Chiari Online Support Group

Neuro won’t look at Chiari I & says there is no Chiari 0

#1

These are my 20 year old daughter’s symptoms…
(* for symptoms which seem to support Chiari)

  • Born with mild encephalopathy due to hypoxia at birth according to birth records.
  • Adopted by me at 11 months old from Russia so no family medical history.
  • Neuro consult at 3 yrs old because I was concerned about fetal alcohol syndrome (FAS).
  • Diagnosed with *mild celebral palsy, mild encephalopathy, *sleep apnea, and ADHD. Only had Fetal Alcohol Effect, not full syndrome.
  • MRI with and without contrast on 3/14/2002 showed *Borderline Cerebellar Tonsillar Ectopia.
  • Sleep apnea corrected with tonsillectomy and adenoidectomy to give more breathing room in her throat which might also IMHO have helped negate any future swallowing problem.
  • Diagnosed with mild *scoliosis at about 12 or 13 years old.
  • Began having *afternoon headaches around 11 yrs old. Was told a result of ADHD meds. Also started being *dizzy when rising and was told it was normal at puberty and would adjust.
  • At 15 had first full blown “migraine” (as was diagnosed) with dizziness, nausea, and vomiting. It lasted 2 weeks. Went back to same neurologist who saw her when was 3 yrs old. He suspected Chiari & did second MRI without contrast on 2/22/2014. MRI results read: No Chiari malformation identified. On flexion and extension and neutral position, CSF sagittal flow imaging at the foramen magnum, there appears to be CSF flow seen along the anterior and posterior margins of the foramen magnum.
  • 7/9/2014 Second sleep study done. No obstructive apnea observed.
  • Saw this neurologist for four visits over 8 months but then insurance changed. I just got his records this last weekend and by the last visit on 10/15/2014, he writes that even though the MRI was negative he still needs to rule out progressive Chiari.
  • In next two years she would experience more low level headaches spiking to the 2-3 week migraines (as they were diagnosed). No specific meds would help, they would just end.
  • 2/15/2017 she fell down steps at home hitting back of her head on the stair.
  • 2/15/2017 began a headache that over two years later has never ended! Started out at pain level 5 and has to progressed to normalized pain level 7 - 8 with some weeks being 9 - 10.
  • Barely finished high school in May 2017 with Hospital and Home program.
  • Has bouts of *dizziness, *nausea, *vomiting, and most of time usually *fatigued. Can no longer have a social life & has basically lived in her room for two years.
  • 3/01/2017 Admitted to UCSF for 5 days of DHE infusions every 8 hours - No relief.
  • 4/20/2017 Occipital Nerve Block - no relief
  • 5/10/2017 MRI w/o contrast read: The craniocervical junction and other midline structures demonstrate no abnormalities. It was done at the same place and compared to the 2/22/2014 MRI.
  • 6/22/2017 30 Botox shots - made it much worse.
  • 7/31/2017 admitted to UCSF for 5 days of Depacon infusions every 8 hours - No relief.
  • 8/16/2017 saw holistic chiropractor who made a *neck adjustment that greatly relieved the pain for 2 - 3 weeks however pain came back and no further adjustments helped.
  • 1/29/2018 admitted to UCSF for 5 days of Thorazine infusions every 8 hours - No relief.
  • 5/2018 Transferred to UC Davis Neurology adult headache specialist. He has only prescribed different meds to try - none of which has worked.
  • Bending over or laughing makes headache worse.
  • Laying down does not make headache feel better. Ice to back of head soothes headache.
  • Approximately 6/2018 her *left shoulder started hurting. Primary Care doctor said pulled a tendon. Has now progressed to tingling down her arm to her pinky finger.
  • 6/15/2018 UC Davis doctor prescribed Aimovig which is the first migraine medicine aimed at lowering a brain chemical called CRGP which is overproduced in migraineurs. This looked like it worked because it lowered the headache for a while but over 6 months the result didn’t hold.
  • Been to ER 15 times in 2 years for headache pain but minimal help with Toradol IVs.
  • Saw primary care dr for *hurting left shoulder and now *tingling in both arms. She did a biceps reflex test and both arms responded too much. Dr said indicated brain overreacting. Also asked her about Chiari but she deferred to the neurologist.

Messaged UC Davis neurologist last week asking him to do an MRI to check for Chiari I and Syringomyelia in case of Chiari 0. He says because last two MRIs were negative for Chiari he will not order a new MRI (even though last MRI was a year and a half ago and headache has lasted for two years!). He says there is no Chiari 0!

Well? I felt really good when we finally discovered Chiari and felt it all falls into place! Doctor says to remember that “many symptoms are not specific and can be totally seen with migraine”. Whatever that means. Really? Is scoliosis seen with migraines? Is sleep apnea seen with migraines? Is shoulder pain and nerves tingling seen with migraines? Is NO response to an Occipital Nerve Block or Botox seen with migraine? Is no response to migraine meds seen with migraines?? NO migraine medicine has worked. NO epileptic seizure medicine has worked. NO new CRGP migraine medicine worked. NO over-the-counter pain med works and has not given my daughter any pain medicine in two years due to rebound headaches.

Sorry I’m going off the deep end here but we are pretty sick of my daughter being sick and not finding a doctor willing to go out on a limb to rule things out. Any encouragement or even discouragement of running after Chiari would be greatly appreciated.

Barb

#2

Hey Barb,
Welcome to Ben’s Friends
Technically speaking there is no Chiari 0 but there is Chiari1 and Chiari 2. Their full title is Arnold Chiari malformations. Here’s a write up on the 2 differing conditions https://www.neurosurgeonsofnewjersey.com/arnold-chiari-malformation-type-1/

I’m sorry to say but your experiences with the medical profession are not unique. The paradigm of “We are the dr’s, so we know it ALL” is very common and neurology, from my experience, is one of the worst for this concrete train of thought. Anything outside of the ‘normal’ train of thought or not within their education is automatically rejected and don’t you dare question a neuro, they will damn near crucify you for the thought.

Some people will not like me saying that, but from my experience as a neuro patient who has questioned, ohhhhh, they do not like that one little bit. And me being me, I wanted answers “What on earth is going on with me?” I stirred the pot a bit too much and the medicos DID NOT LIKE IT ONE LITTLE BIT!!! I was labelled a hypochondriac, that was until THEY, THEMSELVES diagnosed an issue. I’d been telling them for years, decades even that there was a problem. But it wasn’t until I had a major (Emergency) situation that they investigated further. They came out with 'Ohh look what we found…" I was SO VERY ANGRY. ‘Rage’ just does not explain it adequately.

I am not a Dr, so I am not going to give you medical advice but:-
I’m in Australia and our medical system is a bit different to the U.S., we have universal health care, similar to Obamacare and it works quite well. I won’t say it’s perfect but if you need medical care, it’s there.
Here we have GP’s (General practitioners similar to your PCP), then we have physicians, then we have specialists. GP’s are OK for general (‘normal’) healthcare needs and they can refer you onto specific specialists. But for unknown or undefined conditions it can be a bit of a lottery. For undefined conditions a physician is the dr needed. Their role is like an investigator. They take a list of symptoms and find out what is going on. Then they can refer you on to the specific specialists once diagnosis’ have been confirmed. From the information you have provided it sounds like you are dealing with a few issues and not solely neurology based, but also physiological issues. You need someone to take a broad overview, a more wholistic approach. Your daughter coming from Russia, there may also be environmental factors which also need to be taken into consideration, possibly such factors that very few American dr’s have ever seen, let alone had to manage. I would suggest that you may need a medical advocate to oversee the whole process.

The benefit of using a medical advocate is that they a emotional disconnected from it all. And please do not take this the wrong way, it is not meant to offend. But you are a parent, adoptive or not, you are still her parent. As a parent you have an emotional connection, some medicos can be very quick to write parents concerns off as being over protective or emotionally based . But a medical advocate has enough disconnection to maintain a professional view and obtain an outcome minus emotion. They also know the medical systems, how they work and how to achieve a desired outcome within that specific system. In my opinion, You NEED a medical advocate. Here’s some general information on medical advocates
https://www.ahrq.gov/patients-consumers/patient-involvement/health-advocate.html

Now I can see that you are in California, but that’s a fairly large state, so I’m going to provide you with some avenues for finding an advocate by state rather than city.


https://www.ca.gov/agency/?item=office-of-the-patient-advocate
https://cahealthadvocates.org/

I do hope those links can assist you in pointing you in the right direction and hope you can find the answers to the questions you are looking for. Please do keep us informed and if you need further assistance then please ask.

Merl from the Moderator Support Team

#3

Thank you! I’m on it. Found an advocate in our town with a doctor’s degree and a chronic illness herself so she may be able to help. Just gotta find out how much will cost.

#4

To be completely honest, it doesn’t sound like CM to me. Chiari has many very generalized “nonspecific” symptoms that make diagnosis difficult. Many conditions are like that. If she has had multiple MRIs with nothing definite for CM, I think it is unlikely that this is Chiari. I am not a physician, but I do have CM and I am a nurse and I have over 8 years working with neuro patients., including Chiari patients. The shoulder pain with radiation down the arm sounds like something is pinched to me. I feel like, all things considered in your note, that this isn’t Chiari, but something else. I hope you find answers.

#5

Thank you nfekete. The neuro won’t consider Chiari because of the two negative MRIs, so ok. I still would like Syringomyelia looked at but for some reason he’s digging in his heels and won’t order a spinal MRI even though she has scoliosis which hasn’t been measured since about 2014 when it was deemed Risser Stage 4. Doing a spinal tap on Apr 15 which I guess at least he’s checking other things out but could it hurt to get a spinal MRI before you go do a spinal tap??? I feel it’s because I’m looking around and asking about Syringomyelia.

Thank you all for your help of looking into Chiari as my daughter continues to live in her bedroom!

#6

Hello I just want to compliment you on the notes you have taken through your journey to find help for your daughter. Those notes along with the suggestion from ModSupport on a patient advocate have great potential to help find a solution.
I am uncertain if any of the MRIs your daughter has had was a CINE (sin-ee) MRI. These are often denounced by some people and that is common in all aspects of medical testing. I however have experienced their benefit as has a business professional living in my area.
CINE MRIs can detect blood and cranial fluid flows at the base of the brain and if there are flow voids then surgery is sometimes recommended. Insurance companies often do not approve them especially if other standard MRIs do not detect any compression.
Chiari is so hard to pin down with all the symptoms that can present; I never had headaches yet many do. Perseverance does help, but do not over expect of doctors they are as human as we are and I say that even after being treated really bad by some.
One day at a time and you have already found out using cool packs at the back of the head help and so do other simple things: like not overexerting, no stimulants, chiropractic care, and massages. These may work for some, but not all and you must remember that we mentioned what has helped us, yet you and your daughter make your own choices.
Maybe some day you will be able to pass along some of your successes in your journey to all of us here at this Website.
Best to you always!

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#7

Radiologist will only write Chiari on the report if it’s over 5mm
Mine was 6 and only ONCE I had it listed on my MRI report. And I had taken MRI every year and every 2 years since I was 11 years old due to pituitary tumor.
So my advise find a good radiologist. MRI can be taken anywhere, is radiologist that counts for the initial diagnosis. Today, Chiari size doesn’t matter any more. My daughter has 3mm with some symptoms but never diagnosed by radiologist. My neurosurgeon through special program measures her head and her brain. And knows whether brain fits in the skull or not.
Chiari has many symptoms. I had lots of the ones you wrote about. There is no set number however there is many that repeat. You also have to look for other disorders that do affect and are connected to Chiari and makes Chiari progress. Also take a look on the 2 type of surgery they offer. There are doctors that believe that the brain should stay intact and those that once herniated brain will never function again. I went with dr. Bolognese, the second approach and everything either got reversed or I learned how to live with and it doesn’t really bother me. Good Luck and don’t stop fighting.

Also, no neck cracking for anyone with Chiari you gonna make things worse. Deep tissue massage, opening facial for blood flow and nerve relieve. Adhesions will press on the nerve causing numbness, tingling and more.

#8

Hello all! I posted my daughter’s MRI results in my other post but wanted to repost here due to different contributors helping me. Hope that’s OK.

My daughter had her MRI on Sunday where I wrote on the forms to please report ANY Chiari measurements and it worked! The radiologist reported a 3.8 mm left and 4.4 mm herniation on right side. Doesn’t seem to read that there are any flow voids. We are so excited to have some numbers and a name for her two years of suffering. This is a Chiari headache not a migraine caused by her brain over producing CGRP as we have been told from day 1 at UCSF neurology and continued with UC Davis whom I gladly drove 4 hrs to because I thought I’d be getting cutting edge diagnoses from! Not! I had to drive this MRI which the doctor canceled because how dare I try to think outside the CGRP box!

OK, so taking a breath, we have an appointment with our neurologist on Tues and we accidentally have a new patient appointment with her primary care physician. The PC will be necessary for a referral to a neurosurgeon. We want that appointment. So…from the neurologist, I hope he affirms Chiari 1 but am worried he wants to stay with the CGRP story. Am I asking for a CSF flow study, a CINE MRI even though this brain MRI shows no flow voids? He probably doesn’t like CINE MRIS either. Canceling the spinal tap next month although he should NOT want to do that anymore, right? It can make Chiari worse from what I read. Any thing else to ask for to move forward on a Chiari diagnosis? An upright MRI?

What about pain medicine? She hasn’t had any pain medicine in two years due to rebound headaches but she’s in 5 - 10 level pain every day.

Thank you all so much! Your words stay with me and guide me in my attempts to help my daughter. Barb

#9

I presented the brain MRI results which showed 3.8 mm and 4.4 mm herniation to my daughter’s PC and she is referring her to a neurosurgeon. Thank you all for your support!!

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#10

With those symptoms, you need to find her a neuro that will look closer. It appears that’s what sh has.

#11

I mentioned it briefly in another post, but you might want to explore some gentic testing:

https://medcraveonline.com/JNSK/JNSK-07-00245

If you don’t want to wade through it basically it says the world index double for those of Russian and if any one in the family has experience headache there is a 90% occurence to second degree relatives… With no family history with and adoptive kiddo, the only way to know is to test. given some of the other health issues she has had, I’d be very suspicous (sleep Apnea and encephalopathy are high on the list…)

Mutations in the CACNA1A, ATP1A2, SCN1A, and PRRT2 genes are the most common but there are others.

in any event we have found through all of the Bens Friends communities a commonality in genetics and “Rare Disease” that several of us are attending the Global Genes RARE patient Advocacy Summit early this fall along in addition to the Orphan Drug Rare Diseas converence next month.

We are with you Barb - truly and as sytorong an advocate you are for your daughter I KNOW you will get answers soon.

#12

Can you please tell me how to get genetic testing? Will doctor do it or myself? I’m down for it because we don’t know any family history. Thanks!

#13

Well for once you have lucked out! UC Davis is one of the world leaders in Genetics (granted it is horses and Strawberries - more later) Genetic testing is not done by a home kit (23 & me etc) but by a real geneticist. Katherine A. Rauen, M.D., Ph.D. at UC Davis wa who my geneticist buddy told me he would take is child to. I don’t know her but he spoke very highly of her and her entire department. hard for me to sy anything good about UCD not for their medical department but rather their football team. THAT is a horse of a different color. Speaking of horses that is where UCD really excels, interestingly research in horses is the basis of most genome research. That and strawberries. believe it or not Strawberries have more DNA chromosomes and basic genes than practically any other living thing. UC Dvis was just awarded a MASSIVE contract to develop new types of strawberries (from england) that will fund their whole genetics department for years…

#14

Wow! We finally get cut a break! According to Nadia’s insurance online Dr Rauen is in network! Will call on Monday. Primary Care new patient appointment is on Weds where I’ll have to convince her. Neurologist on Tuesday where I’ll try to be good and play nice to get more testing. :wink: Surely he will have the sense to not do the Apr 15th spinal tap even with her < 5 mm herniation, won’t he? She is scheduled to get a spinal tap to celebrate spring break! Did not know about horses and strawberries! :smile:

#15

I’ve forgotten to say in all of this that my daughter has never had “migraine with aura”. It’s always just classified as a migraine. Plus let’s keep in mind that this “migraine” has NEVER stopped. Don’t migraines stop and start?

#16

There are several forms of migraine in a class called Intractable. One of the “requirements” is that they do not
Respond to medication/treatment.