In September of 2001, after many years of symptoms of headaches, culminating with neck pain, numbness in my hands, loss of balance, fainting and increased intracranial pressure, I was diagnosed with Arnold Chiari Malformation and Syringomyelia.
I had already been dealing with Pseudo Tumor Cerebri, or Intra Cranial Hypertension for most of my life and had dealt with a series of misdiagnoses, multiple surgeries, lost visual fields and severe headaches. Once I was diagnosed with Chiari Malformation, a lot of the pieces of my medical puzzle began to fit together. But little did I know where this journey was going to take me. In short, I had 11 surgeries between 2001 - 2005. And I had already had a series of surgeries in the 70's and 80's too, leaving my body looking much like a road map.
Simply put, Chiari Malformation is where your brain is too big for your skull and it pushes out from beneath the opening of the skull and putting pressure on the brain stem. If there is enough blockage over a long period of time, another condition can form, Syringomyelia. A cyst- like cavity called a syrinx filled with spinal fluid opens up within the spinal cord, causing the cord to compress the bony and nerve-laden vertebrae of the spine. My syrinx ran from C 2 - 6. The nerve functions from those nerves have been permanently damaged.
Chiari is not a new disorder. Nor is it overly rare. Many people have it, and are never symptomatic in their lives. But some of us do become symptomatic due to either trauma or the aging process. My neurosurgeons have deduced that in my case I became symptomatic at the age of 13 due to a fall on my tail bone from a slumber party prank. At the time, in 1971, we were in the medical dark ages compared to today's diagnostic tricks. I had headaches and double vision, and after a failed test for a brain tumor it was concluded from the massive amounts of cerebral spinal fluid I was producing that I had Pseudo Tumor Cerebri, or Intracranial Hypertension. Too much CSF was being produced for the body to absorb, so it was sitting in my head causing headaches, double vision and compressing the optic nerves. After a few months of spinal taps to drain off the excess fluid, I had a lumbar peritoneal shunt placed. And that shunt worked for over 30 years, much to the disbelief of several neurosurgeons for the following years.
Anyway, all that stuff aside, diagnosing Chiari in the old days was a diagnosis of exclusion, and not often done. Now, with today's advanced MRI's, doctor's are seeing more of this potentially debilitating condition. But is it being treated any better? The answer is not much. Most neurosurgeons and neurologists still do not see enough Chiari in their medical practices to stay up to date with the latest findings in research. A handful of neurosurgeons and neurologists have dedicated themselves in the last 10 years to the complexities of Chiari and Syringomyelia, and their related disorders. Those of us who are their grateful patients travel many miles for care and wait for months for appointments. We have often been through the wringer of disbelief and misdiagnosis. Many of us have left jobs that we loved and have made radical changes to our way of life to try to give some relief from our many symptoms.
The reason I have included this information on my website is that a portion of my profits is dedicated to helping those with these disorders get the help that they need to seek appropriate medical care. Many face incredible financial hardships to see the doctors that will give them back the quality of life that they deserve. You can find out more about "us" on www.asap.org, and www.columnofhope.com on their message boards. You are welcome to visit with us! I am dedicating a portion of my profits to Wishes and Rainbows, a nonprofit charity that reaches out to patients and their families with Chiari and Syringomyelia and assists them with travel needs, emergency funds. At Christmas, Wishes and Rainbows makes sure that children with Chiari have presents under the tree. Please check out their website, www.wishesandrainbows.org Your purchase through me is not tax deductible, however your contributions to Wishes and Rainbows are, as they are a non-profit,501(c)(3) tax exempt organization, should you wish to make a contribution directly to Wishes and Rainbows. Both ASAP and Column of Hope do great work in supporting those of us with Chiari and Syringomyelia, paving the way to provide funding for research in spinal cord injuries, and gaining better recognition and patient support in the medical sector. ASAP has also been recognized by the Christopher Reeve Foundation for its efforts.
Thank you, and Peace be with you,
Chiari, From www.ASAP.org
What is the Chiari Malformation? (Kee-AR-ee) The Chiari I Malformation is considered a congenital malformation, although there have been some reported cases of an acquired form. It is characterized by a small or misshapen posterior fossa (the compartment in the back of the skull), a reduction in cerebrospinal fluid pathways and a protrusion of the cerebellar tonsils through the bottom of the skull (foramen magnum) into the spinal canal. The tonsils would normally be round but often become elongated as they protrude down the spinal canal. Diagnosis can be difficult because not all patients will have the classical sign of deeply herniated tonsils. Since the advent of MRI, the incidence of the Chiari I Malformation has risen dramatically. MRI is safe and painless and currently the most reliable means available for diagnosing Chiari Malformations. Chiari Malformations are also known as herniation of the cerebellar tonsils, cerebellar ectopia, hindbrain herniation and Arnold-Chiari malformations. A German pathologist, Professor Hans Chiari, first described abnormalities of the brain at the junction of the skull with the spine in the 1890's. He categorized them in order of severity, types I, II, III, and IV. The Chiari type II Malformation is usually found in children with spina bifida or myelomeningocele. Not only is part of cerebellum unusually low and lying below the bottom of the skull, but the brain stem can be malformed in several ways. Types III and IV represent gross herniations of the cerebellum and are very rare. What are the symptoms? Many people with the Chiari I Malformation experience no symptoms. When symptoms are present, they usually do not appear until adolescence or early adulthood, but can occasionally be seen in young children. The majority of patients complain of severe head and neck pain. Headaches are often accentuated by coughing, sneezing or straining. Patients may complain of dizziness, vertigo, disequilibrium, muscle weakness or balance problems. Often fine motor skills and hand coordination will be affected. Vision problems can also occur. Some patients experience blurred or double vision, difficulty in tracking objects or a hypersensitivity to bright lights. Physical examination may reveal nystagmus (involuntary eye movements). Other symptoms include tinnitus (buzzing or ringing in the ear), hearing loss or vocal cord paralysis. Patients may have difficulty swallowing, frequent gagging and choking and, in some cases, sleep apnea may be present. The Chiari I Malformations may also be associated with other disorders such as hydrocephalus (build up of fluid in the ventricles of the brain) or Syringomyelia. Syringomyelia is a disorder in which cerebrospinal fluid enters the spinal cord, forming a cavity known as a syrinx. It is recommended that patients diagnosed with a Chiari Malformation have the entire spine imaged to rule out the presence of a syrinx, since it may be a consideration in treatment and prognosis. Is there a treatment? Surgical procedures to enlarge the posterior fossa are considered a treatment option for patients with the Chiari I Malformation. Techniques are quite diversified amongst neurosurgeons, and patient responses vary greatly. A successful surgery will alleviate pressure on the neural elements and may result in an improvement of symptoms. The decision to treat a Chiari Malformation surgically requires careful consultation between patient and physician. Factors to be considered are the patient's current neurological condition and progression of symptoms over a period of time. Is this condition hereditary? Research into the risk of inheritance for the Chiari I Malformation is still in its early stages. In some families, more than one member has been documented to have the Chiari I Malformation. Familial recurrences are suggestive of a possible genetic component of the condition, but unfortunately there is no conclusive answer to the question of inheritance at this time. It is currently recommended that only those relatives experiencing symptoms commonly associated with the Chiari I Malformation need undergo investigational procedures. In 1998, ASAP funded a Genetic Research Study at Duke University.
Syringomyelia, from www. ASAP.org
What is syringomyelia? (sear-IN-go-my-EEL-ya) Syringomyelia, often referred to as SM, is a chronic disorder involving the spinal cord. For reasons that are only now being understood, cerebrospinal fluid enters the spinal cord, forming a cavity known as a syrinx. (Doctors sometimes use other words such as cyst, hydromyelia or syringohydromyelia) This syrinx often expands and elongates over time, destroying the center of the spinal cord. As the nerve fibers inside the spinal cord are damaged, a wide variety of symptoms can occur, depending upon the size and location of the syrinx. There are two major types of SM. In most cases it is related to a congenital malformation involving the hindbrain (cerebellum) called a Chiari I Malformation, named after the physician who first described it. This malformation occurs during fetal development and is characterized by downward displacement of the lower part of the brain (cerebellar tonsils) beneath the foramen magnum, into the cervical spinal canal. This displacement blocks the normal flow of cerebrospinal fluid. When normal flow is obstructed, a syrinx can then form in the spinal cord. Not all patients with Chiari Malformations will develop a syrinx, however. SM can also occur as a complication of trauma, meningitis, tumor, arachnoiditis, or a tethered spinal cord. In these cases the syrinx forms in the section of the spinal cord damaged by these conditions. As more people are surviving spinal cord injuries, more cases of post-traumatic SM are being diagnosed as the syrinx can form years after the trauma. What are the symptoms? The symptoms of SM are numerous and a person may have various combinations of different symptoms. Symptoms tend to develop slowly, although sudden onset may occur with coughing and straining. Some common symptoms include: loss of sensitivity, especially to hot and cold, muscle weakness and spasticity, motor impairment, loss of bowel and bladder control, as well as osteoporosis and scoliosis. The majority of patients suffer from headaches and chronic pain. Although Syringomyelia was first described over 400 years ago, very little research was performed and as a result little was known about the disorder. Only recently, with the advent of MRI and the dramatic rise in diagnosed cases, have significant advancements been made. In the past, SM was considered a disorder that always resulted in slow, progressive degeneration. Researchers now believe it is possible that some patients may never progress. What should be done? The first step after diagnosis is finding a neurosurgeon who is experienced in the treatment of SM. Finding a specialist is highly recommended. Surgery is the only viable treatment for SM, but not all patients will advance to the stage where surgery is needed. Evaluation of the condition is often difficult because SM can remain stationary for long periods of time, and in some cases progress rapidly. Treatment is aimed at correcting the condition that allowed the syrinx to form, if possible. In cases involving a Chiari Malformation, the main goal of surgery is to provide more space for the cerebellum at the base of the skull and upper cervical spine. This often results in a flattening or disappearance of the syrinx as the normal flow of cerebrospinal fluid is restored. In some cases it may be necessary to insert a shunt into the cavity, rerouting the cerebrospinal fluid from the spinal cord. A successful surgery will stabilize the condition and perhaps gain a modest improvement in symptoms, but over time surgery is not always successful and multiple surgeries may be necessary. Since the natural history of SM is poorly understood, a conservative approach may be recommended. When surgery is not yet advised, patients should be carefully monitored by a neurologist or neurosurgeon. Periodic MRI's and physical evaluations should be scheduled at the recommendation of a qualified physician.
The American Syringomyelia Alliance Project, Inc. (ASAP) is a non-profit, 501(c)(3), member supported organization, chartered in May 1988. Don and Barbara White started ASAP as a grassrootsorganization in their spare bedroom due to the frustration they encountered when Barbara was first diagnosed. Since then ASAP has become a nationwide clearinghouse for information regarding Chiari (CM) and syringonyelia (SM) and related disorders. ASAP sponsors an annual medical conference linking the top medical professionals in the field with those affected. ASAP reviews and funds research annually. In addition, they promote awareness by hosting community fundraisers throughout the nation. Together with its membership, ASAP works toward fulfilling its mission to improve the lives of persons affected by syringomyelia, Chiari malformation and related disorders while we find the cure.