Learning

Glossary

Sat, 27 Sep 2008 21:56:01 -0600

Apnea - to stop breathing intermittently, may be due to compression on the brainstem

Arachnoid - one of the three membranes covering the brain, appears to resemble a spider web. There are 3 layers of covering of the brain and spinal cord- the pia, arachnoid and dura

Arachnoiditis - inflammation of the arachnoid

Aspirate - inhaling fluids into the lungs. (Inability to swallow properly can allow fluid into the lungs.)

Ataxia - inability to coordinate movement. Stumbling when walking, or inability to maintain a fluid gait when asked to walk a straight line.

Atrophic - changes in the muscle that create an appearance of being wasted away.

Atrophy - wasting away of the muscle, often due to lack of use or inability to exercise a muscle.

Basilar Invagination - the base of the skull and the first cervical vertebra dent inward, causing pressure on the brainstem or upper cervical spinal cord.

Catheter - small, soft plastic tube used to drain fluid. A Foley catheter is often inserted into the bladder for surgery. A shunt catheter can be inserted into a syrinx or the ventricles of the brain.

Cerebellum - the lower part of the brain that lies in the posterior fossa and is responsible for coordination and fluidity of movement.

Cerebellar tonsils - the lower part of the cerebellum; in the Chiari I malformation, the cerebellar tonsils hang down into the upper spinal canal

Cerebrospinal fluid - clear, water-like fluid that flows around the brain and spinal cord, and acts to cushion the structures. The total CSF volume in an adult is about 150ml (or about 1 pint).

Cervical - pertaining to the neck

CINE MRI - or CINE MRI flow-study is a special test that looks at the flow of cerebrospinal fluid from the posterior fossa and into the spinal canal.

Clonus - abnormal, uncontrolled jerking of the foot when the ankle is jerked upward.

Cranial nerves - a set of 12 nerves in the brain that control motor and sensory functions.

Cranio-cervical junction - the area where the bottom of the skull connects to the top of the spine

Diplopia - symptom of seeing double, or double vision.

Dura Mater - the tough outer covering that surrounds the brain and forms the spinal canal

Dysequilibrium - inability to maintain proper balance. Often noted as stumbling or falling to one side.

Dysesthesia - an unpleasant sensation. A symptom of pins and needles or tingling when skin is touched.

Dysphagia - inability to swallow properly. Often leads to aspiration.

Ectopia - abnormal herniation, displacement or hang down of tissue

Electrocautery - a small instrument that uses heat and is used in many surgeries to stop bleeding.

Filum terminale - the band that extends from the end of the spinal cord to the tailbone

Hemisphere - one half of the upper portion of the brain.

Herniation - abnormal protrusion (or overhang) of tissue. Some MRI reports will read herniation of the tonsils- this means the tonsils are hanging down too far.

Hoffman's sign - (or reflex) an abnormal reflex that is tested by placing the fingers in a neutral position, and (painlessly) flicking the nail of the middle finger. If positive, the other fingers will abnormally flex in response.

Hydrocephalus - abnormal, over filling of the spinal fluid pockets (ventricles) in the brain. Can cause symptoms such as headache, balance problems, incontinence and memory problems.

Hydrosyringomyelia - a fluid cavity in the spinal cord. May also be referred to as syrinx, or syringomyelia.

Incidental Finding - a finding that is unexpected, or unrelated to symptoms. May refer to a person who has a finding on MRI, but has no symptoms.

Laminectomy - removal of the roof bone (or posterior arch) of a vertebra.

Limb ataxia - inability to maintain fluid movement of the arm or leg. Inability to touch finger to nose properly.

Lumbar - referring to the lower most part of the spine, the low back.

Meninges - the 3 layers of covering that surround the brain and spinal cord. Consists of the pia mater, the arachnoid, and the dura mater.

Meningitis - inflammation of the meninges. May be due to bacterial or viral infection.

Myelogram - special radiology study that involves injecting contrast dye into the spinal canal and taking x-rays of where the dye travels. The test is often followed by a CAT scan of the area. The test is often done to determine if there is blockage of flow or a nerve root that is being compressed. This test is less commonly used than MRI.

Myelomeningocele - herniation or protrusion of the spinal cord through a defect in development of the neural tube- seen in infants. Often associated with Chiari type II.

Nystagmus - abnormal bobbing of the eyes. Can be seen when looking to the side or up and down.

Paraparesis - weakness in both legs

Parasthesia - abnormal sensation in the extremity.

Pericranium - a muscle layer between the skull and scalp.

Periosteum - a muscle layer between the skull and scalp. (Called pericranium when on the skull).

Posterior Fossa - a general term for the compartment in the back part of the skull. This is where the brainstem, cerebellum and cerebellar tonsils lie.

Posterior fossa decompression - a general term for surgery to enlarge the compartment at the back of the skull

Pseudomeningocele - a collection of spinal fluid that develops outside the dura; while small collections are not of concern, larger collections can crowd and compress tissues

Scoliosis - curvature of the spine

Shunt - small, soft plastic tube that is used to drain fluid.

Sleep apnea - to stop breathing intermittently when sleeping. Symptom can be described as waking up short of breath or gasping for air.

Syncope - spells of passing out, or fainting (loss of consciousness). Different from a seizure.

Syringomyelia - the condition of having an abnormal fluid cavity in the spinal cord.

Syrinx - abnormal fluid cavity in the spinal cord.

Tethered cord - when the spinal cord becomes stuck to the surrounding structures due to scarring or a congenital problems or is pulled down by the band at the end of the cord

Thoracic - refers to the area of the chest; from the shoulder blades, through the ribs, to about the belt-line

Tinnitus - ringing in the ears.

Tonsillar ectopia - a small amount of hang down of the tonsil tissue of the cerebellum.

Tonsillar herniation - more descent or hang down of the cerebellar tonsils into the upper spinal canal. The classic description is 3 - 5 mm or more of hang down below the opening at the base of the skull (the foramen magnum).
Tonsils - a small, often rounded mass of tissue. There are several tonsils in the body, including in the throat, in the intestine, or in the brain. In reference to Chiari, indicates the tissue at the bottom of the cerebellum that can protrude downward, causing a plug effect.

Traction - to pull something; in neurosurgery usually refers to pulling on the skull with tongs or a halter
Ventricle - fluid pocket in the brain (4 in all), that holds spinal fluid. Abnormal enlargement of the ventricles is called hydrocephalus.

© 2008 HCA - The Medical Center of Aurora, Aurora Colorado

The Chiari I Malformation

Fri, 9 May 2008 21:19:47 -0600

The Chiari I malformation (CM-I) is an uncommon congenital (present at birth) deformity of the lower compartment of the cranium called the posterior fossa that results in crowding of the brainstem and cerebellum. The lower part of the cerebellum, known as the cerebellar tonsils, protrudes down the opening at the base of the skull (known as the foramen magnum) and into the spinal canal. This results in tissue compression and decreased spinal fluid flow. Although the Chiari (pronounced kee-ar'-ee) I malformation can cause problems in childhood, many people do not develop symptoms until adulthood.

The Chiari I malformation is more common in women than in men though the exact cause is still unknown. People with CM-I may develop symptoms in their 20’s or 30’s, although they can develop sooner or later than this. Multiple symptoms are possible. Among the most common are headache, dizziness, blurred vision, neck pain, weakness, numbness, or tingling the arms, nausea, and problems swallowing.

“When symptoms such as headaches, dizziness, and fatigue persist, they often rob a person of their quality of life. Though not everyone experiencing these symptoms is suffering from Chiari I Malformation, for those that are, we’re here to provide answers and options.” – John Oró, MD
 Symptoms from the CM-I often develop in a slow and insidious manner. They are often vague and progress over a number of years. Many persons have symptoms for years before they are properly diagnosed. About 1 out of 4 patients develop symptoms after a mild or moderate trauma. Examples include falling off a trampoline, falling off a porch, or a blow to the back of the head.

Symptoms
Headache
The most common symptom of the Chiari I malformation is headache. It usually begins at the back of the head and radiates behind one or both eyes, to the top of the head, or to the temples. The headache is often described as a pressure sensation but can be heavy, sharp, or stabbing. For many, the pain varies between these and other sensations.

The headache is often made worse by straining activities (known as valsalva maneuvers) such as coughing, sneezing, or even laughing or singing. Some patients report feeling as though “my head will explode” or “my head will pop off”. Bending forward, or looking up can worsen the headache. When the headache is intense, some patients note dizziness, blurring of vision, nausea, or a feeling of a “mental fog”.

Visual Symptoms
A variety visual symptoms can occur with CM-I including double vision, decreased peripheral vision, seeing flickers of light (“fireflies”), visual loss, blind spots, photophobia (sensitivity to bright lights), spasm of the eyelids (blepharospasm), and jerking of the eyes (nystagmus).

Dizziness & Auditory Symptoms
Dizziness is frequently seen in persons with CM-I. It is usually intermittent, often worse with the headache, and more noticeable when changing position. A sensation of spinning (vertigo), ringing in the ears (tinnitus), poor balance (disequilibrium), and decrease or loss of hearing may also occur.

Difficulty Swallowing & Hoarseness
People with CM-I may develop difficulty swallowing (dysphagia), either with liquids or solid foods, or a feeling of “catching” in the throat when attempting to swallow. The progression of this symptom may be rapid and result in aspiration (inhaling fluid or food products into the lungs). Change in voice character and timber is a common complaint. Hoarseness is often noted first by a family member or friend. Some persons report inability to modulate voice when singing or speaking loudly.

Pain
Persons may complain of neck or arm pain. Often the pain is worse with exertion, fatigue, or lifting. The pain may be dull and aching, or shooting and stabbing. It is usually present or worse on one side. Along with pain, some patients experience weakness of the hand or arm, or difficulty with fine movements of the fingers. If syringomyelia is present, the pain may be more widespread.

Numbness
Tingling, numbness or pins and needles sensation often occur in the same areas as the pain. Numbness can progress over months to years and involve the lower extremities and trunk. These symptoms are usually more noticeable if a person has a syrinx.

Problems Walking
Problems walking may be described as unsteadiness or listing to one side. Persons may report falling, or bumping into walls or doors. Infrequently, persons may present with spasticity (stiffness) of the legs. These symptoms occur more commonly with syringomyelia.

Respiratory, Heart, and Abdominal Symptoms
Effects of the CM-I on the respiratory and heart centers can cause shortness of breath, chest pain, episodes of rapid heart rate (tachycardia), black out spells, and hypertension. Abdominal symptoms may include nausea, abdominal pain, or vomiting.

General & Cognitive Symptoms
These include poor sleep (insomnia), fatigue and depression. Some note problems with memory, thinking and speech. People report the feeling of a “brain fog” with difficulty in thinking and concentration. Difficulty in finding the right word is may occur.

Other Possible Conditions
It is important to note that other conditions can result in symptoms similar to that of the Chiari I malformation. Other conditions that should be considered include:

Migraine
Cluster headache
Rebound headache
Pseudotumor cerebri
Occipital neuralgia
Post-concussion syndrome
Hydrocephalus
Multiple sclerosis
Chronic fatigue syndrome
Fibromyalgia
Sleep apnea
Spinal fluid leak
Cervical disc degeneration
Psychiatric disease

Evaluation & Management
To learn about the evaluation and management of Chiari I malformation and syringomyelia, please visit the Treatment page.

Frequently Asked Questions: The Chiari I Malformation

Fri, 2 May 2008 21:22:28 -0600

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.
www.ASAP.org
http://health.groups.yahoo.com/group/ChiariConnectionInternational/
http://www.chiariconnectioninternational.com/
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 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:
www.asap.com
www.chiariconnectioninternational.com
www.chiaritimes.com
www.conquerchiari.org
www.csfinfo.org
www.pressenter.com

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?
Read more here....

45.   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.

46.   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.

49.   Is slurred speech, voice hoarseness and finding the right word common in CM1?
These symptoms are common in patients with CM1. Slurred speech is related to problems with the function of the cerebellum. Hoarseness of the voice is due to compression of the nerves that go to the voice boc. Change in the pitch and quality of the voice are also affected in some people.

49.   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.

50.   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.

51.   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.

52.   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.

53.   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.

Frequently Asked Questions: Syringomyelia

Thu, 24 Apr 2008 21:24:17 -0600

1. What is syringomyelia?
Syringomyelia is the condition of abnormal fluid collecting inside the spinal cord. The cavity itself is called a syrinx.

2. 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-shaped are at the base of the skull that connects to the spinal canal. This blockage changes the fluid pressure around the spinal cord and causes fluid to accumulate in the spinal cord tissue. Spinal fluid pulsates with each heartbeat. Coughing and sneezing cause even greater changes in the pressure inside the spinal canal.

3. 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.

4. Do all people with Chiari I malformation have a syrinx or syringomyelia?
No. Many patients with CM1 do not develop syringomyelia.

5. 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). For many, the improved spinal fluid flow will 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 know, the treatment decisions are more difficult. Some people may respond to sectioning the band at the and of the spinal cord, the filum terminale. If the syrinx is small and the symptoms are under control, observation and follow-up MRI scans are suggested.

Frequently Asked Questions: The Tethered Cord

Wed, 23 Apr 2008 21:25:53 -0600

1.   What is a tethered cord?
The spinal cord is tethered when it is not able to slide normally inside the spinal canal. Scarring from a previous spinal injury can cause the cord to be stuck to the spinal canal. Or, something at the bottom of the spinal canal can hold it down. This may be due a tumor or congenital malformation or the band at the end of the spinal cord (the filum terminale) can be too short or too tight.

2.   Does tethered cord ever occur in CM1 patients?
Yes. A few reports reviewing previous cases suggest that CM1 patients may, in a few cases, also have a tethered spinal cord from a tight filum terminale. This is a new and controversial area and no prospective studies evaluating this have yet to be published. (Prospective studies are studies that collect information at the beginning and throughout treatment and follow-up, instead of only reviewing previous files.)

3.   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, and severe growing pains during childhood and adolescence. It can also result in scoliosis. The relation of tethered cord to elongation of the brainstem in some persons with Chiari malformation is currently being investigated.

4.   What is the treatment for tethered cord?
If the tethered cord is due to scarring within the spinal canal, in some cases, the scarring can be released at surgery. For those with a short or tight filum terminale, an operation can be performed to cut the band. The band itself is made of fibrous tissue. If the tethering is due to a tumor or a congenital abnormality, the surgery is usually more complex.

New Chiari Book Published

Thu, 10 Apr 2008 12:49:30 -0600

In February 2008, Dr. John Oro’ and Diane Mueller, ND, NP published The Chiari Book: A Guide for Patients, Families, and Health Care Providers. The book was written to increase awareness of the Chiari I malformation and syringomyelia and offer hope to those affected.

The Chiari Book is based on the experience gained in evaluating over 1,200 persons for the Chiari I malformation and syringomyelia. In 1998, Dr. Oro’ and Dr. Mueller, developed a specialized clinic at the University of Missouri Health Sciences Center in Columbia for the care of patients with these disorders. In 2005, moved to The Medical Center of Aurora in Aurora, Colorado.

Illustrated with drawings and photographs, the book is written in a manner that we hope is understandable. Its 96 pages are organized into 10 chapters and 3 appendices:

1. Introduction
2. A brief review of Neuroanatomy
3. The Chiari Malformations
4. Syringomyelia
5. Symptoms of Chiari malformation
6. Other illnesses that can mimic Chiari malformation
7. The diagnosis of Chiari malformation and syringomyelia
8. Treatment options for Chiari malformation and syringomyelia
9. Special considerations in Chiari (children and adolescents, pregnancy, elderly)
10. Summary
APPENDICES:
Chiari and Syringomyelia Articles
Glossary
Internet Resources

The book is available at Amazon.com at this link: The Chiari Book

Your comments are welcome. Let us know how we can improve a future edition.

E-mail: info@chiaricare.com