See if they are connected to a hospital. 

In my experiece with Chiari, doctors really don't know much about it. You really have to pressure them to get in with a doctor who actually does decompression surgery.  I've been through numerous doctors and many of them don't do enough of it. 

Make sure to research everything. Get copies of all tests. The only treatment for Chiair is surgery. If you don't want to do that, you can use drugs for symptoms, but they won't make the underlying condition go away. 

Good luck!!

3

Hi! Just wanted to make a comment about borderline CM.

I have 3mm cerebellar herniation, 5 neurologists over 2 years said it was incidental, couldn't be responsible for all my symptoms.

I saw a neurosurgeon this September, he diagnosed me with CM I after one cine MRI. Had craniocervical decompression surgery 3 weeks ago. Haven't had headaches or any other symptoms for two weeks already.

My point is few mm herniation can cause significant problems as it was in my case and then there are also those with large herniations and no symptoms.

About symptoms-first things you need to understand-there are no two patients alike.

In your case there are some things which point to CM but there are some key points missing. You have to get the cine MRI results. If there's CSF flow obstruction then there's no more doubt about the diagnosis.

Take me for instance-gradually, over 5 years, increasing pressure headaches at the back of my head-they became more often and severe. When I had my pain episodes I basically couldn't move-I felt like turning my head will make it split open, not to mention standing up or lying down. If in 2011. I had a 12-24h pain episode once every two months then in September 2016 I had headaches every day or 6 days a week lasting from 8-24h. That was the main thing, but as my headaches got worse over the years the fatigue, diziness, arm and feet tingling, cold shivers with normal body temperature, nausea and vomiting appeared. Ringing in ears and floating ink blots in front of my eyes with the worst pain episodes. Insomnia had become a normal thing for me.

And about walks-until winter 2014/2015 when the fatigue became too severe I was walking 8-10km 3-4 times a week. I didn't give up those walks because they made my symptoms worse. I gave up gym training because it made my symptoms worse. In my case anything that put strain on my shoulders and neck aggravated the headache and other symptoms accordingly.

And I tried a lot of different medication over last two years-nothing helped with the pain. Not even slightest relief. That was one of the major arguments why my NS suggested surgery.

I wanted to make a short comment but I always do this-I can't contain myself. I'm pain free for two weeks now, but the memories are too vivid. It was my own personal hell. I apologise for the blabbing.

4

Thanks for all the info Lineel.

Interesting what you say about the cerebellum being pushed against the brainstem even though the tonsils aren't pushed through the foramen magnum. What I find weird is I have had two MRI's, one from 2011 and one from 2016. Whoever read the MRI's said the borderline Chiari had not changed bewteen the two scans. If that is the case why would I suddenly have more symptoms now?. Chiari is very confusing in how it presents itself.

The dizziness is strange, the ENT thought Bppv, did the Epley several times, it didn't help. What does seem to help is the Brandt Daroff maneuvers.

I have decided to go down to Boston and see a Neurosugeon to get his advice. I  am guessing my case is going to be a case of waiting and seeing what happens. 

Thanks again for your advice.

Emis Moderator comment: I have removed the link as users can easily find the information on downbeating nystagmus using a search engine rather than linking to a specific website. If users want the specific link use the Private Message service to exchange.

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5

Hey Madara, thank you so much for sharing your story. I am going to see a neurosurgeon down in Boston, hopefully he will send me for a Cine Phase MRI. You are right that if there is CSF flow there will be no doubt about the diagnosis. 

With your pressure headaches, did the pressure feel like it ran right down the middle of the neck, starting at the base of the skull?. The ink blots you talk about, do you mean floaters?. I have had those for years, they have increased over time, I have veils of them in my vision. Luckily our brains learn to tune them out. 

I hear you about exercise, I feel that I am finding it hard to push through tennis. I am determined to not give up, I am adjusting my serve and in general taking it easy. One good thing is I started tennis about two years ago, I had two MRI's five years apart and the herniation never changed. So hopefully that means tennis is not affecting the herniation. 

Thanks again Madara, glad to hear you are doing well!

6

Thanks, Tim

About the headaches - they started like a muscle tension going up from my shoulders, to the back of my neck, to the back of my head. The tension turned into pressure at the base of the skull and it sort of went up to middle part of my head, and stopped deep behind the eyes if that makes sense. When I moved my head or tried to sit up/stand up immediately the pressure started to build up at the base of the skull, it went up to where I described, and it expanded behind my eyes. It felt like presure waves with every heartbeat. It makes sense because CSF is produced/reabsorbed with every heartbeat if there's a CSF flow blockage, it sort of hammers the brain with every heartbeat.

About those ink blots - you got the point right, English is a foreign language for me, so I didn't know they were called floaters. But I had them and tinnitus (ringing in ears) only when the headaches were really severe, usually I'd get them with those long pain episodes lasting longer than 12h.

And about giving up the gym - working out did nothing to the herniation. It acted as a trigger for the headache.

CM was starting to seriously affect my life back in summer of 2014. I had a head CT in July 2014, it showed 3mm herniation, then as my symptoms progressed and I went from one neurologist to another, over the last two years I had two MRI's and another CT - always the same result. The NS who diagnosed me and did my surgery ordered additional cine MRI, it showed the same 3mm herniation and the CSF flow blockage.

I really hope they'll find some other reason for your headache, the one you can actually cure.

CM is no fun. Nobody deserves it. Yes, I feel good, but it has cost me a lot - too much of my time has been spent in pain, in appointments, in hospitals, and bloody hell - I had to have a brain surgery to get the quality of my life back. Oh, and, let's not forget - symptoms can come back. So - fingers crossed you don't have CM  

7

Thanks for the description of your headaches, I definitely have the pressure at the back of the head, doesn't go through to the eyes luckily. Did you ever get an increase in the headache when coughing, sneezing etc?. For the past two nights I have slept on an incline and that seems to help a little bit. My symptoms definitely get worse as the day goes on, starts out mild and by the end of the day the pressure is pretty bad. Did yours do that?

From what you say I am definitely going to push for a Cine MRI, got a referral today for a neurosurgeon in Boston.

I too hope my symptoms are not down to Chiari, keeping my fingers crossed as well. Thanks again for all of the advice.

8

Yes, coughing, and sneezing made the pain worse.

I would wake up somewhere between 8-9AM feeling tired, but no pressure in my head, until 2-3PM usually the pressure had built up, headaches started and they would go on until the night, sometimes until the next morning.

9

Symptoms may get worse just because your cerebellum is getting squished- you may have some blockage of CSF flow. Symptoms may also be due to some other disease. My symptoms got worse very suddenly and the CINE MRI showed blockage on one side.