1.    What is the Chiari 1 Malformation?

The Chiari malformations are complex, neurosketal 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 your 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. To you it feels like a regular MRI scan. The physician uses the results from the the clinical history, neurological examination, MRI scan, CINE study, 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 if the cerebellar tonsils are below their normal position and the amount of brain tissue crowding at the base of the skull.  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 neurologic 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.

www.ASAP.org

www.conquerchiari.org

www.pressenter.com

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 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 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 breathe for the patient during surgery a breathing tube is inserted while the patient is asleep. The tube is taken out after surgery during 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 solids 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 the Sickness Impact Profile. They found that 84% of the people reported significant improvement in their symptoms. 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 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.   What is a Tethered cord?

The spinal cord is held much lower in the spinal canal than normal in persons with tethered cord. This may be due to the band at the end of the spinal cord (the filum terminale) being tighter than normal. Or it may be due to a tumor or congenital malformation. A new area of study is whether the spinal cord in a normal position can still be tethered by a tight filum.

20.   Does tethered cord ever occur in CM1 patients?

Yes. There are a few reports reviewing previous cases that suggest that CM1 patients may, in a few cases, also have a tethered spinal cord. This is a new and controversial area and no prospective studies evaluating this have been published at this time. (Prospective studies are studies that collect information at the beginning and throughout treatment and follow-up, instead of just reviewing previous files.)

21.   What symptoms can tethered cord cause?

Tethered cord can cause urinary urgency, urinary and/or stool incontinence, difficulty starting the urinary stream, constipation, diarrhea, difficulty with sexual relations, difficulty standing over 60 minutes, low back pain, leg pain, numbness in the soles of the feet, difficulty riding or driving a car, severe growing pains during childhood and adolescence. It can also result in scoliosis.

22.   What is the treatment for tethered cord?

If the tethered cord is due to a tight filum in the lower spinal canal, an operation can be performed to cut the band. If it is due to a tumor or a congenital abnormality, the surgery may be more complex.

23.   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 found to be in 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.

24.   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.

25.   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. Episodes of paroxysmal rage caused by the Chiari 1 malformation have been reported in a few children.

26.   How are children diagnosed with CM1?

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

27.   Is pregnancy possible with CM1?

Yes. Many women with CM1 have normal uncomplicated pregnancies.

28.   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.

29.   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.

30.   Are there trusted CM1 web sites?

There are several trusted websites that we have suggested to our patients. The first is our own.

A sample of other websites include:

www.asap.com

www.pressenter.com  part of the World ACM Association

www.chiariconnectioninternational.com

31.   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.

32.   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.

33.   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.

34.   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.

35.   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.

36.   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.

37.   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.

38.    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.

39.   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.

40.   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.

41.   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.

42.   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.

43.   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..

44.   Can CM1 cause seizures?

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

45.   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.

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

Read more here....  

47.   My ears ring and I am having trouble hearing. Why?

The crowding of the cerebellar tonsils can put pressure on the cranial nerves. This may be the cause of decreased hearing.

48.   What is causing me to snore at night? What can I do about it?

The breathing centers are often disrupted by CM1 and syringomyelia.  This is called Sleep apnea.  People who have sleep Apnea stop breathing for 10 to 30 seconds at a time while they are sleeping. These interruptions can occur up to 400 times a night.  Sleep evaluations often help to determine if a machine to help with proper breathing is necessary at night. The machine is called a continuous positive airway pressure device or CPAP.

49.   I am tired all of the time, even after eight hours of sleep. I can't seem to wake up. What could be causing this?

There are many causes of fatigue. Sleep apnea as described in the answer to question 45 could be the cause. Pain and getting up several times to urinate can also cause daytime sleepiness.

50.   What is syringomyelia?

Syringomyelia and is the condition of abnormal fluid collecting inside the spinal cord. The cavity itself is called a Syrinx.

51.   What causes syringomyelia in people with CM1?

Although the exact mechanism of development of syringomyelia is unknown, there are many theories about the formation of a syrinx cavity. One theory suggests that the herniated tonsils such as in the Chiari I Malformation, results in blockage of the spinal fluid flow at the funnel at the base of the skull that connects to the spinal canal. This changes the fluid pressure around the spinal cord and causes fluid to accumulate in the spinal cord tissue. With each heartbeat, the spinal fluid pulsates. Coughing and sneezing cause even greater changes in the pressure inside the spinal canal.

52.  What is the best way to determine if I have syringomyelia?

The best way to diagnose syringomyelia is to have an MRI of the cervical and Thoracic spine.

53.   Do all people with Chiari I malformation have a syrinx or syringomyelia?

No. Many patients with CM1 do not develop syringomyelia.

54.   What is the treatment for syringomyelia?

The best treatment for syringomyelia is to remove the block to normal spinal fluid flow if there is one. In patients with syringomyelia due to the blockage caused by a Chiari I malformation, the goal is to create more room at the foramen magnum (through a posterior fossa decompression as described above) and improve spinal fluid flow. For many, this will often result in the syrinx slowly decreasing in size, although it may not disappear completely. The important point is to keep the syrinx from growing and allow it to relax.

When syringomyelia is due to spinal trauma, release of the scar bands around the spinal cord can result in a decrease in the size of the syrinx. When it is due to a spinal cord tumor, the best treatment is usually removal of the tumor if possible. When the cause of the syrinx is not known, the treatment decisions are more difficult. Some people may respond to sectioning of the filum. If the syrinx is small and the symptoms are under control, observation and follow-up MRI scans are suggested.

55.   Is slurred speech, voice hoarseness and finding the right word common in CM1?

These symptoms are common in patients with CM1. They are likely related to problems with the function of the cerebellum. Change in the pitch and quality of the voice are also affected in some people.

56.   How can I find out if all of the medications I take are safe to take together?

When a new prescription is filled the pharmacist will usually tell patients if there is a medication that should not be taken with another. If you are taking multiple medications from different physicians it is a good idea to get all of your prescriptions filled at the same pharmacy for this reason.

57.   I take a variety of herbal preparations. Are there any that I should avoid with CM1?

Herbal preparations do not have the scientific studies backing their effectiveness or safety that prescription drugs have. The pharmaceutical industry spends millions of dollars developing, testing, running clinical trials, and getting FDA approval before a drug is released. The quality and strength of herbal preparations are not regulated in this country. The interactions of many herbal preparations with prescription drugs are not known.  Prescription medications interactions with other prescriptions drugs and common foods or drinks are well understood. For example a common drug to lower cholesterol, Lipitor®, when taken with grapefruit juice can cause toxicity in some people.

58.   Are there any specific herbals that I should avoid before surgery?

The list below is not inclusive. The herbals listed below may interfere with other drugs or increase the risk of bleeding . They should be eliminated at least two weeks before surgery.

Chamomille, Clove, Dong Quai, Echinacea, Evening Primrose Oil, Fenugreek, Fever Few, Garlic, Ginger, Gingko Biloba, Ginseng, Panax, Goldenseal, Horse Chestnut, Kava Kava, Licorice, Meadowsweet, Poplar, Prickly Ash-Northern and Southern, Quassia, Red Clover, White Willow/Willow Bark.

59.   Is it OK for my chiropractor to crack my neck?

High-velocity chiropractic is not advised in persons with the Chiari malformations. Injuries have occurred.

60.   Why am I depressed and anxious? Should I be treated?

Depression and anxiety can be caused by chronic illness, pain and uncertainty. All or some may be present in CM1 and syringomyelia. Many patients are placed on medications for depression and anxiety.

61.   What other diseases cause symptoms like CM1?

There are many diseases that can cause some of the same symptoms as CM1. This list does not include all disease states. A partial list of other causes are: hypo/hyperthyroid, vitamin deficiency, anemia, medication toxicity, environmental toxins, alcoholism, mediation toxicity or interactions, depression, anxiety or panic disorders, stroke, brain tumor (especially of the cerebellum), Arachnoid cysts or the cerebellum, encephalopathy, cerebellar Atrophy, multiple sclerosis, cerebellar abscess, brain trauma/injury, diabetic neuropathy, polyneuropathy, post polio syndrome, disorders of the cervical or thoracic spine, syringomyelia, Myasthenia gravis, amyotropic lateral sclerosis, and neurosarcoidosis.