Q & A with Dr. Menezes

by Dr Arnold Menezes Professor of Neurosurgery University of Iowa Hospitals and Clinics and member of the ASAP Medical Advisory Board

I have a Chiari malformation with a 7mm tonsillar herniation. Does the herniation become greater over time?

The diagnosis of Chiari Malformation on magnetic resonance imaging is not only with a tonsillar herniation of 4-5 mm into the cervical canal but also with other associated findings. The literature shows that the tonsillar herniation may regress or otherwise. There are many other factors such as the timing of the MRI as well as the presence of hydrocephalus.

In patients with Syringomyelia are new symptoms always a sign that the syrinx has progressed?

The patient is always made aware of occurrence of new symptoms so as to alert the health care personnel to the possibility of either progression of the radiographic abnormality or some other simultaneously occurring phenomenon. Thus, it is very important to pay attention to the development of new symptoms in patients with Chiari Malformation and Syringomyelia.

Q & A with Dr. Benzel

by Dr.Edward Benzel. A neurosurgeon and Director of Spinal Disorders at the Cleveland Clinic in Ohio, and member of ASAP’s Medical Advisory Board.

I suffer from a lot of pain caused by a moderate-sized syrinx and Chiari. I am scheduled to have a decompression. What are the chances of the pain improving after the surgery?

Since many patients have pain that is unrelated to the syrinx and a chiari malformation, the chances of the pain improving following surgery are not 100%. There are many factors that play a role in this process. One should be very careful to not be excessively optimistic regarding the relief of all symptoms following chiari malformation or syringomyelia surgery. It, however, is reasonable to expect at least a 50-60% chance of pain reduction following the operation. This obviously must take into consideration the nature of the pain, which is difficult to understand without personal one on one communication between the physician and the patient.

What is a dura leak, how do I know if I have one and what should be done about it?

The leakage of spinal fluid through the covering over the brain and spinal cord called the dura mater (dura). This covering is generally very tough. However, following surgery, such as surgery for a chiari malformation, leakage of spinal fluid through the dura mater (dura) may occur. It may be obvious because it leaks into the soft tissues around the surgical site through the skin. This drainage is evident. It unfortunately may lead to infection and other complications that therefore should be surgically treated.

A dural leak could also occur but not extend up to the level of the skin. This type of leak may not be as evident. A bulge under the surface of the skin may be the only physical sign. People with such leaks may complain of headache, particularly while upright.

Stress and Chronic Illness

by Rick Rader, Morton J Kent Habilitation Center

Definition of a Life Crisis

A crisis may be defined as an acute stress situation that is a threat to our sense of self. Typical situations or events that may trigger a crisis reaction include:

  • A major disaster involving an entire community, such as a flood, tornado, earthquake or air crash
  • Sudden death of a family member, especially a child
  • The loss of one’s home, e.g., through fire, earthquake, storm
  • An acute traumatic injury or illness resulting in a chronic physical disability
  • Sudden loss of a job or a job-threatening situation, including business takeover, layoffs, malpractice lawsuits, work injury

A crisis can, and usually does, strike suddenly. We may be a casual bystander, not directly involved with the specific incident (such as witnessing a traumatic auto accident), but still suffer a crisis reaction. When we are in the middle of a crisis, we don’t generally understand what is happening or what might happen in the immediate future. Distorted perceptions are common. Other typical responses include depression, confusion, anxiety, foreboding, feelings of panic, fear of impending loss and shock. In helping a loved one or a peer (or even ourselves) through a crisis, it is important to recognize the following psychological and emotional reactions to a crisis:

  • A loss of sense of reality and ability to adequately differentiate between our emotional state and the realities of the external world.
  • Feelings of helplessness that serve to increase tension even further: “Why can’t I relieve this horrible feeling?”
  • Refusal to acknowledge emotions such as anxiety or physical symptoms inability to predict or anticipate future events.
  • No clear understanding of the cause of an event, inability to address the question, “What will happen to me next?”
  • Loss of psychological and emotional equilibrium, often followed by renewed efforts to cope.

Most of us will experience several crises during our lives, and it is important to be prepared. While reactions to crisis often involve anxiety and depression, the negative effects of these emotions can be relieved by understanding their causes.

Overcoming a Life Crisis

In most cases, the passage of time alone is sufficient to restore healthy psychological and emotional functioning after a crisis. However, it may also be necessary to consult a doctor or therapist to obtain objective insight and emotional support during such periods. This assistance is typically of a short-term (crisis intervention) nature. Often, all that is needed is to learn a new set of coping techniques for dealing with the stress presented by the extraordinary life event creating the crisis.

After a divorce or a breakup of a relationship, for example, we may feel completely alone in the world. Emotional support from others is needed to fill the void and reduce the isolation, loneliness, despair and need for validation. Family and friends are often the first line of support in such situations.

Events tend to occur so quickly during a life crisis that we don’t have time to develop new behaviors to cope with the related stressors. For this reason, role-playing and other techniques designed to teach new coping skills can be useful in helping the victims of crisis. This kind of help can even be obtained before a crisis occurs, and can be thought of as a psychological inoculation against disaster!

If it is likely that, at some point, a crisis situation will occur in our lives, we can prepare by learning problem-solving methods that allow us to respond appropriately when the time comes. Those with dangerous occupations can be taught methods for assessing their options in the event of an injury or psychological trauma. This involves the use of rational, non-emotional problem solving to handle the problem and make decisions regarding recovery.

Other stressful events, such as the loss of our home or personal belongings through fire or theft, can be dealt with by assessing the situation from a different perspective. This can take the form of cognitive restructuring, a method of modifying thoughts, perceptions and emotions related to a situation. The result: We learn to re-frame the loss so that grief and despair are not immobilizing. A sense of thankfulness to be alive and hope for rebuilding can foster a more positive attitude about our acute life situation.

Another important aspect of crisis intervention is to counteract the expected psychological and emotional withdrawal by helping the victim reach out and share the sense of grief, loss and despair. A major step in restoring psychological and emotional balance is to link our personal loss to losses suffered by others.

Deriving a sense of meaning and purpose from suffering, as well as understanding the nature of our own grief, can provide the strength needed for survival.

Believe it or not, life crises can have a long-term positive impact. For example, a recent sturdy of high school students who had suffered life crises such as gang violence or the death of friends indicated that 87 percent of those studied later developed a more positive view of life. The researchers stressed that many people have the ability to transcend pain and adversity, a position previously under-emphasized in other studies. In other words, cognitive changes were brought about by these individuals’ own painful experiences, changes that provided a means by which their understanding of life’s meaning could grow.

During a crisis, the courage to continue with life even while suffering personal grief is nurtured by providing compassion and help to others. A prime example is the founding of the group Mothers Against Drunk Drivers (MADD). MADD was founded by a Texas woman whose daughter was fatally injured by a drunk driver; instead of withdrawing into her own loss, despair and self-pity, she discovered that there were hundreds of other families who shared her grief and decided to do something about it. MADD now sponsors support groups for families who have been victims of drunk drivers, as well as funds national campaigns against drinking and driving. Essentially, she utilized a personal act of compassion to focus on the needs of others who had suffered similar losses, thus accelerating her own process of self-healing.

It is interesting to note that Chinese ideogram for “crisis” and “dangerous opportunity” are the same. A crisis can be seen as testing our attitudes, resources and creativity. Just as maintaining our physical, emotional and mental health helps us to take full advantage of positive opportunities, it will also facilitate our coping with a life crisis. It allows not only for more effective short-term coping-but also aids us in turning adversity into personal growth.

Reality Check

Learn to put stressful situations in perspective by asking these questions:

  • Does this situation reflect a threat signaling harm, or a challenge signaling opportunity?
  • Are there other ways to look at this situation?
  • What exactly is at stake?
  • What is the worst that can happen?
  • What are you afraid will occur?
  • What evidence do you have that this will happen?
  • Is there evidence that contradicts this conclusion?
  • What coping resources are available?

Your illness may bring out some of the best aspects of your personality, such as determination and patience, but it will also bring out some of the worst. It has the same effect on relationships. The problems you and your partner were having before the diagnosis will persist until you correct them. If the two of you had a difficult time making decisions about priorities, or talking about money, or relating to each other physically, you are probably still going to have to keep working to improve these aspects of your relationship.

Just like other people, sick or not sick, your problems do not go away by themselves. It is tempting to throw up your hands and say, “I’m not well. How can I deal with that?” or “I don’t think that’s important anymore. Why don’t we talk about real problems-like mine?” It’s not fair to use the illness as an excuse to quit on your partner, or yourself, or your relationship. You still have a responsibility to care and share and participate in your relationship like an adult.

Your perspective may change, however. Your illness, or the illness of your partner, may change the way you think about your problems and the way you approach them. You both may decide that some of the old issues are not issues any longer. Or, you both may decide that your time together is too precious to spend it discussing trivial differences. If a chronic illness has one beneficial effect, it is to hasten the process of change. These changes can be very positive if you know what you are trying are improve.

Post-Traumatic Syringomyelia

by James W. Little, MD, PhD, Associate Professor in the Department of Rehabilitation Medicine at the University of Washington, and Assistant Chief of the Spinal Cord Injury Service at the VA Puget Sound Health Care System

Originally published in the Fall 1998 issue of Spinal Cord Injury Update.

Syringomyelia is an uncommon but disabling complication of SCI. Although more than half of all people with SCI develop a cyst in the spinal cord at the injury site, only about 4% develop syringomyelia, in which the cyst fills with fluid and expands. This enlarged cyst, or syrinx, can damage the spinal cord and cause pain, loss of sensation, or weakness. Other symptoms may include low blood pressure with light-headedness, sweating, increased or decreased spasms, and impaired bladder emptying. In some cases, syringomyelia results in major loss of function.

Syrinxes (the dark area in the center of the spinal cord, which is a lighter grey, shown at right) form when the normal flow of cerebrospinal fluid is obstructed by either a bony narrowing of the spinal canal or the presence of scar tissue at the injury site. Coughing, sneezing, bearing down, or lifting a heavy weight may contribute to syrinx development because these activities increase pressure in the veins, forcing fluid into the cyst. Spinal fluid is thought to flow through channels that act as one-way valves: fluid flows in but little flows out. Pressure builds in the syrinx until it enlarges and ruptures, damaging normal spinal cord tissue and injuring nerve cells.

Sryinx

Syrinxes are diagnosed by magnetic resonance imaging (MRI), but patients can often detect the presence of a syrinx by testing themselves for loss of pin-prick and temperature sensation, which are early signs of syringomyelia. Quantitative strength tests and nerve conduction tests are useful for monitoring syrinx status and assessing the effectiveness of treatments. Nerve conduction tests can also help detect other causes of neurologic decline that may mask or occur simultaneously with a syrinx, such as carpal tunnel syndrome, other peripheral nerve entrapment, or spinal cord impingement.

Surgery is often used to prevent syrinxes from expanding. A shunt tube can be placed in the syrinx to drain fluid into the abdominal cavity. In a dural graft procedure, the space around the spinal cord is enlarged to allow free flow of fluid and reduce pressure. A cordectomy involves cutting across the spinal cord and opening up the syrinx to release fluid. All of these surgical procedures have risks and limitations, and none is ideal in patients with incomplete injuries and preserved motor function below the syrinx because of the risk that the surgery may result in the loss of some or all of this function. Although shunts are standard treatment in patients with complete injuries, there is a risk that the shunt tube will occlude (close up) and the syrinx will reaccumulate fluid, resulting in further neurologic decline. In some patients who receive dural grafts, symptoms worsen despite the surgery. Patients should continue to be closely monitored after surgery for signs of further neurological decline.

Non-operative treatments for syringomyelia usually result in only modest and temporary improvements. Water pills and vasoconstrictor medications may help reduce fluid formation around the spinal cord. Avoiding activities that increase venous pressure – high force and breath-holding exercises, a head-down position, and bending the trunk so the chest rests on the thighs – may reduce the risk of syrinx expansion. Quad coughing, in which a care giver compresses the abdomen during an attempted cough to improve its effectiveness, may cause pressure in the inferior vena cava (a large vein in the abdomen) that can be transmitted to the syrinx. To avoid direct pressure on this vein, compressions can be performed to one side of the center of the abdomen. Draining the syrinx with a needle can also yield temporary benefits, but fluid tends to reaccumulate. Treatments currently available are most useful for preventing further neurologic decline but may also improve strength and decrease pain.

Syrinxes and their symptoms vary a great deal from patient to patient. Weakness and pain may or may not be present, and weakness can develop rapidly or gradually. Patients with syringomyelia often need a wide range of rehabilitation services to help them manage pain and adjust to new weakness or loss of function. Regular follow-up with a rehabilitation practitioner experienced in the long-term management of syrinxes is essential in order to monitor symptoms, guide treatments, and prevent neurologic decline.

Reprinted with permission from Spinal Cord Injury Update, the newsletter of the Northwest Regional Spinal Cord Injury System (University of Washington Department of Rehabilitation Medicine, Box 356490, Seattle, WA 98195-6490; 206/685-3999).

The Prevalence of SM in the U.S.

by Marcy Speer Phd, Duke University

Syringomyelia is considered a rare condition, and early estimates suggested that approximately 21,000 individuals were affected with this condition. Given the current estimates on U.S. population size from the recent census, this figure would suggest that about 1/18,000 Americans have syringomyelia. However, this number probably underestimates the true frequency of syringomyelia. The “perfect” study to estimate the frequency of syringomyelia would involve testing a large group of people (probably more than 10,000) selected at random to see how many have syringomyelia. This type of study has never been done, and so we have tried to estimate the prevalence of syringomyelia by pulling information from the scientific and medical literature.

We started by listing the common causes of syringomyelia. Common causes include syringomyelia associated with spina bifida, post-traumatic syringomyelia, spinal cord tumors, arachnoiditis, and idiopathic syringomyelia. By searching the literature, we found estimates for how frequently each of these conditions occurs in the population and how frequently syringomyelia is associated with them (Table 1a). We looked for upper and lower estimates of how frequently these conditions occurred to get a range of numbers. The most common cause of syringomyelia, however, is related to the hindbrain (for example, those associated with Chiari type 1 malformation, basilar invagination, etc.) and there is no available estimate of the prevalence of Chiari type 1 malformation or other hindbrain-related abnormalities available. Therefore, we utilized data published by Dr. B. Williams in 1997 and determined that about 70% of syringomyelia is due to hindbrain-related abnormality, and then we used algebra to calculate backwards (Table 1b). By combining these numbers, we estimate that the prevalence of syringomyelia in the U.S. population is between 141,420 – 209,187 people; in other words, about 1 in 1,300 to 1 in 1,900 Americans have syringomyelia.

This estimate is about 10 times higher than the earlier estimate. Could this be too high? Yes, there are several potential problems with these calculations that may cause our estimates to be too high. For instance, any of the estimates of the frequency of the causes of syringomyelia that we used to generate our overall number could be incorrect. And, some physicians may argue that syringomyelia associated with spina bifida or spinal cord injury or other causes should not be included in these numbers. The most problematic contribution to these numbers comes from our estimate of the frequency of hindbrain related abnormalities, likely the most common cause of syringomyelia. Since no good estimate of the prevalence of Chiari type 1 malformation or other types of hindbrain-related abnormality are available, the numbers utilized here could be inaccurate. On the other hand, if the relationship between fibromyalgia and/or chronic fatigue syndrome with Chiari type 1 malformation proves to be true, these numbers could be higher.

If Regis Philbin were to ask, “Is this your final answer?”, we would have to say emphatically ‘no!.’ We can say with certainty that more than 18,000 individuals in the U.S. have syringomyelia, but how much higher is not well established. Time and added insight from research will continue to refine our estimates of the prevalence of syringomyelia, but we feel this is a good starting point based on available information.

Exercise Concepts for Individuals with Syringomyelia

by Jim Cavanaugh, PT, NCS Duke University

Many individuals with Syringomyelia (or Chiari I Malformation) can benefit from a consultation with a physical therapist familiar with the evaluation and treatment of neurological problems. Answering “yes” to any of the following questions indicates that you may have needs with which a physical therapist can help you.

1. Do you have difficulty

  • Positioning, turning or moving in and out of bed?
  • Moving from sitting to standing?
  • Moving from a wheelchair on to your bed, commode, or car seat?
  • Getting on and off the floor?
  • Getting in and out of the tub or shower?
  • Walking over level ground, indoors or out?
  • Walking over uneven ground outdoors?
  • Climbing stairs?
  • Falling, tripping, or stumbling?
  • Walking endurance?
  • Poor balance or weakness which limits your walking?

2. Do you have physical difficulty performing household tasks or work-related duties?

3. Do you have questions about exercise? Do you exercise regularly? Are you involved in a lifetime fitness program?

4. Do you experience pain and/or stiffness in your spine or extremities?

5. Do you experience excessive fatigue or dramatic fluctuations in your energy levels?

6. Do you have difficulties using mobility aids (crutches, canes, wheelchairs) or other equipment?

  • Is your equipment appropriate?
  • Is it well maintained?
  • Does it fit well?

Individuals with injuries or conditions which affect the brainstem and/or spinal cord experience a variety of symptoms and degrees of disability. In all cases, however, there is a potential risk for diminished activity levels and mobility. The secondary affects of this decline can result in further medical problems and functional limitations.

Although sensory loss, neurogenic pain and weakness, bowel/bladder dysfunction, and spasticity generally are not reversible through exercise, their impact on function may be moderated. In addition, secondary symptoms of disuse weakness, muscle and joint stiffness, fatigue, musculoskeletal pain, and unsteadiness may be improved through specific exercise.

Currently there are no available medically sanctioned generic exercise guidelines for individuals with Syringomyelia. All individuals are recommended to consult with a physical therapist familiar with neurological conditions for specific recommendations. Physical therapy services are available in every state and often require a physicians prescription.

Four Year Study of Chiari I Malformation

Four Year Study of Chiari I Malformation Redefining the Chiari I Malformation- a study of 364 patients. Highlights of ASAP conference presentation by Dr. Thomas Milhorat, Professor and Chairman of Neurosurgery University of NY, Brooklyn

Before MRI, Chiari Malformations were based on a set of pathological observations made in a limited number of patients more than 100 years ago. The current understanding of the Chiari I is a condition that involves herniation of cerebellar tonsils into the spinal canal. The condition can be associated with Syringomyelia and malformations at the base of the skull. Currently it has a narrow definition by radiologist- the herniation must be at least 3 millimeters beneath the opening of the skull; otherwise it is not regarded as a Chiari. Radiologists further disagree whether it should be 3 or 5 millimeters. This radiographic criteria is considered the beginning and the end of diagnosis. Since MRI there has been dramatic rise of cases and with it a great number of unanswered questions.

  • What is the cause of the Chiari Malformation?
  • What do patients have?
  • What does it produce? What is the clinical syndrome?
  • What is the risk of inheritance?

To answer these questions, a prospective study was begun with 364 patients. A database was created with family history; complete neurological exams and all radiographic findings were presented. Each patient had MRI’s of the head and spine.

Measurements were made of all the structures of the posterior fossa. A quantitative measurement was done and then using a sophisticated technique of volume measurement they measured the volume of the posterior compartment, the volume of the cerebellar and brainstem tissues in the posterior compartment and the volume of the cerebrospinal fluid.

The patient study included 126 patients with Chiari Malformations only, and 238 Chiari patients with a syrinx. There was a 3 to 1 preference female over male. Many had lifelong complaints, but the age of onset was defined as the age when the patient first sought medical attention. The mean age was 25 years. Diagnosis was made at a mean age of 30 years.

89 patients or of the study population could identify a precipitating event that brought on symptoms. The most common happening was an accident of some sort -such as motor vehicle, sports injury, bump etc. Some experienced Chiari Symptoms after childbirth or spinal taps. Coughing and sneezing brought on symptoms in some. The most common complaint of patients with Chiari with or without Syringomyelia was headache. This was a specific headache that began in the suboccipital region as pressure and pain and in some it radiated to the vertex of the head and behind the eyes, less often it radiated posteriorly down the shoulders. Often it was accentuated by postural changes or valsalva maneuvers (coughing, sneezing, straining) and in females it tended to be worse preceding menses.

Visual phenomena were common such as blurred vision, seeing stars or wavy lines, uncomfortable feeling in bright lights; less common was double vision. Females were often diagnosed with MS based on the ocular symptoms. Otoneurological symptoms were the leading cause of seeking medical attention. 74% of the patient population had a Meniere’s like syndrome with two or more of the following complaints: dizziness, disequalibrium, pressure in the ears, tinnitus (ranging from high pitched sounds to sounds of oceans flowing), decreased hearing, true vertigo, images jumping in from of the eyes. Nystagmus were the only major finding upon clinic examination despite all these symptoms. All types of nystagmus were found.

66% of patients had two or more cranial nerve, brainstem or cerebellar disturbances. They included apnea (episodes of irregular breathing during sleep), hoarseness, tremors of the hands, poor circulation, pain in throat, pain in the face, true syncope, shortness of breath, hypertension; 25% described palpitations. Two people can have the exact Chiari Malformation and one can be totally asymptomatic. The presence of a Chiari does not impute a set of symptoms.

We have come to understand the Chiari Malformation in the following way. It is a volumetrically too small posterior compartment of the skull. The posterior compartment is formed by the occipital bone in the back; this particular part of the bone is called the supraocciput. It’s formed by a roof, which is made up of a membrane called the tentorium. It has a front, which is the clivus. Then there is a mystical opening at the top of the tentorium and there’s a mystical opening at the bottom known as the foramen magnum. Now in the Chiari I malformation, the volume of this compartment is too small. The tentorium is sloped like the steeple of a church, not the roof of a house, where the normal tentorium comes out like this, the Chiari tentorium tends to be high rising. The back bone, as a consequence, tends to be short. In many patients with Chiari the clivus is short. Some patients with Chiari have basilar impression where the tip of the second vertebral body, the odontoid, tilts back. We refer to that as retroflexion of the odontoid. These make a small posterior compartment. Oh, and did he leave it out? There can also be herniation of the cerebellar tonsils through the foramen magnum. Milhorat has come to understand this as one of the consequences of a too small posterior fossa- not the actual phenomena. This is not the whole problem, but just a reflection of the too small posterior compartment.

n Milhorat’s study population, 34 patients or 9% had typical Chiari symptoms- typical headaches, visual disturbances, Meniere’s like syndrome, lower cranial nerve disturbances, 2/3 had syrinxes. They had a small posterior fossa, they had a high steeped tentorium, they had a short supraocciput, and they had a small clivus. But you know what? Their tonsils were not down enough to be called a Chiari Malformation so these patients were kicked out of the group. But if you look here at the T-2 weighted images where spinal fluid is white behind the cerebellum you can see this is a compressed posterior fossa with an absence of CSF posteriorally and lateral to the cerebellum. This is a Chiari Malformation with the tonsils at the level of the foramen magnum, and not grossly below it. So we are redefining the Chiari Malformation as not simply how far the tonsils come down, but it is a diagnosis, which includes 7 specific signs. They include: the volume of the posterior fossa being small, CSF volume being reduced, compression owing to smallness of the bones in the back and most of them have varying degrees of tonsillar herniation. The failure to have greater than 3 mm to 5 mm herniation does not exclude the diagnosis of Chiari I in our new definition.

In all 364 patients the most common and reliable finding was compression of the CSF spaces posterior and lateral the cerebellum. 332 of these patients had tonsillar herniation greater than 5 mm but 32 did not and we did not exclude them. These are patients, who had syrinxes, had headaches, they knew they had Chiari but they were excluded from having a Chiari and it was “idiopathic Syringomyelia” because they didn’t have the 5 mm tonsillar descent. These patients are now redefined as Chiari I Malformations.

In a desire to be more precise about the small posterior fossa, a control study of 50 normal people and 50 patients with presumptuous diagnosis of Chiari I were age and sexed matched with the following measured: the length or tentorium, angle or slope of tentorium, length of supraocciput, length of clivus, degree of tonsillar herniation.

The Cavalieri Method was used to calculate total volume of the posterior fossa and total volume of CSF spaces around the cerebellum. In patients with Chiari I, the size of the supraocciput was significantly smaller, clivus was significantly smaller, tentorium angle was increased, and there was an average of 9.8 mm tonsillar descent in this group. Normal tonsils should lie 2mm ABOVE the foramen magnum.

Professor Chiari and others throughout time have regarded the Chiari I Malformation as the least severe form of a series of hindbrain malformations. But could it be just something wrong with the bones of the skull and the consequences of compressing spinal fluid and compressing local structures contribute to the symptomotology and it has nothing to do directly with anything being wrong with the central nervous system?

Based on this study there is strong evidence to suggest it is a defect of mesodermal origin. It is a disorder of bones, and structures that support bone- muscles and ligaments and not nervous tissue. None of the 364 patients had evidence of neural defects such as missing lobes or abnormalities of the central nervous system. All patients had evidence of skull based dysplagia. Interestingly, the location of these bony changes- the back of the skull, the tentorium, the front of the posterior fossa, the anomalies at the cervical-medullary junction- are consistent with a defect of a very particular part of mesoderm. All these structures form together in the embryo and all are linked to one another in development.

This is powerful evidence that the Chiari I Malformation, although it shares the name of Chiari, is not a malformation of the central nervous system, it’s not a developmental anomaly of the hindbrain of the cerebellum and brainstem. But it is a too small posterior cranial fossa, owing to a maldevelopment of the skull.

How do symptoms occur? CSF that is compressed has to go somewhere. In man, under normal conditions, we secrete spinal fluid. We can increase that secretion but we cannot decrease it. It happens at .37 cc every minute of our lives. When we’re upright, sleeping, running, resting. We produce 500 cc a day. It’s formed in the ventricles, circulates down through the 3rd ventricle into the aqueduct of Sylvius, into the 4th ventricle. It exits and then it surrounds all the structures of the nervous system. There is a watery bath between brain, spinal cord, cerebellum and the inner table of the skull. In Chiari patients there is reduced volume. Where does it go? Some of it is distributed up into the head, squeezed up and some is squeezed down and this may very well contribute to the formation of SM in some patients. We believe this displacement of spinal fluid into the cranial cavity may be a suitable explanation for the typical Chiari Symptoms. Distribution of CSF out of the posterior fossa into the semi-circular canals can produce in some a sea-sickness syndrome- dizziness, disequilibrium, pressure in the ears, tinnitus, and so forth. It appears to all begin with a too small posterior fossa that compresses the spinal fluid at the back of the head.

Direct bony pressure can also produce complaints. If the compression of the skull is too great, if the retroflexed odontoid (basilar impression) is too great you can have direct compression of cranial nerves or even brainstem. That can produce problems with swallowing, sleep apnea, hoarseness, tremors, and palpitations. Symptoms of Chiari I appear to be due to two basic phenomena that share in common a small back of the skull, a bony dysplagia of the base of the skull and cervical-medullary junction. These are increase of CSF pressure both in the head and in the spinal canal and direct compression of nervous structures. It is not a malformation of the brain itself.

This anomaly can occur sporadically, and probably occurs most often in this fashion. It can also be associated with genetic transmission in some families. The precise mode of that transmission is under intense investigation thanks to a grant from ASAP to Duke University. 12% of the 364 study population had two or more affected family members.

In closing, Milhorat emphasized once again that an MR finding of a Chiari I does not impute a set of symptoms. Nor does it impute a prognosis except we have learned if you have a Chiari I Malformation you will do well to wear your seatbelt and stay out of harms way, and a Chiari I Malformation certainly does not impute surgical treatment.

Coping with syringomyelia and Chiari

by Shawna Countryman

A diagnosis of syringomyelia (SM) and/or Chiari (CM) may affect us in many different ways. Dealing with the various facets of a long-term condition can leave one feeling alone, frightened and confused. Not only will we have to deal with the physical attributes but also the emotional aspects. Emotions can vary from anger, disbelief, frustration, denial, loss of control, depression, fear, anxiety or uncertainty. An effective way to fight back is to make the most out of our lives. Take the time to experience the good things in life, the things that give us real pleasure.

One of the most important things to remember is that this diagnosis is not an end, but the beginning of a new learning life experience. Although it would be nice to pick and choose what our life experiences would be, we are usually not given that opportunity. With the right focus, we can decide whether to look at our situations as negative or as positive. We can choose the direction in which we take the experiences. Living with SM/CM will bring many unexpected challenges, but that doesnt mean we cant be in control of our lives.

If we are living with a chronic condition, we are courageous. A longstanding disorder can add a new set of challenges to our lives. In the process of meeting these trials, we will continue to learn how to confront our fears and move beyond them. We may feel that we lose some of our independence if we have to depend on family members, friends and health care professionals more than we did in the past. None of these changes are easy. While SM/CM brings many challenges, there are ways we can face them and live productive, healthy, fulfilling lives.

Change our outlook: Realize that only we can change our outlook on life. Some situations cant be changed, so it is up to us to learn how to deal with the situation effectively. Maintaining a positive attitude can decrease stress and help us to get the most out of life. We may not be able to change our diagnosis; however, we can become involved so that we get the most out of our lives by giving ourselves a new perspective as well as renewed hope.

Educate Ourselves. Learn all that we can about syringomyelia and Chiari. Knowledge is power. The more we learn, the better we will be able to empower ourselves. Develop a Healthy Attitude. A healthy attitude will help us to balance our positive and negative thoughts. Focus Our Attention on Positive Things We Enjoy. Continue a hobby, project or skill, or start a new one. Express Ourselves. Find ways to express our feelings in a positive way, such as writing in a journal, exercising, painting or joining a support group. Develop and Use Support Systems. Share our feelings with family, friends, physicians, counselors or others who have the same diagnosis. Realize We Are Not Alone. We may feel isolated and that no one understands what we are going through. Know that we are not alone. Meet and share with others who are going through some of the same things we are. Laugh. Become involved in activities that make us laugh. No matter how sad we feel, laughing can make the world seem like a better place. Relax. Learning how to relax is one of the most important ways to cope with stress in a healthy way.
Love. Love ourselves, our families, our friends and others who are important to us. Read. Read something that inspires us. Whether it is fiction, non-fiction, poetry or literature, find something we are interested in and start reading. We can join a book club or web ring, which enables us to discuss our thoughts with others having the same interest.

Everyone sees situations differently and has different coping skills. By understanding our reactions and ourselves, we can learn to deal with our diagnosis effectively. Some may be able to continue daily activities as always. Others may have to trade in their favorite activities for new ones. There is no single right way of coping. Each of us must figure out what works best. However, a combination of the following coping skills is ideal.

Emotional Identification – we may prefer to deal with our feelings and find social supports.

Distraction Identification – we may use hobbies or activities to help take our mind off of the situation.

Task Identification – we may feel comfortable analyzing the situation and taking action to deal directly with the situation.

Just as stressors wear us down, being active can rejuvenate, restore and refresh us. The following list may help us identify what we can do to remain active. By taking action, we have more control over our lives.

Volunteer Work. Helping others can take attention away from our own worries. Find an organization whose mission and goals we support; give to others.

Use Relaxation Techniques. Meditation helps to ease the mind so we can think calmly throughout the day. It also helps us to focus on the positive. Meditation puts us in control of our thoughts. Other ideas are deep breathing, yoga or massage.

Hobbies. Take time to focus on a hobby. Whether it is writing, photography, painting, crafts, collecting, gardening, sports or any other hobby, do it with passion and enthusiasm. Consider it nourishment for our souls.

Socialize. Become more active in church; attend a gathering, a concert, arts and crafts fair or a support group.

Exercise. If we are able to participate in exercise, it will help to keep our bodies and minds healthy. If we cannot actively participate, we can go to a park and savor the environment; delight in the flowers, birds and trees. Watch others who are also enjoying being outside.

Get Away. Taking a break from our day-to-day routine can be stimulating and/or relaxing.

Get Enough Rest and Sleep. It is important to give our bodies the rest and sleep they need, especially when we are dealing with a chronic condition.

Watch Our Diet. Alcohol, caffeine, sugar, fats and tobacco all put a strain on our bodies. A diet with a balance of vegetables, whole grains, fruits and high in protein but low in fat will help create good health.

Devote Some Time Each Day to Ourselves. Take time to relax by listening to music, reading a book, watching a good movie, or playing a game.

The late, great amateur golfer, Bobby Jones, was diagnosed with syringomyelia in 1956. He described how he faced this challenge when he said, I still cant accept this thing. I fight it every day. When it first happened to me I was pretty bitter, and there were times when I didnt want to go on living. But I did go on living, so I had to face the problem of how I was to live. I decided that I’d just do the very best I could.

Our situation can bring out the best in our character, such as patience, determination, motivation or empathy. Or it can bring out the worst. It is up to us as to which one we let prevail. People today can get so caught up in work or daily routines that they forget to look at the big picture. Having SM/CM may give us no alternative other than to slow down our hectic lifestyle. Look at this as a positive aspect, for it provides us the time to look at our priorities and make changes if necessary. When we live with a chronic condition, every aspect of life takes on a new dimension. Our daily decisions and choices are taken into account more carefully. Take time to let the sun shine on our faces, smell the air after a gentle rain, or just listen to the activities of nature that surround us. And most of all, remember that we are not alone.

About the Author:

My name is Shawna Countryman, and I was diagnosed with syringomyelia (SM) and Chiari malformation (CM) in 1996. After childhood and adult years of medical problems and numerous misdiagnoses, it was a relief to finally find out what was wrong. However, little did I know I would be in for the battle of my life. After searching for months and going from doctor to doctor, I finally found a wonderful neurosurgeon and physician. This, however, was not done without a lot of determination and will power to fight and never give up hope.

I had an occipital cranial decompression a few days after my 30th birthday in 1997. After the surgery, I was in need of a neurologist. Most I found were not familiar with SM or CM. One neurologist denied I had either disorder, even after the surgery and after several MRIs confirming the diagnosis. I was told I needed to see a psychiatrist instead. He stated that if I did have SM or CM, I would not be up walking around like I was, that I would be paralyzed. During the family history interview, I mentioned that my mother had been diagnosed with lupus. He said, So, do you think you have that, too? and laughed. This was one of the biggest turning points in my life.

Always a strong woman, I had taken the diagnosis and the surgery with stride and continued to maintain a positive attitude. However, on this day, after a great deal of humiliation and shock, I sat in my car in the parking lot and cried for two hours, unable to drive home. I could not believe this man treated me the way he did or that he was a well-known neurologist. After this experience, I was determined to help make a difference for all of us suffering from the effects of SM and CM. I decided to begin by making a coping brochure. I did extensive research on positive coping strategies and interviewed almost 100
people who also suffer from these disorders. I would like to thank those who donated their time to answer my interview questions. The benefits are clearly evident with the outcome of this brochure. May this inspire all of us to fight for the best quality of life possiblebecause we deserve it! Never give up. This experience has made me appreciate life so much more. I do not take life for granted, and I thank God for each day I am given. Whether it is a good
day or a bad day, it is another day that gives me the fortitude to become a stronger, more determined, more courageous woman than I ever imagined.

With a BA degree in Psychology, I am now pursuing my Masters in Counseling Psychology. I currently work with individuals with mental retardation and those with developmental and mental disabilities.

Always remember that together we can make a difference, not only in our own lives but also in the lives of others. When we have hope, we have everything. Count our blessings, not our troubles. I leave you with one of my favorite quotes:

Happiness is not a state to arrive at, but rather a manner of traveling. – Ben Sweetland

Love & Peace to You All,
Shawna Countryman

Canine Syringomyelia

by Clare Rusbridge BVMS DipECVN MRCVS

CavalierCould mans best friend help in the quest to understand syringomyelia? Chiari malformation occurs in dogs and, in some breeds, such as the cavalier King Charles spaniel, it is very common indeed. The Cavalier is a lovable and loving spaniel that was established as a breed in 1928 in response to a reward offered for recreating a spaniel similar to those depicted in portraits of the era of the British King Charles II. Their friendly disposition ensures that they are one of the most popular toy dogs in Britain, and they are also increasingly common in other western countries, including the USA.

Clare Rusbridge, a veterinary neurologist working in England, met her first dog with Chiari malformation, Beau, in 1995. Beau had been sent to Clare because he had a forelimb, (i.e., arm), weakness. In addition, he appeared to suffer from intense neck and shoulder irritation, especially when he was walked with a neck collar and leash. A consequence of this was that he scratched in the air with one hindlimb whilst walking. At first Clare was bewildered, and despite many tests, she could not find the cause of Beaus strange signs. It subsequently became apparent that Beau was not the only Cavalier with this scratching behaviour. Finally, in 1997, Clare was able to get access to a MRI scanner that could image animal spinal cords. Five dogs, including Beau, had a scan revealing that they all had syringomyelia and a Chiari malformation. Eight years on and hundreds of Cavaliers all across the world have now been diagnosed with the disease.

Cavaliers with syringomyelia usually have their first signs between 6 months and 3 years of age; however, dogs of any age may be presented. Progression of the disease is very variable. Some dogs have the tendency to scratch with mild pain only and other neurological signs never, or very slowly, develop. Others can be severely disabled by pain and exhibit neurological deficits within 12 months of the first signs developing. Many people suffering with syringomyelia may find the clinical signs experienced by dogs familiar. Pain is by far the most important feature of the disease. Many ask the question, How can you tell a dog is in pain? It is not very difficult, especially if the dog in question screams for apparently little reason, i.e., having its ear groomed. Once friendly, pets may become withdrawn and stop wanting to play. Owners often report that their dog is worse at night; when first getting up; during hot or cold temperature extremes; when excited; or related to posture, i.e., preferring to sleep with head elevated. The dog may seem to be overly sensitive to touch on one side of the neck, ear, shoulder, chest,  and often scratch at that area. Some dogs develop a scoliosis and some have weakness. Treatment of dogs is very similar to humans with the same condition. The bond between dog and owners can be as close as between humans, making it very difficult to make decisions about treatment such as surgery.

How does this help human sufferers? There are many possibilities:

  1. Understanding the genetics. Chiari malformation is inherited in this breed and, if the causal gene or genes could be identified, then scientists could study what this gene does and why Chiari malformation and syringomyelia develop. Clare Rusbridge and her team, with considerable help from Cavalier breed clubs and owners, have established a worldwide DNA collection from affected dogs and their families. They now have over 800 samples and are working with a neurogenetics laboratory in Montreal with the ultimate aim of finding the gene(s).
  2. Better understanding of the symptoms. Why are some dogs/people so much more painful than others? Why do some not make the expected improvement after surgery? Why do some patients have an intensification of pain after surgery? These are all questions that study of the dogs could help answer.
  3. Understanding how and why syringomyelia develops. In some litters of Cavalier puppies, it is highly likely that most will develop syringomyelia. These dogs could be studied, i.e., with serial MRI scans from an early age before and after the syringomyelia has developed. As a natural model of Chiari malformation, these dogs can provide useful information
  4. Improving treatment of both people and dogs. Better understanding of the disease leads to the possibility of more effective drugs and surgery. The Cavaliers in question are loved pets and are cared for by veterinary neurologists. They vastly outnumber human patients so there is a greater possibility to learn from successful experiences and apply these to humans. Similarly, human sufferers can provide dog owners with an understanding of what their pets can be experiencing and ensure they are provided with care.

Syringomyelia is a devastating disease in both dogs and humans. We hope that the veterinary and medical professions working together on this devastating disease can help man and his best friend

References

  • Rusbridge C and Knowler SP, 2003 Hereditary aspects of occipital bone hypoplasia and syringomyelia (Chiari type I malformation) in cavalier King Charles spaniels. Veterinary Record 153: 107-112
  • Rusbridge C and Knowler SP, 2004 Inheritance of Occipital Bone Hypoplasia (Chiari type I malformation) in Cavalier King Charles spaniels. Journal of Veterinary Internal Medicine 18, 673-678.
  • Rusbridge C, MacSweeny JE, Davies JV, Chandler KE, Fitzmaurice SN, Dennis R, Cappello R and Wheeler SJ, 2000 Syringomyelia in Cavalier King Charles Spaniels. Journal of the American Animal Hospital Association 36: 34-41.
  • Clare Rusbridge BVMS DipECVN MRCVS Stone Lion Veterinary Centre, 41 High Street, Wimbledon, SW19 5AU Tel: 00 44 208 9464228, Fax: 00 44 208 7860525 neuro.vet@btinternet.com