Why Are Chiari Malformations So Difficult To Being Accepted By Doctors?

[anneliese]

Hi CCF,   Recently, MSNBC Dateline did a story on a woman having to turn to the world wide web for help getting a diagnoses/treatment of Arnold-Chiari Malformation type 1.  She went through alot of specialists everyone knew nothing that Chiari Type 1 causes problems.  She finally had her Chiari decompressed after getting help on a forum recommending a doctor who specializes in this rare disease.  

I had to go through the same thing trying to get help making contacts with other patients and doctors looking for someone who could tell me if it was chiari.  Had to switch my health insurance two times  inorder to have treatment done.  Sorry to go on so long but it took me six years to get treatment and now no guantees about if the wait for treatment caused permanent damage to my spinal cord.

I hope this Dateline episode alerts both medical community and people in genaral about it and how to get treatment.  I just will never understand why it is so difficult to diagnose even if it is rare.  But, I do want to Thank CCF and MedHelp for their assistance over the last couple years trying to find answers.

Maybe more research will be done in this area.

Thanks, Anneliese

[CCF Neuro[P] MD, RPS]

thanks for the comment.

CCF Neuro MD

[Dave G.'s http://www.chiaripaper.cjb.net]

Chiari Malformation Information Page
http://www.chiaripaper.cjb.net

Available in 5 languages:
English, Spanish, French, German, Italian

Read Dr.Milhorat's MEDLINE Paper (May 1999)
for free in that page or read it now, click:
http://www.chiaripaper.cjb.net/milhorat-english.htm

Tables and charts available too,
http://www.chiaripaper.cjb.net/


Other recommended sites:
http://i.am/chiari
http://www.asap4sm.com

[CCF Neuro[P] MD, RPS]

Dear Joseph:

A Chiari I malformation is where the posterior fossa is small and the tonsils of the cerebellum and the medulla protrude into the spinal canal space.  This is not a lesion caused by a truamatic accident, you are born with it.  I would find another reason for the hypertension.  The migraine headaches might be the result of his accident, trauma induced migraine headaches.  

Sincerely,

CCF Neuro MD

[Joseph]

Friend with facemask injury and symptoms = similar to Arnold-Chiari Malformation Type I?
     While scubadiving a friend of mice received a strong kick to the face mask (tremendous pressure on orbits). Shortly thereafter he has experienced persistent sensitivity to light, constant migraine headaches and increased blood pressure. Could the pressure from the blow to the face mask have transferred pressure to the brainstem area causing problems with blood pressure and poor CSF drainage (accounting for migraines and sensitivity to light). Any suggestions welcome in this tremendously life-altering condition (his condition has not changed since the injury years ago). Thanks much.

[CCF Neuro[P] MD, RPS]

Dear Ned:

I hope that it will be the cure.

CCF Neuro MD

[ned]

Hi CCF,
I wanted to update you.  I had a third ventriculostomy instead of the shunt.  The operation was performed by Dr Ward @ MCV in Richmond, VA with Dr Young.  They both saw the success rate as a viable option for me.  I had the surgery on 3/2/00 and so far so good!!  

http://www.nsc.vcu.edu/
Medical College of Virginia (neurosciences center)

http://www.nsc.vcu.edu/physicians/ward.html
Dr John Ward - Director of neurosciences

[CCF Neuro[P] MD, RPS]

Dear Martha:

A Chiari type I malformation is a congenital malformation (you are born with it) and not a trauma induced entity.  A protrusion of 5 mm is borderline at best, the texts usually mention 6 mm as the diagnostic criteria.  But, 1-2 mm may be measurement error.  I am not sure how a mild buldge would cause nerve root damage without compression of the spinal cord itself.  So, I cannot explain why you would have symptoms.  Changes in balance problems can be inner ear problems, sensory problems, or posterior column problems (spinal cord).  That would have to be worked out in a good neurological exam and ENT exam.

Sincerely,

CCF Neuro MD

[Martha]

Hi
I was recently diagnosed with a ChiariI malformation via MRI.5 mm
below the foramen magnum.
The MRI also noted minnal diffuse bulging C3,4,5 through C6,7, with some nerve root damage.
There is a fluid signal1x1.5 in the posterior aspect of the cerebellum hemisphere. Some sort of arachnoid cyst in the left side or encephalomacia. Mucocele left sphenoid sinus.  
My problems started after a car accident in which a deer came through the window causing a loud explosion type sensation within
the vehicle.
My symptoms include back head and neck pain, loss of sensation
in areas of my left arm, balance problems mainly when my eyes are closed.  If I tilt my head up,I fall frankly left. I feel my personality drastically changed after this accident and work constantly with short term memory problems. Driving in the car
causes the most discomfort but need to drive alot due to my job.
The doctor said these things are not related to the accident. I am having a difficult time with this since I had no symptoms before.
I had a normal CT scan and MRI 12 years ago for employment exam.
I am following with a ENT for more tests for a possible tear in the vestibular area.  I hurt and open to any sugesstions.

[CCF Neuro [P] MD, RPS]

Dear Ned:

With a structural reason for your hydrocephalus, I think your only option is a shunt as suggested.  Unfortunately, people who read films may not be neurologists or neuroradiologist.

Sincerely,

CCF Neuro MD

[ned]

I have been diagnosed with aqueductal stenosis and hydrocephalus.  It took a caring primary care physician and a good support system (family) to help pay for non-covered physicians.  I was diagnosed with a blockage in the aqueduct of sylvius.  The doctors say my only option is to have a magnetically adjustable shunt placed in my head.
I wanted to know if there are any other options.
I had two neurologists tell me it was a pulled muscle in my neck causing the pain.  I was precribed zanaflex, oxycontin, buspar, trazodone, and accupuncture.  I was appalled that 4 different doctors looked at the same films and saw different things.
I would suggest anyone with difficult dianoses talk to the doctor's I ended up with:

Neurologist Sidney Mallenbaum,MD in Virginia Beach, VA [phone]

Neuro-Surgeon Warren Foer, MD in Virginia Beach, VA [phone]

Neuro-Surgeon Robert Young, MD at Mediacl College of Virginia in Richmond, VA

[CCF Neuro[P] MD, RPS]

Dear Kevin:

Hydrocephalus is when the CSF fluid is not being absorbed properly,  This could be due to blockage of the CSF or due to abnormal reabsorption of the CSF.  A spinal tap would show increase pressure (hydrocephalus caused increased CSF pressure) and also papilledema of the optic discs (opthalmological exam) would also show increased CSF pressure due to hydrocephalus.

CCF Neuro MD

[kevin p]

What exactly is hydrocephalus? And im not sure i was actually diagnosed with it .. like i said i had the C.A.T. Scan the it was reported on the scan the doc seemed kinda confused .. but then told me that i had it .. and that i should come back on monday, that they would go over my symptoms and try and figure things out ..
I cannot have an MRI i still have clips in my head from the tumor operation when i was younger.. So i would imagine this might be real hard to confirm ?
thanx for the input
KevinP

[CCF Neuro[P] MD, RPS]

Dear Kevin:

It is very difficult to accurately diagnose a Chiari I type malformation based only on a CT scan.  The MRI is the essential test needed as the definition of the Chiari I is the downward lie of the cerebellar tonsils in the spinal canal, measuring 6 mm below the base of the brain.  I would not trust a CT to give a diagnosis.  The usual symptom of a Chiari I is hydrocephalus.  For the vast majority of people, it is symptomless.

Sincerely,

CCF Neuro MD

[kevin]

hello could someone please tell me what Chiari Malformations are in great detail .. i just had a C.A.T. scan and was diagnosed with it .. my doctors dont have any idea what could be causing it.. i had a pituitary tumor when i was 2. im now 29 never had any reoccurances of it.. and there is still no signs of a tumor.. But im scared, They told me if they didnt find out what was causing it that it could cause paralysis and all sorts of other lovely things .. i ve been living in pain for the past 2 years with no relife and all along i thought it was a back prob.. but they tried to diagnose me with everything from anxiety.. to erratic heart beats and so on .. anyone with info on this please leave me a msg or email me at ***@****
Thanx for any replies in advance
Kevin

[CCF Neuro[P] MD, RPS]

thanks for your comments.

CCF Neuro MD

[MSG To Bonnie...From Anneliese]

Hi Bonnie,  Sorry, Missed your message earlier at this site. The CCF Doc gave a great suggestion on getting a second opinion. Though, I would even try asking your primary to reffer you to a neurosurgeon for evaluation for he/she could look at your Mri Scan...My ACM type 1 was not easy to get diagnosed until I went to a neurosurgeon who sees these over just your regular doctors.

I had several radiologists/neurologist agreeing and disagreeing over it being a true Arnold-Chiari Malformation Type 1 and being symptomatic-Which most docs will tell you they are not symptomatic.  I never gave up and a few docs orginally wanted to label me with that PsycoSomatic Illness bit but I would not let them do that!!  Be persistent about your help care!!  

Try visiting the website at http://egroups.com  

Take care, Sincerely Anneliese

[CCF Neuro[P] MD, RPS]

Dear Bonnie:

I hope you don't mind me jumping in here.  The MRI would show if you had a Chiari I type malformation.  Both the radiologist and neurologist should have picked up on it if it was there.  It does sound like your stuck in your health plan.  You may want to think about self pay, that might be the only way to get a second opinion.  If you do decide on this, I would gather all the information, take it with you and go and find out if a new pair of eyes can help decipher what is going on.

Sincerely,

CCF Neuro MD

[Bonnie]

Anneliese,
     I watched Dateline too and was facinated by this disorder.  I have made yet another appointment with a neurologist in an attempt to diagnose what two other neurologists cannot.  I've had 2 MRI scans, a CT scan, visits with an ENT, my family doctor, a psychologist, numerous blood tests and 2 visits with an orthopaedist.  No one had any idea what was wrong with me.  Four years ago I started having pressure and burning on the top of my head followed by numbness and tingling on the right side of my face and body.  The numbness caused drooling.  Since that time, I have severe dizzy spells, blurred vision, incontinence, unexplained joint pain and swelling, pain and stiffness at the top of my spine near at the base of my neck (I have not been able to turn my head to the left for 18 months) and many more symptoms.  The first neurologist I saw ordered an MRI.  It showed white spots on my brain, but said everyone has white spots.  She asked me if this might be related to my "mood problems".  I am 43 years old.  When I was 17 I was diagnosed with depression.  I saw an associate of hers and was asked why I was in when I saw his associate two months prior.  I explained that she was unsure what this was and I wanted a second opinion.  He was clearly upset and I felt as if I was wasting his time.  He went through my symptoms and ordered physical therapy for my neck. He had me see an ENT to rule out an inner ear disorder.  It was all clear. He also suggested psychiatric care and I was evaluated by a psychologist who wanted to do an MMPI.  I was told by this neurologist that I would have to live with this.  He assured me there is nothing wrong with me as there are no physical symptoms.  I saw an orthopaedist who does not have a clue why my left ankle is double in size and pained.  He referred me to his associate who had no idea what is wrong with it either.  He suggested I get psychiatric help as this might be a somatic disorder. After watching Datelline, I called the neurologist and asked if the is could be ACM1.  She said, "I think not.  I would have caught that."  I asked her if she was looking for ACM and was told no, she wasn't.  I have been happily married for 25 years and have four children.  I have been in therapy off and on for different issues, but know this is not a somatic disorder.  My husband has seen the numbness that the doctors write off as they cannot see it when I'm in the office.  I told them I have no control when it happens, so I cannot command it to happen.  The physical therapist called the neurologist in because she was concerned about my carotid artery.  She felt something wrong in my head and side of my neck.  He came in for a few seconds and said it is just fine.  I don't even know what a carotid artery is, but I felt I got better care from the physical therapist.  My problem is the symptoms are worsening.  My insurance only covers this group of neurologists and my attempts to see others have failed.  How do I keep the doctors from writing this off as a psychiatric disorder?  My family doctor of 30 years knows me and is quite concerned about this.  He cannot refer me out to another neurologist as it is not allowed through Health Partners Insurance.  I have made another appointment with the neurologist, but fear they will not take me seriously.  Any suggestions?

[CCF Neuro[P] MD, RPS]

Dear Carrie De Quattro:

My initial impression is that there is a nerve problem and not a problem of a bone graft site.  I would definitely see a neurologist and there are a couple of good ones at CCF in Florida.  Maybe an EMG to define where the lesion might be.  Then I would make sure the MRI is up-to-date and see where both the image and EMG point as far as where the lesion is located.  Medication will manage the pain only so well (as you know by experience).  If the scar tissue from the last operation is part of the problem, it may be that another operation to reduce it might be a option.  If the spinal has undergone further degeneration then another surgery might help matters.  However, one does not know until everything is looked at.  He is on a very low dose of neurontin, and before starting a morphine drip, I would go up on the dose of neurontin.  We have used up to 6-7 grams per day for cancer pain.  Get a good evaluation before making a decision.  I wish you luck.  As a former teenager drummer who loved jazz, I really hope that your husband can get back on the skins and light up the house.

Sincerely,

CCF Neuro MD

[Carrie DeQuattro]

DEAR Doctor,
In 1989 my husband had a laminectomy at L5-S1 due to an automobile ramming his vehicle from behind.
The bone was taken from right hip and scar tissue has now caused my husband to be in chronic pain.
His Pain Dr. is recommending Morphine pump but he is very relluctant to this procedure.
My husband is a world renowned drummer in the blues and jazz arena,however due to this debillatating condition he can no longer support our family and has resorted to Social Security for assistance.
My husband is only fifty two yeras of age and has alot of music to make but as you know this condition makes it impossible.
He is now having to use a walker and if I am not with him he falls often due to loss of feeling in right leg and pain in foot.
all of the pain is radiating from the hip where the bone was taken for graft.
Here are impressions from last MRI dated 09/09/98
   1. grade 1 to 2 antertior Spondyolisthesis with faintly visualized residual pars intra-articularis defect with post surgical fusion changes identified.
there is stable anterior positioning of L5 onto S1 in both flexion,extension and neutral projections.
   2.Increased lumbar lordosis that might predispose to strain and instability.Degenerative disease with vacuum phenonmenon
L5/S1 level.
My husband is currently recieving 120mg.MS Contin per day along with
400mg Neurontin per day also Clonazapan for sleep and Zanex to keep calm.
We are a christian family and are praying that my husband gets relief and know that I have to search out medical help wherever possible.
We have had a multitude of diagnosis however are very leary  as this is a matter of his being back on the drum stool making great  music or not.
As you can imagine it is very heartbreaking to see a man who was traveling all over the world playing with all of the greats in the music industry,playing at such Venues as the Montreax Jazz festival,San Francisco Jazz Festival and so many more,to now using a waler or crawling on the floor at home and  getting no sleep for crying out in excrucioating pain due to scar tissue around the Sciatica.
Please Sir if you have any suggestions that can help it would be greatly appreciated.
We live in florida and there is a Cleveland Clinic in Ft. Lauderdale, however we are willing to travel anywhere to get help.
                        Sincerely  
                        Carrie De Quattro

[CCF Neuro[P] MD, RPS]

Dear Annieliese:

It doesn't matter what I think.  What you need to do is concentrate on your condition and your recovery.  Do the right things for your recovery (which will likely be unique, for all health problems are usually individual).  What I or someone else might do in our practice, at this point is not germane to you.  You need to recover, gain back your life, and then push on and live your life as full and to the best you can.

CCF Neuro MD

[Is It Because Symptoms Reappear After De]

Hi CCF,  Thanks for the quick reply.  Wandering if your feeling towards decompression for Chiari is because the surgery is not 100% at resolving symptoms and because it is the brain stem that you have to deal with, I guess not an area to mess around.  

I don't know what the statistics are on dealing with patients with  ACM regards to for decompression?  Is it better to just deal with the progressing symptoms and figure this condition is not operative and basically physically disabiling condition.  I kind of feel that even though I had surgery 8 mos ago I only had a temporary relief of my neurological problems.  Now, Symptoms are coming back on me.  Alot of other patients who I keep in touch with are getting the same results.  We are all scrambling to get relief and going from one specialist to another and are still faced with problems.  I work in a teaching hospital and can see both sides of the picture.  But, being the patient and there are days that I worry that I either end up in a wheel chair or worse.  It hard for me to get anyone to tell me straight out what to expect.  Will I end up paralysed or have breathing problems due to me brain stem being compressed.  My past attacks where being told I was going to have to be put on a ventilator because of Respiratory Failure were very frightening!!  So could you tell me if this condition if symptomatic leads to terminal condition.
The nurses where I work are amazed that I am still living with this condition.  I gather brain stem compression is not a good thing?

Thanks for your help, Sorry to sound on the negative side but my condition is getting worse. I wait to be scheduled for another Flow study.  If not good next approach is a shunt or more decompression and dural plasty.

Thanks , Anneliese

[CCF Neuro[P] MD, RPS]

Dear Anneliese:

Thanks for your comments.  But, the truth is that most Chiari I malformations are non-symptomatic and are not a problem.  Many patients who have MRI of their brain for other reasons have Chiari I malformations and have no symptoms.  There are some patients who are taken to surgery without proper CSF flow studies and therefore no documentation that the Chiari I is causing a problems, but they do develop a problem after surgery.  So, the issues are not as black and white as one would believe.  

CCF Neuro MD