newly diagnosed overview

Disclaimer: This presentation is intended for informational purposes only and may or may not apply to you. The editor and publisher are not doctors and are not

engaged in providing medical advice. Always consult a qualified professional for medical care. This publication does not endorse any doctors, procedures, or

products. © 2003-2005 C&S Patient Education Foundation

Feeling Lost?A quick overview of Chiari &

Syringomyelia

2© 2003-2005 C&S Patient Education Foundation This presentation is intended for informational purposes only. Always consult a qualified professional for medical advice.

Arnold -WHO?

Chiari malformation (ACM, CM) is a condition where part of the brain, the cerebellar tonsils, descend out of the skull and crowd the spinal cord

– Hans Chiari first identified the disorder in the 1890’s– Type I is shown above, Type II is more serious and is associated with Spina Bifida,

Type III and IV are very rare Cause unknown, believed to be primarily a congential condition Affects people of all ages, however usually diagnosed in children and adults

in late 20’s – early 30’s Classic definition involves the length of the herniation (>3-5mm)

– Research has shown little correlation between length of herniation and symptoms; focus now on flow of cerebrospinal fluid (CSF); malformation blocks normal flow

Leads to a secondary condition, syringomyelia, in some people

Joe/Jane Chiari

Chiari malformationaka tonsillar ectopiaaka herniation

Cerebellum

Spinal cord

Joe/Jane Average

Foramen Magnum


Leads To What? Syringomyelia (SM) is a

neurological condition where a fluid filled cyst, or syrinx, forms in the spinal cord

The syrinx expands the spinal cord, sometimes to more than twice its normal diameter, and stretches the nerve tissue paper thin

Why a syrinx forms is unknown– Several theories, none proven

or without problems SM affects children and

adults of all races and ethnicities

Can cause permanent nerve damage and paralysis

Syrinx

Joe/Jane Syringomyelia


CM & SM: Two Sets Of Problems

Chiari Malformation (CM) is the leading cause of syringomyelia (SM), so some people must deal with two sets of problems

The direct link between CM and SM is poorly understood Spinal trauma and tumors are other leading causes of SM

Chiari Type 1 70%

Chiari Type 2 19%

Trauma 5%

Arachnoiditis 5%

Spinal Tumor 1%

Causes of SM

Source: Duke University Prevalence Study

CM

SM


Symptoms

• Symptoms vary widely among individuals (list not inclusive)• Pain and headache are the most common symptoms• Some people have CM and even SM without symptoms• It is not known why/how people become symptomatic

Severe headaches, brought on by coughing and straining

Weakness, stiffness,and pain in arms and legs

Trouble swallowing and sometimes speaking

Balance problems

Numbness in hands and feet,unable to feel hot and cold

Loss of bladderand bowel control

Extreme pain in neck and shoulders

Loss of fine motor control

Syringomyelia Chiari Malformation


Diagnosis Everyone is different

– Some people are diagnosed soon after onset of symptoms– Some people go years before an accurate diagnosis– Some asymptomatic (or slightly symptomatic) people are

diagnosed incidental to another medical event/condition In the end most people see a Neurosurgeon

– Neurological exam• Check for loss of sensation in hands, balance problems, reflexes,

walking, strength, etc.– MRI (Magnetic Resonance Imaging)

• Lay perfectly still inside a giant magnet that makes strange noises, but also makes great pictures of your brain and spinal cord

– CINE MRI• MRI shows static anatomy, CINE MRI shows the flow of CSF

(cerebrospinal fluid) and whether it is obstructed Surgery is the only real treatment

– Based on MRI and symptoms, the patient and Neurosurgeon will decide whether surgery is warranted

– Sometimes the Neurosurgeon will recommend monitoring the situation if the symptoms aren’t that bad and there is no syrinx


Surgery Is A Treatment, Not A Cure

Many neurosurgeons consider decompression surgery to be “technically” straightforward

There are many variations on the surgical technique; none are conclusively proven better than others

Unfortunately, surgery doesn’t always work; repeat surgeries are sometimes necessary

Surgery is fairly traumatic and can not reverse nerve damage that has already occurred

Complications include infection and spinal fluid leakage (from dura patch)

1. Goal of decompression surgery is to make more room; this in turn reduces crowding and may cause an accompanying syrinx to shrink

2. A piece of skull is removed3. Sometimes the dura, or covering of the brain, is

opened and a patch sewn in to make it bigger4. Sometimes, the top vertebrae are shaved down 5. Sometimes, part of the cerebellum is removed, or

cauterized6. An alternative surgery is to insert a shunt to

redirect Cerebrospinal Fluid


Surgery: What To ExpectEvery case is unique, but a typical experience might be:

Surgery: ~ 4 hrs. ICU: One night Hospital: 3-4 days

Rest at home: 3 weeks to 3 monthsNormal Activity: Variable


Surgical Outcomes For Chiari Only – Data is not conclusive, but…

– Up to 80% may have significant improvement– Repeat surgeries are sometimes necessary– Factors that influence outcome may include complex anatomy,

duration of symptoms, co-existing conditions– Lasting problems may include exertional headaches, balance

problems, trouble bending over– Long-term recurrence of symptoms is a problem

For Chiari with syringomyelia – Even less conclusive data, but…– Fewer people recover fully– Lingering symptoms are more common – Chronic pain is a common lasting effect– Other lasting problems may include bad shoulders (from nerve

damage), trouble walking and intolerance to heat– Syrinx can take up to a year to shrink


Things To Keep In Mind Surgeons tend to use a radiological definition of success

– Syrinx has shrunk, restored CSF flow– Discuss specific outcomes with doctor – what is the chance of

being symptom free? Will my main symptoms go away? Not clear why surgery fails (up to 20% of the time) Each person is unique

– Anatomy, symptoms, other conditions Over time symptoms will come and go, there is always a

chance of recurrence– Traumas, such as car accidents, can flare up symptoms

Most people will modify their lifestyle to fit their situation– Some go on disability– Some seek pain treatments– Some modify their activities


How Many People Are Affected?

0

50,000

100,000

150,000

200,000

250,000

300,000

350,000

400,000

450,000

1 2 3 4

Prevalence Estimates in US

(20,000)

(418,000)

(209,000)

(141,000)

There in no accurate count of the number of people with either condition, but consider the following:

1. NIH estimate of number of people with syringomyelia in US, no details or backup provided

2. Lower estimate of Duke University (Dr. Speer, 2003) prevalence study of people with syringomyelia in US

3. Upper estimate of Duke U. prevalence study of people with syringomyelia in US

4. Number of people with Chiari, syringomyelia, or both; assuming 50% of ACM patients develop syringomyelia; this extrapolates to >9,000,000 world-wide


State of Research

These are complicated conditions and there are many unanswered questions about the causes of, and best

treatments for them.

Known

????

Suspected>200,000 afflicted by SM/CM

Some genetic component

Surgery improves CSF flowAbnormal CSF dynamics

Pain

Causes paralysis

Why does a syrinx form? Will my children get it?

Why do surgeries fail?Why do syrinxes recur?

What makes people symptomatic?

What is the CM/SM link? How can pain be treated?


However, There Are Many Areas Of Active Research Surgical technique

– The best surgical approach is a highly debated topic with many publications

– How much bone to remove? Should the dura be opened? Should some of the cerebellar tonsils be removed? When should a shunt be put in?

Radiological– The advent of MRI, and now CINE MRI, revolutionized the diagnosis of

ACM and SM– Radiologists are developing new protocols to obtain even more

information CSF characterization

– Bioengineers and others are working to mathematically quantify the spinal system in order to gain a fundamental understanding of the conditions

Genetic– There are enough family clusters of Chiari to indicate a possible genetic

factor in the disease. Geneticists are working to find a “Chiari” gene Syrinx formation

– There are several active theories of how syrinxes form Pain Management

– Many types of doctors are researching novel treatments for pain


US Government Has Not Stepped Up To The Plate

ALS(20,000)

Huntington’s(30,000)

MS(400,000)

Parkinson’s(500,000)

SM(209,000)

Chiari(~400,000)

Disease (US Prevalence)

0

500

1000

1500

2000

2500

3000

3500

4000

11 10

Number of US Federally Funded Research Grants (1998 – 2002)

975

1,702

3,585

758

Since 1998, there have been only 11 Federally funded studies on syringomyelia, involving only 3 researchers

Source: NIH CRISP database


Economic ImpactEstimated Annual Costs of SM/CM in US:

Doctors Visits $ 32,000,000

MRI Scans $ 200,000,000

Surgeries $ 750,000,000

Rehab/Home Care $ 240,000,000

Disability Payments $ 1,475,000,000

Total $ 2,697,000,000

Lack of knowledge about SM/CM translates to ~2.7 Billion in annual costs; this does not include lost wages, productivity, etc.

Source: Internal analysis


C&S Patient Education Foundation

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Disclaimer: This presentation is intended for informational purposes only and may or may not apply to you. The editor and publisher are not doctors and are not

engaged in providing medical advice. Always consult a qualified professional for medical care. This publication does not endorse any doctors, procedures, or

products. © 2003-2005 C&S Patient Education Foundation

Thank You

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