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Chiari Malformation Community
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Avatar universal

stage 1 of chiari malformation does it get worse

type 1 chiari malformation, does it get worse and is it heredity?
309 Responses
555358 tn?1292535661
Hello,

I'm not a health care specialist, just someone that has had chiari malformation surgery and has done research, so don't take my word as gospel, but basically "yes" to both questions.

All types of Chiari are congenital - that means you are born with it. And you can pass it along. That's not to say you will for sure, I've heard the statistics are around 14% chance - not a huge number, but a number to pay attention to.

I believe there are 5 types of Chiari. The scale of severity is rated 1 - 4, with 4 being the most severe. Types 3 and 4 are very rare. You don't go from one type to another, but they can get progressively worse in each catagory. For instance if you have Chiari 1, you won't ever have Chiari 2, but what you have can get worse. If you've been diagnosed with Chiari 1 and they haven't mentioned surgery, you'll just need to keep track of any symptoms and get regular MRI's.

The most common form of Chiari Malformation is Type 1, it often manifests later in life (not at birth, but sometimes in children) with headaches and cerebellar symptoms like dizziness and memory/concentration problems. The cerebellum in the lower back of the brain starts extending down past the skull into the spine. This herniation can, and often does, get worse. If you are diagnosed with Chiari 1, you generally will be told how far the herniation extends in millimeters. At around 8 mm, doctors start considering surgery. This isn't a set number and can be more or less, often the decision is made because of possibe damage to the spine - syringomyelia ( a blockage of spinal fluid that can lead to paralysis) is often (almost always) eventually assoiciated with Chiari. Mine was at 20mm and I was starting to show signs of paralysis when mine was discovered, so I was rushed to surgery. I only knew of the existance of Chiari for 3 weeks before my surgery, some people may be diagnosed and watched for years, even decades, before they need surgery.
  
Type 2 and beyond are often noticable at birth. They often causes partial or complete paralysis below the spinal defect and generally have noticable malformed brains at least on an MRI.

Type 3 causes severe neurological defects.

Type 4 involves a failure of brain development

I've also heard of Chiari "0", which is a form that doesn't have a tonsillar herniation, but can still cause symptoms.

There, that may be a lot more info than you were asking for or need, but...I sorta got on a roll :).
570413 tn?1299536261
Can Chiari get worse?  

Yes it can, but it doesn't always.  It will not progress to from Chiari I to Chiari II, though.  You either are born with type I or type II (or 3, 4, etc.).  There are people that live there entire life never knowing that they have Chiari I.  


Is Chiari hereditary?

Yes it is congenital.
Avatar universal
chiari can get worse depending on size and etc . you may need surgery .
you can get Chiari from auto accidents etc and yes you can be born with it .
It is not a life sentence . Go to a NS not a NL . see what they say .
Avatar universal
I have alot of the symptoms of Chiari ! and finally was diagnosed. I have less than 3mm herniation which my neurosurgeon says is still in the normal range. So I was sent to a headache clinic for treatment to rule out migraines. Went to the DR again yesterday for the headaches and she said I should go back to the neurosurgeon to discuss what to do next. She said if the neurological symptoms I was having were due to migraines they would have improved along with the pain. From what I have read Chiari is rare but for it to be so rare there sure are many of us out there suffering needlessly because our doctors don't have the proper education to at least know what it is and where to send us for help. I also have suffered since I was a child and have recently become very symptomatic. While talking to my siblings there are 4 of us that are having similar symptoms one has been confirmed and the others may or may not go to find out due to money issues and insurance. My biggest problem is the headaches and the constant tingling and pain. Sleeping is the worst. The headaches come in the middle of the night and they hurt so bad. Laughing is a real issue the pain is so intense it travels to my face, jaw and numbs my tongue. I feel like I'm paralyized. I can't speak or do anything but just sit there and hope it will pass quiclky. It's so scary!! I'm glad there are people like you we can talk to. Thanks for being there for us
555358 tn?1292535661
You are welcome. I'll try to answer any questions that I can.

Have your bother talk to his Primary Care Physicain and explain to them about Chiari and point out that 2 siblings have it and he needs to get checked. This way, the PCP can order it, thus insurance should cover it. Have him print of a basic fact sheet about Chiari for his PCP to read and even place in your brothers charts. A lot of PCP's know very little if anything about it.

If this bothers or insults his PCP or he refuses to schedule an MRI . . . change doctors!

The quicker this is diagnosed, the quicker it can be dealt with. Time is a MAJOR factor in Chiari.
Avatar universal
Thanks for the info I'll pass it on!!  Maybe you could answer a ? for me. If there is little or no herniation and the MRI hasn't changed much in 10 years how is it the symptoms keep getting worse. I woke up a couple of weeks ago and my left side of my tongue was completely numb. It felt like somebody was trying to pull it out with a pliers.. the pain was unbelievable. Did you have problems when you laughed?
620923 tn?1452919248
COMMUNITY LEADER
there is a recently new description of chiari zero.....because they r finding that in some cases the amount of herniation does not mean there r 'nt ne symptoms.

when was ur last MRI?...I have seen that some on here had very little herniations and with an onset of symptoms had another MRI to find it grew!!

Good luck
Godspeed
"selma"
Avatar universal
Selma Ive read alot about Chiari 0. I know I have had almost every one of the typical symptoms since I was a kid and they have recently gotten worse. My last MRI was 3 months ago and the results showed low lying cerebral tonsils but it didn't say how low. I was told less than 3mm. The neurosurgeon said she didn't want to do surgery on a "normal" brain so she sent me to a headache specialist to rule out migraines. They were able to lessen the pain with topomax 25mg but all the neuro stuff didn't go away/ She says it deffinately is Chiari and suggested I go back to the neurosurgeon to talk about whats next. I think after reading some of these notes I 'm going to make an apointment with Dr. Frim. My sis has been in convo with you ..Rosebud3 thanks for your support!!!
620923 tn?1452919248
COMMUNITY LEADER
did u have a cine MRI?...to check for a syrinx?....also did they check for tethered cord?
I too have had symptoms since I was a child.....the brain pain from straining,laughing.....even hanging upside down.....they just sent me to a phyc....wanted to medicate me for anxiety......my most recent flare up of  symptoms started in May '07 I fell....no reason....but I sprained my left ankle and tore the minescus(sp?) so that required two surgeries...since then my balance is off, I am stressed to the max, I have tremors in my hands and numbness in my extremidies.....

Dr Frimm is very good......he is with a pediatric hosp, but many of the chiari drs do take care of adults as well....u'll be in very good hands.
how is ur sister doing? did she have her appointment?
say HI to Steve for me.

Good luck
Godspeed
"selma"
680984 tn?1241644632
Hi!  I am new to this forum, but so glad to have found it!  I feel like I have finally found others who understand what it is to go through the constant headaches, the pain, the numbness in my hands and feet, etc.  Mine has been getting progressively worse over the past 18 months or so.  I now slur my words, have been loosing my memory and stumbling alot.  I have been having a terrible time getting a diagnosis.  I have had this going on for 30 years - daily headaches for 30 years - and all I get is that I must be stressing myself out.  I KNOW that there is something physically wrong with me, but I can't get a doctor to say it.  Then I heard about Chiari and I felt like it was a gift from God.  My first appt. with a NS is on Nov 24 and I pray he finds something - anything.  I just don't know what to do if he treats me like the Neurologist have treated me.  Anyway - sorry to go on.  It is just so great to read messages from other people like me and know I am not alone - thank you.
620923 tn?1452919248
COMMUNITY LEADER
U may get the same thing u got from the NL.....because not all NS r specialists for chiari....where do u live?.....there are many chiari specialist out there, but depending on where u live u may need to travel.....I traveled an hour to hear the same thing as I got locally so I am going to where I know the experts r!!....chiari being congential means u had this all ur life and for 30 yrs of it u have suffered......sometimes it can be normal life that can cause CM to suddenly start to worsen......Have u had a dx of chiari , or suspect it's chiari?....u need an MRI and then u need to know if u have a CSF blockage...CINE MRI will let the dr know if there is ne thing other than chiaari going on.

Keep us posted
Good luck
Godspeed
"selma"
680984 tn?1241644632
Hi Selma!  Thanks for all of the words of wisdom.  The NS I am going to actually does specialize in Chiari and he was recommended in a really weird way to me through my husbands therapists because his daughter has Chiari.  I have actually had a few MRI's but no one has ever looked for this.  The very first one I had the report said one small sentence that I had a Stage I Malformation, but that was never repeated again.  Since I didn't know what that mean, and my reg dr didn't know, we didn't think it meant anything.  That was 8 years ago and since then I have not even heard the word Chiari at all.  That is why I am praying this will be a positive diagonosis for me.  I just had another MRI with contrast on Tues, so hopefully it will show up this time.  By the way, I live in a small town in Wa state, so I will be driving almost 4 hours to see this dr.  I am just grateful for this site.  I feel like I have found a whole group of people who truly understand what it feels like everyday.  Thanks.
555358 tn?1292535661
Have you been to the Virginia Mason Medical Center in  Seattle, WA? They have chiari specialist there I believe? Dr. Michael Elliott Dr. Mariko Kita.

680984 tn?1241644632
No I haven't been to Viginia Mason.  I am going to see Dr. Jacob Young in Bellevue.  He does practice at VM, though.  If he doesn't work out, I will check out the others you have recommeneded.  Thank you, Shane.
620923 tn?1452919248
COMMUNITY LEADER
keep us posted on how ur dr visit goes.

Good luck
Godspeed
"selma"
Avatar universal
I was amazed by the caring people I have found on this site too. I look forward coming home from work just to hear how others are progressing with their appointments. It's like having an extended family ...and they really care!!!! Shane and Selma seem to understand more and can explain things better and they give us newbies alot of info.

my doctors have dx me with low lying cerebellar tonsils and was told 10 years ago I had to go to Chicago to get part of my skull removed. I thought the doctor was a quack so I dismissed his findings. I have had tingling in my face, feet, hands and tongue for years. About 7 months ago the daily headaches started along with the dizziness and upset stomach. I too slurred my words and can find them sometimes. I pleaded with the doctor to please help me so he sent me to get MRIs of my brain and cervical spine. They found a non cancerous spot on my c-6 along with a bone spur. My doctor thought he found my problem and told me I had to have surgery so he sent me to the NS. The NS dx Chiari and sent me to another NS to have the surgery since he didn't do it anymore. That doctor wanted to make sure I wasn't just having migraines so she sent me to a headache clinic. They tried a few types on meds which relieved the pressure and the headaches werent so severe (although the side effects of the drugs were worse than the headaches somedays) the headache clinic has sent me back to the NS to discuss the next step. I haven't made the appointmnent yet for one I'm scared and two the holidays are right around the corner. The third and biggest one is my husband is disabled and can't work so if I go down we will be in trouble financially. I work as a manager for a large welding supply company and am around hazardous materials daily. They have been very supportive but I'm sure there is going to be a time when I'm going to be a safety hazard and I'll probably will have to quit. I'm not looking  forward to that at all!!  Anyway enough with me ... Let us know what you find out from your NS maybe you'll get the Xmas gift you've been waiting for... I was so relieved when I finally found out there was something wrong and I wasn't really crazy!!!   Hope you feel better
Avatar universal
My 5 year old son was diagnosed with a chiari 1 in September with an 8mm measurement. They told me that he should be at least at 10mm before it causes him problems. He does have a short neck because he has some short vertabraes and fused vertabraes in his neck as well. We just went back to the neuro because he is complaining of his legs constantly either they feel like people are running all over them, they are asleep or they just hurt. my son told the neuro that he fell down the stairs and the neuro decided to order an mri of the spine now. He is constanly holding the back of his head, telling me things don't look right (he said he sees two of things, the neuro said he can't see two of things since he is blind in his left eye), constant leg pain, ocassional hand pain and arm pain, slurred words that we attributed to him being so tired all the time, he raises one side of his mouth when he smiles or talks (started in the last 6 months). Can these symptoms happen or progress that quickly? I am not happy when they are deciding treatment based on a number. They say becasue he can still function he is fine. The child has been through at least 15 surgeries in his 5 years of life so far....he has a high tolerance for pain. What do you guys think?

Any help would be greatly appreciated. I need to understand what he is feeling and how to help him.

Thank you and best wishes to all. It is hard to watch my son moan and groan in his sleep night after night, I can't imagine how everyone here feels as well.
Julie
620923 tn?1452919248
COMMUNITY LEADER
Julie, it appears u were to see a NS that is not a chiari expert.U need a specialist for ur son.
We have a thread with names of chiari drs members have used....if u don't find one close to u , we can help u locate one. U may find that u might have to travel to get to one.
A chiari specialist will know that chiari without a herniation can cause issues.

I don't know what else u son has endured, but I would deff find a chiari specialist.

Please continue to update us on ur son's condition, and know u can post here for support there r other parents here u can ask how they r managing.

Good luck
Godspeed
"selma"
Avatar universal
i have diagnosed chiari malformation 1 six month ago and I'm on Dimax but i'm having sevear back pain. I leave in San diego area please could you help.
555358 tn?1292535661
Diego,

What are you needing?
I'm not familiar with Dimax, is that a pain killer?

Do you have a Chiari Specialist in charge of you case?
620923 tn?1452919248
COMMUNITY LEADER
Welcome to the Chiari forum.....as Shane asked is ur attending dr a chiari specialist? It is very important that u have an expert.

I am not familiar with Dimax either......

I hope u continue to post here, it's a great place for info and support!!

Good luck
Godspeed
"selma"
Avatar universal
my 7 yr old was diagnosed with a 15 mm chiari type 1 and recommended for surgery not to be pushed off, the doctor also mentione da possible need of fusion, that really goot me scared when he told me the fusion would cause her to loose 40 percent of her neck movements in all directions. does anyone know about chiari being associated with the need of fusion, or the riskd s of not taking a fusion.
HELP
620923 tn?1452919248
COMMUNITY LEADER
Hi and welcome .

15 mm is considered a big herniation.The fusion ur r askin about is for cranial instability....one dr explained it  as if u put a pumpkin on a pointed stick....it will not be able to stay at the top....it will slide down....this is y they do the fusion.

I believe many with this also have EDS...Ehlers Danlos syndrome...it is a connective tissue disorder and it is common in those with chiari.
My one friend has it and if u would like more info please let me know.

I know these conditions r very scary and overwhelming, but we r here and will help ne way we can.

Godspeed
"selma"
Avatar universal
Thank you,
the funny thing is my daughter does not haver any symptoms or pain or chiari symptoms (nor any eds symptoms) we just took the mri because of scoliosis appearing.
The "pumpkin on a stick" won't that get cured after the chiari surgery,
Do all Docs that treat chiari perform the fusions i heard that they tend to stay away from fusion do to the risks and results involved, if a fusion is necessary does that mean she is in  risk of god forbid the pumpkin falling off the stick if not done rigth away,
meanwhile i will be taking more mris to see the severity of the level of fusion if necessary, we are praying that it wont be necesarry, because that would make her an "invalid" in a way for life..
Were looking into Dr menezes of Iowa or Dr Michael Scott of Boston's children hospital...
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