Re-Intro & Chiari

October 14, 2002

Hi all...

I believe I should re-introduce myself, this time with my real name -

my name is Eurico and I'm from Canada. I posted a few weeks ago about

Chiari I. I have a 9 year old child who's had 2 surgeries in the

U.S....one for Chiari I malformation. We consulted with a ton of

specialists over a period of 5 years or so regarding her many

progressive neurological symptoms, which forced us to travel to the

U.S. Several specialists told us she did NOT have Chiari I. Two said

otherwise and decompression surgery alleviated her many symptoms,

proving the many wrong (some dozen or so neuroradiologists,

neurosurgeons and neurologists).

A few months ago, I saw a post at MGH by Deborah Setzer. It mentioned

diagnoses of PD, MSA and Chiari I, the latter of which was deemed

insignificant. I recommended to Deb and to consult with a

Chiari expert at North Shore's Chiari Center (being renamed to Chiari

Institute) and not to rule it out as they had been told it was

probably an incidental finding. As some of you may know, there is

suspicion that Deb's problems may be Chiari related, although it's not

sure yet if it's Chiari per se, but perhaps elevated ICP, often found

in Chiari patients - perhaps we'll know more in a few months after Deb

has been detoxicated from her meds and I hope she finds the root cause

of her problems.

You must know that Chiari I malformation can cause a multitude of

possible symptoms and combination of symptoms, including those

demonstrated by MSA, PD, MS and PSP. I've seen a small number of such

MRIs and most showed some cerebellar tonsillar herniation (which is

pretty much the only criteria used by most doctors in diagnosing it -

in fact, most doctors will only consider it significant to symptoms if

the herniation is significant, usually more than 5mm below the

foramen magnum, the hole in the skull where the cranium and spine

meet). The tonsils are two lobes which exist at the inferior section

of the cerebellum - when there is sufficient compression, these

tonsils are often forced down into the spinal canal, restricting

proper CSF (cerebrospinal fluid) flow and compressing on neural

tissues and even arteries.

Milhorat et al (from North Shore in Manhasset, NY) published " Chiari I

malformation redefined " May 1999, in a peer-reviewed medical journal,

Neurosurgery. They have found that tonsillar herniation is ONLY ONE

factor required to diagnose it and that the 5mm herniaton threshold

required to diagnose it is not accurate. In fact, our child had no

tonsillar herniation (0mm) and Dr Milhorat diagnosed Chiari I back in

May 1998, almost 5 years since she first started experiencing

symptoms (they didn't all come at once, either). We obtained a

concurring diagnosis from Dr Frim in Chicago as well, another

neurosurgeon. There are very few doctors who know about these new

findings, understand it and more importantly, accept it. The cynicism

probably stems from the fact that doctors learnt about Chiari I one

way in med school ... now someone is telling them years later that

what they know and what they've been telling people for years is

incomplete and inaccurate.

If you look at the MRI picture and " Opening Screen Illustration " text

at the bottom of the following page, you will see that Milhorat

performed Chiari surgery on a patient without any tonsillar herniation

and the patient had alleviation of symptoms:

http://www.neurosurgery.org/focus/july01/11-1-nsf-toc.html

This is basically stating the 5mm tonsillar herniation criteria is

wrong. My child is also proof of this.

My child did NOT have the classic tonsillar herniation for Chiari I

but Chiari surgery alleviated 20-25 symptoms. Some Chiari patients

have only a couple of symptoms; some have in the order of 30 or so;

some have more and some have less. Chiari I is basically compression

in the lower back part of the skull called the posterior fossa (it's

believed that an underdeveloped occipital bone causes less space for

all that stuff in there, secondarily causing compression) -

the posterior fossa consists of the cerebellum, brainstem, arteries,

CSF cisterns and other neural tissues - compression in the area of the

human body that controls pretty much everything. Think about it.

People who do NOT exhibit at least 5mm tonsillar herniation will not

be diagnosed with " significant " Chiari I by most docs - most such

patients are either told it's insignificant or it's an incidental

finding or not told at all because the doctors think it cannot cause

any problems. Let me tell you - they are wrong! I've been a member of

a large Chiari forum for 5 years now and have read many stories of

patients who have minimal herniations of the cerebellar tonsils, many

of whom end up travelling to one of the few experts who understand and

believe the new criteria and many end up with alleviation of symptoms.

Many of these people go years being diagnosed with other disorders

before finding out their root cause.

I'm not suggesting you all have Chiari I, nor am I suggesting any of

you do. However, Deb was diagnosed with it and her problems may be a

result of it or something related - I've seen another MSA patient's

MRIs and I saw minimal Chiari - I recommended this person consult with

a Chiari expert and the feedback from the doctor suggested Chiari I

and this person has been asked to get more imaging done to help

confirm the diagnosis (don't ask me who as I've been asked to keep

this quiet for now.

I feel it's important that you know about this new redefinition of

Chiari I - just because a few doctors looked at your head/cervical

spine MRIs and said nothing was abnormal, it doesn't mean they're

right. We found out the long and hard way....however, it was the best

thing we did as it saved our child - she was experiencing progressive

life-threatening problems.

I'm sure some of you may think this is all hog-wash (you may say,

" I've had tons of some of the best doctors in the world look at my

MRIs and they didn't say anything about Chiari " - yes, I know - we,

too, consulted with some of the top experts across North America and

they missed it too). Well, you're entitled to your opinions - that's

fine. But so am I. What's most uncomfortable about me doing this is

that I don't really know the people on this list and feel like I'm

barging in. However, I know two people on this list and both seem to

have Chiari or something Chiari-related. That's enough for me to tell

you this. It's up to you whether you want to explore this as a

possibility for your problems. MSA, as far as I know, and like many

other syndromes, is diagnosed pretty much solely on symptoms. So what!

Chiari I can mask itself as many syndromes, one reason why it's so

often misdiagnosed. And if seen by a true expert, it can be confirmed

radiologically.

I have seen quite a few MRIs of Chiari patients over the years.

Chiari I is very often misdiagnosed and it often takes patients years

before being properly diagnosed with it.

I'd be happy to answer any questions regarding this. Regards,

Eurico

October 14, 2002