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This FAQ is intended
for informational purposes only and in no way represents an attempt to provide medical advice.
This information may or may not apply to your case and anyone with a question or
concern about their health is strongly encouraged to consult with a medical
professional.
After you've reviewed
the FAQ, please provide us with your Feedback.
Chiari
Malformation -
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What is a Chiari Malformation?
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Is Arnold-Chiari different from Chiari?
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What are the symptoms?
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Does the size of the malformation matter?
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How is a Chiari Malformation diagnosed?
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What is a cine MRI?
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What is a borderline Chiari?
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How did I get this condition?
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I don't have any symptoms, but an MRI shows a malformation.
What does this mean?
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Does Chiari run in families? Will my children get it?
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Is there a genetic test to see if someone has Chiari?
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How is Chiari treated?
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How do I know whether to have surgery?
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What is the surgery like?
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Is the surgery always successful?
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What are the risks of surgery?
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What will happen to me if I don't have surgery?
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How long will it take to recover from surgery?
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How can I find a doctor with a lot of Chiari experience?
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I had surgery, but I'm still in a lot of pain. What can I
do?
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Am I eligible for disability?
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How many people have Chiari?
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I'd like to talk with other people who have this. Is there a
support group?
Syringomyelia
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What is syringomyelia?
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What causes syringomyelia?
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Why does a syrinx form?
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What is idiopathic syringomyelia?
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What are the symptoms of syringomyelia?
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Does syringomyelia cause paralysis?
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How is syringomyelia diagnosed?
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I have a syrinx but don't have any symptoms, what does this
mean?
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How is syringomyelia treated?
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What is the surgery like?
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Will I get better after surgery?
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What happens if I don't have surgery?
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How long will it take to recover after surgery?
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What is neuropathic pain?
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Am I eligible for disability?
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How is post-traumatic syringomyelia different from Chiari
related syringomyelia?
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How many people have syringomyelia?
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Is there a support group for syringomyelia?
Chiari -
1.
What is a Chiari Malformation?
Chiari Malformation Type I (CM) is a neurological disorder where part of the
brain, the cerebellum (or more specifically the cerebellar tonsils), descends out
of the skull into the spinal area. This results in compression of parts of
the brain and spinal cord, and disrupts the normal flow of cerebrospinal fluid (a
clear fluid which bathes the brain and spinal cord). Return
To Top.
2.
Is Arnold-Chiari different from Chiari?
Not everyone uses the same terminology when describing Chiari. Some
people use Arnold-Chiari (ACM) interchangeably with Chiari. Others only
use Arnold-Chiari to refer to Chiari Type II which involves more of the brain
descending out of the skull, is predominantly diagnosed in children, and is
commonly associated with Spina Bifida. Other terms for Chiari include tonsillar ectopia
and hindbrain herniation, meaning the cerebellar tonsils are out of position.
Return To Top.
3.
What are the symptoms?
Because Chiari involves the nervous system, symptoms can be numerous and
varied. In fact, one large study showed that the vast majority of Chiari
patients reported 5 or more symptoms, and 49 distinct symptoms were reported by
2 or more patients. Despite this variety, the most common Chiari symptom,
and the hallmark of the disease, is a headache. Usually, the Chiari
headache is described as an intense pressure in the back of the head and is
brought on, or aggravated, by exercise, straining, coughing, sneezing, laughing,
bending over, or similar activities. Other common symptoms
include balance problems and fullness in the ears. In very young children,
trouble swallowing is one of the most frequent symptoms. When thinking
about symptoms, it is also important to keep in mind that once a person's health
is compromised in one way (with Chiari for example), secondary problems are more
likely to develop, especially if a person is in chronic pain. Research has
shown that people with chronic pain are much more likely to develop other
chronic conditions, so not every symptom may be a direct result of a Chiari
Malformation. Return To Top.
4.
Does the size of the malformation matter?
Traditionally, Chiari Malformation has been defined as the cerebellar
tonsils descending more than 3-5mm out of the skull. However, research has
shown there is no real correlation between the amount of descent (or herniation)
and clinical symptoms. Some people with herniations of less than 3mm are
extremely symptomatic and some people with quite large herniations are symptom
free. Because of this, doctors are now focusing on whether the
cerebellar tonsils block the normal flow of cerebrospinal fluid (CSF). The
current theory is that disruption of CSF flow is a more important measure than
the size of the herniation. Return To Top.
5.
How is a Chiari Malformation diagnosed?
An MRI (Magnetic Resonance Imaging - a non-invasive test which uses a large
magnet to create a picture of internal organs) can clearly show if the
cerebellar tonsils are out of position. However, since the definition of
Chiari is changing, most doctors will use a combination of reported symptoms, a
neurological exam, MRI results, and their experience and judgment to determine
if a person has Chiari. Unfortunately, there is no single, objective test
which can clearly say that someone has a Chiari malformation which is causing
problems. Return To Top.
6.
What is a cine MRI?
Cine MRI is a type of MRI where the machine is programmed to measure the
flow of cerebrospinal fluid (CSF). Doctors use this to see if the
cerebellar tonsils are blocking the normal flow of CSF from the brain to the
spinal area and back. While many doctors now consider cine MRI to be a
routine test in diagnosing Chiari, some experts question it's usefulness and are
reserving judgment. Return To Top.
7.
What is a borderline Chiari?
Since Chiari was traditionally defined based on the size of tonsillar
herniation, a borderline Chiari can refer to when the cerebellar tonsils are
descended only a couple of millimeters out of the skull. Alternatively,
borderline Chiari can refer to someone with mild symptoms which may not be
directly attributable to Chiari. Return To Top.
8.
How did I get this condition?
Chiari was originally thought to be a congenital condition - meaning you are
born with it. While this may be true for many people, published case
studies have also demonstrated that Chiari can be acquired and even reverse
itself if the source of the problem is removed. It is not known how many
cases are congenital and how many are acquired. Complicating the situation
is that for reasons that aren't clear, some people develop symptoms as children,
and some people develop symptoms as adults. What triggers symptoms is not
fully understood. Return To Top.
9.
I don't have any symptoms, but an MRI shows a malformation. What
does this mean?
This is sometimes referred to as an incidental finding. Someone has an
MRI for an unrelated reason, but it shows a Chiari malformation - meaning the
cerebellar tonsils are descended - yet the person has no Chiari type symptoms.
As MRI's become more common, this is happening more frequently and is
one reason that diagnosing Chiari can be difficult and can not be based on an
MRI alone. Return To Top.
10.
Does Chiari run in families?
An ongoing study at Duke University has identified more than 100 families
where two or more members are affected by Chiari. This implies that for
some cases there is a genetic basis for Chiari. It is not known, however,
what percent of cases may have a genetic component. In other words, this
does not mean that the family of everyone with Chiari is carrying a Chiari gene.
Return To Top.
11.
Will my children get it? Is there a genetic test to see if someone
has Chiari?
A Chiari gene has not yet been identified, so there is currently no genetic
test. Return To Top.
12.
How is Chiari treated?
If the
symptoms aren't severe, doctors may recommend just monitoring the situation with
regular MRI's and treating the symptoms individually. However, if symptoms
are interfering with quality of life, are getting worse, or if the nervous
system is being impaired, doctors may recommend surgery. The most common
surgical treatment, performed by a neurosurgeon, is known as decompression
surgery (see details in Question 14). An alternative surgery involves
placing a shunt (a tube like device) to channel the flow of CSF and relieve
pressure. Return To Top.
13.
How do I know whether to have surgery?
The decision whether to have surgery is up to each individual and their
doctor. Some of the factors that are considered are the severity of
symptoms, whether the symptoms are getting worse, whether the nervous system is
being compromised, whether there are any complicating issues, and the surgeon's
own experience and judgment. Unfortunately, there is no single, objective
measure to say whether someone should have surgery and many patients will find
that different doctors may have different opinions. Some doctors are more
aggressive in their treatment approach and some are more conservative. A
recent survey about when to recommend surgery showed that there was general
agreement among surgeons in the extreme cases - no or mild symptoms, don't
operate; severe, progressive symptoms or syringomyelia, operate - but there was
little agreement in the middle. In one of the survey's hypothetical cases,
the surgeons were split almost evenly down the middle on whether to operate or
not. Return To Top.
14.
What is the surgery like?
Decompression surgery is a general term used to refer to any of a number of
variations on the same basic procedure. The goal of the surgery is to
create more space around the cerebellar tonsils and restore the normal flow of
CSF. The procedure involves removing a piece of the skull in the back of
the head near the bottom (craniectomy). Often part of the top one or two
vertebra are also removed (laminectomy). At this point, depending on the
individual case and doctor, some doctors will also open the covering of the
brain, the dura, and sew a patch in to make it larger (duraplasty). There
are many variations in how the surgery is performed, including (but not limited
to) how much bone to remove, whether to open the dura, what type of material to
use for a dural patch, whether to shrink or remove the cerebellar tonsils, and
whether to replace the missing piece of skull with anything.
Unfortunately, there is no consensus, and no strong evidence, on which technique(s) is the best. Because of this, it is important for patients to
understand specifically what their surgeon will be doing and why. The
procedure itself lasts several hours and most people will spend a night in the
ICU and an additional couple of days in the hospital. Return
To Top.
15.
Is the surgery always successful?
As with any surgery, the chance of success depends on the individual case,
so each person should ask their doctor what their chance of having a successful
surgery is. It should be noted that success can mean different things to
different people, so it is best to ask specific questions such as what are the
odds I will be symptom free; what are the odds I will be mostly better; and what
are the odds I will get worse.
Unfortunately, there
is not a lot of strong surgical outcome research, but there are enough reports
to get a general idea of the overall success rates. For patients with just Chiari
(no syringomyelia), up to 50% become symptom free after surgery, with another
10%-30% improving significantly. On the flip side, for 10%-20%, the
surgery will be a failure and they will likely require additional surgeries.
Keep in mind these are not scientific numbers and each patient should discuss
their own chance of success with their doctor.
Return To Top.
16.
What are the possible complications of surgery?
This is another question that is important for every patient to ask their
doctor so that they fully understand the risks and potential outcomes of surgery.
Many of the complications of decompression surgery have to do with
opening the dura and research has shown that opening the dura does increase the
complication rate. There is a risk of infection and sometimes the patch
that is sewn in leaks or becomes scarred. A more serious complication -
not necessarily related to opening the dura - occurs when the brain slumps further into the spinal area after the surgery.
Return To Top.
17.
What will happen to me if I don't have surgery?
The natural progression of Chiari - as doctors call it - varies from person
to person and is not well understood. For example, why do some people
develop symptoms in their 30's while others have symptoms their whole life?
For many people with no or mild symptoms, the symptoms will not get worse and
surgery will not be necessary. However, there are also anecdotal reports
of symptoms becoming rapidly worse, sometimes after a sneeze or a fall. If
a patient does not have surgery, many doctors will recommend monitoring the
situation with routine MRI's and neurological exams.
Return To Top.
18.
How long will it take to recover from surgery?
As to be expected, recovery will vary from person to person and will depend
in part on a person's overall health and fitness before the surgery.
Barring any complications, some people recover from a successful surgery in a
few weeks, others take a few months, and others may take more than a year. Your
doctor may suggest a physical rehabilitation program to regain strength and
flexibility in your neck and may refer you to a physiatrist - a doctor of
physical medicine and rehabilitation. One factor that people sometimes
overlook during recovery is that if they were inactive due to severe symptoms
for a long period of time prior to surgery, they will need time to regain a
general level of strength and conditioning. Return To Top.
19.
How can I find a doctor with a lot of Chiari experience?
The American Association of Neurological Surgeons (AANS) does not recognize
Chiari as a sub-specialty. This, combined with liability issues and the
difficulty in establishing expert-level criteria (what does it take to qualify as an expert?)
make it difficult to put together a list of Chiari experts. Each person
must find a doctor they are comfortable with. Some people like to see
university based researchers, some would prefer a regular neurosurgeon; some are
willing to travel for surgery, others aren't; some want a surgeon they can
relate to, others think surgical skill is more important. When trying to
find a doctor, some things to consider are
how many Chiari surgeries they do a year, how many total surgeries they do a
year, are they up to speed on the latest thinking on Chiari, how they relate to
patients, and what type of reputation they have among patients and the medical
community (this is by no means comprehensive). There is no right answer to these questions; they are just
intended as a way for a patient to feel comfortable with their doctor. One
way to find a doctor is to ask around. Ask people in your community, ask
any medical professionals you know, or go on the internet to find what you are
looking for. Return To Top.
20.
I had surgery, but I'm still in a lot of pain. What can I do?
One possibility is to see a pain specialist. A certified pain doctor
will perform a thorough examination to determine the exact cause of your pain
and may recommend therapies such as acupuncture, trigger point injections, over
the counter medications, or prescription medications. Unfortunately,
neuropathic pain - pain caused by damage to a nerve - can be very difficult to
treat. Anti-seizure drugs, like Neurontin, work for some people but can
have strong side effects. Many Chiari patients have found that they must
try different things and see what works best for them.
Return To Top.
21.
Am I eligible for disability?
Many people with Chiari have qualified for government disability. Some
people have been able to get disability easily, while others have had to fight
for it. The American Syringomyelia Alliance Project (www.asap.org)
has put together a form to help people with Chiari and syringomyelia get the
benefits they deserve. Return To Top.
22.
How many people have Chiari?
There is no exact answer to this, because a rigorous study to determine this
has not been performed. Once thought to be rare, the increased use of
MRI's has shown that Chiari is much more common than originally believed.
Confusing the issue is the question of how you define Chiari. Many people
may have cerebellar tonsils that descend out of the skull, but they have no
symptoms and probably never will. Studies have shown the incidence of this
tonsillar ectopia may be as high as .5%-.7% of the general population.
However, this does not mean that all these people have Chiari. Estimates
for the number of people with true Chiari range as high as 500,000 in the United
States. A more conservative estimate of 300,000 would mean that 1 in 1,000
people have Chiari, or 0.1% of the population. Return To
Top.
23.
I'd like to talk with other people who have this. Is there a support group?
Conquer Chiari has built an on-line meeting place for people in the Chiari
community to confidentially connect. It is not a message board, but rather
a way to meet people like yourself, exchange contact information, and develop
supporting friendships. The meeting place can be found at:
On-line Meeting Place
In
addition, there is a Yahoo based message board at
http://health.groups.yahoo.com/group/chiari/.
Return To Top.
Syringomyelia -
1.
What is syringomyelia?
Syringomyelia (SM) is a neurological disorder where a fluid-filled cyst -
or syrinx - develops inside the spinal cord. This cyst can grow over
time, causing the spinal cord to expand and stretch nerve tissue.
Eventually, the syrinx can cause permanent nerve damage and paralysis.
Return To Top
2. What causes syringomyelia?
One researcher noted that syringomyelia is not really a disease unto itself,
because it is always the result of something else. By far, the most
common cause of syringomyelia is a Chiari malformation. Although there are
no strong statistics, about 30%-50% of people with Chiari also have
syringomyelia. Syringomyelia can also form after a spinal cord injury
(SCI), such as from a car accident or fall; this is called post-traumatic
syringomyelia (PTS). PTS can form months or even years after such an
injury. Syringomyelia can also be the result of a tumor or mass in the
spinal cord. Return To Top
3. Why does a syrinx form?
Over the years there have been several theories as to why a syrinx forms
(mostly dealing with the effects of a Chiari malformation on cerebrospinal fluid
flow), but none have been universally accepted or proven. One current
theory states that the cerebellar tonsils act like a piston and beat down into
the spinal area with every heartbeat. This piston motion then forces
cerebrospinal fluid (CSF) into the spinal cord itself, where it forms a syrinx.
However this theory does not account for evidence that the pressure inside a
syrinx is higher than outside and that syrinx fluid does not exactly match CSF.
Return To Top
4. What is idiopathic syringomyelia?
In rare cases, there is no discernable cause for why a syrinx forms; this is
referred to as idiopathic syringomyelia. Recent research has shown,
however, that even in some cases of idiopathic syringomyelia there is crowding
at the skull-spine junction, even though there appears to be no Chiari
malformation. In these cases, the surgery used to treat Chiari has been
successful in treating idiopathic syringomyelia. Return To
Top
5. What are the symptoms of syringomyelia?
Because the syrinx is putting pressure directly on nerves, the number one
symptom associated with syringomyelia is pain. Many patients report severe
pain in the neck, upper back, and shoulders. Doctors refer to this as the "cape effect" of
syringomyelia - meaning pain in the area where a cape is draped over the
shoulders. For patients with a syrinx located in the thoracic region of
the spine, the pain may be in their chest, stomach, or lower on the back.
In addition, many people with syringomyelia lose strength in their arms and legs
and develop numbness in their hands and feet. Additional symptoms include
trouble regulating body temperature, abnormally stiff muscles, and loss of
bladder and bowel control. Return To Top
6. Does syringomyelia cause paralysis?
Yes, an active syrinx can eventually lead to paralysis.
Return To Top
7. How is syringomyelia diagnosed?
An MRI (Magnetic Resonance Imaging) exam of the spine can clearly show the
presence of a syrinx. If a Chiari malformation is found on an MRI, usually
an MRI of the entire spine will be ordered to look for syrinxes.
Return To Top
8. I have a syrinx but don't have any symptoms, what
does this mean?
For reasons that are not well understood, some people can have a syrinx -
even a large one - and not have any symptoms. While some people will stay
symptom free, cases have been noted where people become symptomatic suddenly and
deteriorate rapidly. Return To Top
9. How is syringomyelia treated?
Because of the risk of permanent nerve damage, if symptoms are significant
or progressing, most surgeons will recommend surgery of some type. The
treatment for cases where there is a syrinx but no real symptoms is more
controversial. Some surgeons will recommend surgery anytime there is a
syrinx, while others will take a wait and see approach and monitor the situation
with routine MRI's and neurological exams. Return To Top
10. What is the surgery like?
For Chiari related syringomyelia, the most common procedure is a Chiari
decompression (see Chiari Q14). Alternative procedures
include placing a shunt (a tube like device) to divert cerebrospinal fluid
around a Chiari malformation, or trying to drain the syrinx by placing a shunt
directly into it. Return To Top
11. Will I get better after surgery?
Unfortunately, there is not a lot of good, long-term, surgical outcome
research which adequately measures a patient's quality of life after surgery.
For Chiari related syringomyelia, surgery will reduce the syrinx, or at least
stop it from growing, up to 80% of the time. However, this does not
always translate into a significant improvement in symptoms, and many people
still experience some pain - and other symptoms - after surgery. There is
currently no way to identify beforehand who will get better with surgery and
what symptoms will improve. However, some research has indicated that the
longer someone has symptoms before they receive surgery, the less chance they
have of a successful outcome. It is important for every patient to discuss
the possible surgical outcomes, in detail, with their doctor, so they know what
to expect. Return To Top
12. What happens if I don't have surgery?
The natural progression of syringomyelia is not well understood. For
people with symptoms and an active syrinx, the syrinx may eventually lead to
paralysis. For people without symptoms, however, the future is less clear.
A recent study followed 11 people with syrinxes, but no symptoms, for more than
10 years. Of the 11 subjects, only 1 eventually developed symptoms and
required surgery. The decision to have or not have surgery should be
carefully discussed with your doctor. If surgery is not performed, the
doctor may recommend closely monitoring the situation with routine MRI's and
neurological exams. Return To Top
13. How long will it take to recover after surgery?
Recovery will vary for each individual, will depend in a large part on
whether there is permanent damage from the syrinx, and in some cases can take
years [Ed Note - I still feel like I am getting better and stronger 5 years
after my surgery]. What many people don't realize is that even with a
successful surgery, the syrinx may take up to a year to collapse and may not go
away completely. In addition, many people will have had symptoms for years
before surgery, so while some things will improve quickly, others may take years
to improve. Often recovery can be a series of ups and downs, with
long periods of improvements punctuated by temporary setbacks.
Return To Top
14. What is neuropathic pain?
Neuropathic pain refers to pain that is caused by damage to the nervous
system. Unfortunately, neuropathic pain is all too common among
syringomyelia patients and can be very difficult to treat. There are many
articles on this website about different ways to cope with pain.
Return To Top
15. Am I eligible for disability?
Some people with syringomyelia have been successful in claiming disability
benefits. The American Syringomyelia Alliance Project (www.asap.org)
has put together a form to help people with Chiari and syringomyelia get the
benefits they deserve. Return To Top
16. How is post-traumatic syringomyelia different
from Chiari related syringomyelia?
Up to 25% of spinal cord injury patients will develop a syrinx months - or
even years - after the initial injury. The mechanisms underlying the
syrinx formation are not well understood and PTS is difficult to treat.
Surgical options include correcting any bone deformities in the area, creating
more space around the syrinx, and draining the syrinx with a shunt.
Unfortunately, less than half of PTS patients show long-term improvement after
treatment. Return To Top
17. How many people have syringomyelia?
Syringomyelia was once thought be extremely rare with early estimates of
only 20,000 people in the Unites States having it. Increased use of MRI
has since shown that syringomyelia is more common and, while there has not been a
rigorous study to determine how many people have it, a Duke University analysis
estimates that between 100,000 - 200,000 people in the US may have
syringomyelia. Return To Top
18. Is there a support group for syringomyelia?
Yes, the American Syringomyelia Alliance Project (www.asap.org)
offers an on-line message board and several regional support groups.
Return To Top
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