The Chiari Care Center

1. What is the Chiari 1 Malformation?

The Chiari malformations are complex, neuro-skeletal deformities presumed to be present at birth. The Posterior Fossa is located in the lower back part of the cranium. It contains the brainstem, Cerebellum, and cerebellar Tonsils. It is believed that in the Chiari I malformation the posterior fossa does not form correctly during fetal development and is too small to hold all of the contents that it should. Type I malformation has been defined as cerebellar tonsils that descend 3 mm-5 mm out of the skull due to crowding in the posterior fossa causing an obstruction of the Cerebrospinal Fluid (CSF) flow. However, some people may have symptoms with less descent. What is important is whether the flow of spinal fluid is being blocked and whether the brain tissue in the area is compressed.

2. Who gets Chiari 1 Malformation?
The Chiari 1 malformation can occur in adults, children and occasionally infants. It occurs three times more often in women than in men. There is no particular ethnic or geographic distribution.

3. Can Chiari occur in those not born with the deformity?

Yes. This is called secondary Chiari and can be caused by hydrocephalus, tumors which displace the brain structures, and trauma which causes the brain to swell. Some people that have a Shunt in the lower spinal canal may also develop secondary Chiari. Whether the condition of tethered spinal cord can "cause" Chiari is controversial.

4. How is CM1 diagnosed?

Chiari is diagnosed by clinical evaluation and a combination of studies. An MRI (Magnetic Resonance Imaging) is ordered. This is a non-invasive test using a large magnet which creates a picture of the inside of the brain and cervical spine. The MRI pictures show if the cerebellar tonsils are below their normal position. Physicians do not treat just based on displacement of the cerebellar tonsils. An MRI CINE study may be ordered to asses the flow of spinal fluid through the base of the skull. This feels like a regular MRI scan. The physician uses the results from the the clinical history, neurological examination, MRI scan and other studies, along with their clinical experience and medical judgment to diagnose Chiari.

5. What does an MRI scan show?

An MRI scan creates an anatomical picture of the brain and spinal cord. In Chiari it can show the degree of crowding at the large opening at the bottom of the skull called the foramen magnum. Brain tissue compression and distortion can be analyzed and the shape and changes in the bony structures can be evaluated. An MRI of the spine can show the presence of Syringomyelia.

6. What is a CINE study and what is it used for?

A CINE is a special MRI that is used to assess the flow of spinal fluid through the large opening at the base of the skull called the foramen magnum. It is used in some cases to determine if spinal fluid flow is being blocked.

7. What is a neurological exam?

This is one of the most important pieces of the diagnostic process. Although some persons with radiographic evidence of Chiari I malformation on MRI will have a completely normal examination, others will have important abnormal findings (or deficits). The neurological exam provides additional information on how the complex network of nerves are functioning or not functioning. The exam includes a series of simple questions and neurological tests that provide important information and aid in the diagnostic process. During the neurological examination, thinking, memory, cranial nerve function, sensory and motor function, reflexes, and coordination are tested.

8. Does CM1 cause disability?

Chiari malformation can cause temporary and permanent disability and is dependent on the individual. Many troubling symptoms that prevented working and playing often disappear after surgery. Other symptoms may not be easily resolved and may take time and effort on the part of the patient and health care professionals to solve so that the patient may get back to a normal lifestyle.

9. I am diagnosed with CM1, what type of doctor should I see?

You should see a neurosurgeon who is experienced in the evaluation and treatment of persons with the Chiari 1 malformation. A neurosurgeon is: a specialized physician that evaluates and treats people with diseases and problems of the nervous system that may require surgery.

10. How can I find out if the doctor is experienced?

You can simply ask the neurosurgeon about their experience in the treatment of persons with Chiari malformations and/or syringomyelia. The most important thing is to establish a close working relationship with your neurosurgeon. Have them review your scans with you and explain the disorder. You can also go to one of the informational Chiari sites on the Internet. Most have a list of neurosurgeons skilled in treating Chiari across the country. These websites are a good source of information for patient suggested neurosurgeons.

11. I have CM1, is there anything that I should not do?

Pay attention to how you respond to various activities and use this as a guide. For many people with Chiari, lifting weights, straining, bending forward, or looking up may worsen their symptoms. Contact sports and activities such as ridding roller-coaster and jumping on trampolines are often restricted before treatment. To learn more about Chiari and contact sports, visit the Chiari Times Blog at www.chiaritimes.com

12. How is CM1 treated?
Treatment depends on the symptoms. For people who have mild symptoms, medication is often used to help control the symptoms. For people who are unable to participate in normal day-to-day activities or are suffering from uncontrolled pain, surgery may be required.

13. How long does the surgery take?

Each individual is different. In general surgery usually takes between 3 to 4 hours.

14. What can I expect in the hospital?

Hospital admission is usually the morning of the surgery. The preoperative area is the first stop. An intravenous line (IV) will be started to provide fluids until the ability to take fluids orally is possible. Before entering the operating room a relaxation medication is given. Several sticky patches are placed on the chest in order to monitor the heart during surgery.

After entering the operating room medications are given for a deep sleep (general anesthesia). Once in a deep sleep, electrodes are attached to the skin to monitor nerve function during surgery. This is called evoked potential monitoring. To protect the airway and support breathing during surgery a breathing tube is inserted while the patient is asleep. The tube is taken out after surgery while the patient is still sedated. A catheter is placed in the bladder while the patient is asleep to drain urine from the bladder during and removed the day after the surgery so getting up to use the restroom is not necessary immediately after surgery.

Waking up takes place in the recovery room. In the recovery room several simple tasks are required of the patient. The nurse will instruct the patient to squeeze her/his hands, wiggle their toes, and state their name. Once awake, the patient will be able to deliver their own pain medication through a pain pump. The pain pump is set to keep the patient from receiving too much medication. This method of pain control is usually stopped the first morning after surgery and oral pain medication and medication to relax muscles is started.

In most cases sitting in a chair and walking is begun the day after surgery, although a few people are up a little the first evening. This activity helps to prevent blood clots and lung congestion. Walking also gets the bowel working more quickly. Ice chips are provided for a time after surgery, then liquids, and finally solid foods are added. Most people are in the hospital 3 days.

15. What is the outcome following surgery?

Diane Mueller and Dr. Oro' have published the only Chiari outcome study using a quality of life measure, the Sickness Impact Profile. They found that 84% of the people reported significant improvement in their symptoms when studied one year after surgery. Some people have remarkable recoveries, others have improvement with lingering problems. Recovery takes time and you should set realistic expectations for yourself.

16. I have CM1 and hydrocephalus. What is hydrocephalus?

Hydrocephalus is an accumulation of cerebral spinal fluid within the ventricles of the brain. It results from blockage of normal spinal fluid circulation. Hydrocephalus may result from developmental abnormalities, infection, brain injury or brain tumors.

17. Will CM1 surgery make the hydrocephalus better?

Often the hydrocephalus will resolve once the blockage of spinal fluid flow has been relieved by Chiari surgery. Occasionally, a shunt may have to be placed in order to remove the excess fluid from the brain ventricles.

18. What are the dangers of hydrocephalus?

The dilation of ventricles with spinal fluid places pressure on delicate brain tissues. Symptoms of hydrocephalus may include headache followed by vomiting, nausea, blurred vision, diplopia (double vision), problems with balance and coordination, difficulty walking, urinary incontinence, drowsiness, irritability, and other changes in personality or memory changes.

19. Can CM1 reoccur?

Some people have recurrent symptoms after a period of good response to surgical treatment. There are various causes for this and a careful evaluation is needed. Sometimes the cause is not the Chiari itself but other conditions such as cervical discs causing headache or occipital neuralgia (pain from the nerves at the back of the head).

20. If I have CM1 will my children have it?

For most patients CM1 does not run in families. In some cases, however, it can be passed from the mother or father to their children.

21. How does CM1 affect children?

Many of the signs and symptoms that occur in adults are also present in children. However, young children may not be able to accurately report their symptoms. Rarely, children may present with Cerebellar Fits or episodes of Paroxysmal Rage.

23. How are children diagnosed with CM1?

If you have CM1 and your child has neurological symptoms ask your pediatrician to order an MRI scan of the brain for a positive diagnosis.

24. Is pregnancy possible with CM1?

Yes. Many women with CM1 have normal uncomplicated pregnancies.

25. How can I help to protect my unborn child from Neural tube defects?

Ask your OB/GYN physician about taking the supplement folic acid during your pregnancy.

26. If pregnant do CM1 patients have to have a cesarean section?

No. Many women have normal vaginal deliveries. This decision should be made after a discussion with your OB/GYN physician.

27. Are there trusted CM1 web sites?

These are some of trusted websites that we have suggested to our patients:

28. Are there patients that I can talk to about CM1?

Most of the patient websites just listed have other patients that you can chat with online.

29. Are there any support groups that I can attend?

Chiari and syringomyelia are recognized more frequently than in the past. Because of this there are not many support groups available. You may want to find out about support groups near you by looking at websites. The easiest way to find out if there are groups in your area is to Google support groups for Chiari or syringomyelia.

30. How can I make my family and friends understand what I am going through?

Sometimes it is difficult for family and friends to understand your disease because physical symptoms are usually invisible to others. The best remedy for understanding is education. Consider taking a family member with you when you have an appointment with your neurosurgeon. Have a short list of important questions (about 3) that you would like to have answered during your visit.

The poem So I Say summarizes what many Chiari patients deal with. It may be helpful to your family and friends.

31. Should I tell my boss that I have CM1?

Many people with the diagnosis of Chiari or syringomyelia continue to have productive careers long after they are diagnosed. There are many state and federal laws that address work issues for people with disabilities or chronic illness. Check with your state government about available hotlines and information about discrimination in the work place. There are 2 federal laws that may be useful. They are: The Americans with Disabilities Act (ADA), and The Family and Medical Leave Act (FMLA).

After you know your rights, you must decide if you want your boss to know about your condition.

32. Why does crowding and poor CSF circulation cause symptoms?

The CSF surrounds the brain and spinal cord and provides protection for the delicate nerves contained within and circulates constantly. Most of the symptoms of Chiari I malformation can be attributed to obstruction of spinal fluid flow or compression of the lower brainstem and Cranial nerves.

34. What symptoms does it cause?

Some of the most common symptoms in Chiari 1 malformation include: headache, dizziness, poor balance, pain, pins and needles sensations, numbness, difficulty swallowing, weakness in the extremities, vision problems, ringing in the ears, shortness of breath, difficulty sleeping, memory problems, difficulty with speech, nausea, depression and anxiety, cardiac symptoms, and problems with the bowel and bladder. Symptoms vary from person to person.

35. How can I manage my headaches?

Headaches are often caused by coughing, straining, laughing, bending or lifting. Headaches can also be caused by overuse of mediations used to treat headache. Persons suffering from headaches should make sure to drink an adequate amount of water as dehydration can contribute to headache. Many people report some relief of headache while resting in a darkened room, occasional use of migraine medication, application of heat or cold to the area, and use of certain anti-seizure medications.

36. Is there anything I can do to help with dizziness?

Abrupt movements of the head and getting up quickly can cause dizziness. It is important to remember that there can be other causes the symptoms commonly associated with Chiari. A consult with an ear nose and throat specialist may help to rule out another reason for the dizziness such as problems with the inner ear. Sudden movements or laying flat may increase dizziness.

37. Why am I falling and having trouble taking walks?

Chiari causes crowding of the cerebellar tonsils. The cerebellar portion of the brain is responsible for the coordination of voluntary muscular movements. The cerebellum interacts with the structures of the brainstem and helps to execute a variety of movements such as balance and walking.

38. The constant burning pain in my arms is keeping me from thinking well at work and sleeping. What can I take for this pain?

As with other types of neuropathic (nerve) pain, narcotic pain pills do not tend to work well. This type of pain is treated in a variety of ways. The most common treatment is with certain antiseizure medications that can be used to control neuropathic pain.

39. I can not feel my feet. Is this permanent?

It is difficult to say if the numbness is permanent. Many symptoms such as numbness get better after surgery. Sometimes the nerves have been compressed for a long time and recovery is often not complete.

40. I am choking on liquids and sometimes solid food. Is this dangerous?

This is a dangerous problem. If choking is present fluid or food can be inhaled into the lungs causing an aspiration pneumonia. A speech pathologist can often help evaluate this problem.

41. What is causing my blurred vision and my jumpy vision?

Compression of the cranial nerves can cause these problems and increased pressure in the brain can also affect vision..

42. Can CM1 cause seizures?

There is limited knowledge on this topic. The literature suggests there is some correlation in children.

43. If CM1 is present at the time of birth, why do symptoms show up so much later?

The reason that symptoms in many people do not show up until later in life is unknown. It may be related to the repeated pulsations in the area and the fact that spinal fluid flow becomes a little more restricted as we age. Many people report symptoms after an injury such as a motor vehicle accident, whiplash, and falls.

44. Why is the Chiari malformation frequently unrecognized or ignored causing delayed diagnosis and treatment?