Research- Arnold Chiari malformation is an abnormality at the base of the brain which causes the brain to be pushed down into the top of the spinal cord, which is lower than usual. Chiari malformation is divided into three groups, depending on the severity of the abnormality. In 1883, John Cleland became the first person ever to describe, or discover Arnold Chiari malformation. Unaware of Cleland's work, two colleagues, Professor Chiari and Dr. Arnold, also reported cases of Chiari malformation in 1891. That is how the disorder got it's name. The only way to positively identify a Chiari malformation is by having a MRI, which being a new piece of technology, caused many people to be misdiagnosed before the invention of the MRI machine. There is a wide range of symptoms from Chiari. The only treatment for Chiari malformation is neurosurgery, although there are four different choices as to which surgery is performed. Chiari malformation is a disorder that can change the lives of the people it affects.
Purpose- The purpose of this experiment is to investigate whether or not symptoms in Chiari are related to the gender of the person affected.
Hypothesis- The symptoms in people with Chiari are not related to whether the person is male or female.
Procedure- This experiment was conducted in the following way:
1. Locate participants through Internet.
2. Prepare survey.
3. Send out surveys and consent forms to participants.
4. Separate male responses from female responses.
5. Record top five most common symptoms from female responses.
6. Do the same for the male responses.
7. Compare results.
8. Observe differences in top five symptoms from each group.
9. Make graph to show results.
Results- Both males and females top five symptoms are headaches, ear problems such as ringing in ears, hearing loss, and ear pain, vision problems such as vision loss or blurred vision, swallowing or choking problems, and loss of balance or dizziness.
Conclusion- By conducting this experiment, I have learned that symptoms caused by Chiari malformation are not related to whether the person(s) affected is male or female. Both males and females had the same top five symptoms. I chose to do this project because I have a Chiari malformation and it is a personal interest to learn more information about something that affects me.
Acknowledgments
Many people helped to make it possible to conduct my experiment. I sent my survey out to eighteen different people all around the world. They have all been willing to take the time to answer my survey questions and sign and return the consent forms.
Purpose Statement- The purpose of this experiment is to investigate whether or not symptoms in Chiari malformation are related to the gender of the person affected.
Hypothesis- The symptoms in people with Chiari are not related to whether the person is male or female.
Variables- The independent variable is that all participants have Chiari malformation. The dependent variables are the symptoms caused by Chiari and the gender of each participant.
Materials
Computer
Access to the Internet
Participants that have Chiari malformation
Questionnaires or surveys
Signed and returned consent forms
Procedure
I. Locate participants through Internet.
2. Prepare a survey.
3. Send out surveys and consent forms to each eligible participant.
4. Separate male responses from female responses.
5. Record top five most common symptoms from female responses.
6. Record top five most common symptoms from male responses.
7. Compare the results.
8. Observe differences in top five symptoms from each group, if there is one.
9. Make a graph to show the results.
Graphs of Female and Male responses
Results- Both males and females top five symptoms are headaches, ear problems such as ringing in ears, hearing loss, and ear pain, vision problems such as vision loss, or blurred vision, swallowing or choking problems, and loss of balance or dizziness.
Conclusion By conducting this experiment, I have learned that symptoms caused by Chiari malformation are not related to whether the person(s) affected is male or female. Both males and females had the same top five symptoms. I chose to do this project because I have a Chiari malformation and it is a personal interest to learn more information about something that affects me.
Arnold Chiari malformation is a developmental abnormality at the base of the brain which causes part of the brain to be pushed down into the top of the spinal cord. Chiari malformation is divided into three groups, depending on the severity of the abnormality.
John Cleland was the very first person ever to describe Arnold Chiari malformation. This was done in 1883. He described pathological findings in nine infants. These findings were elongation of the brainstem and the cerebellum extending into the spinal cord. Unaware of Cleland's work, Professor Chiari reported two types of abnormalities in the back of the brain in1891. Type I had the tonsils of the cerebellum extending below the opening in the skull where the spinal cord meets the brain. Type II had the medulla and fourth ventricle descended. Spina bifida was also associated with Type II. One of Professor Chiari's colleagues, Dr. Arnold, added to the description of Chiari malformation. This caused the name to become Arnold Chiari malformation. Schwalbe and Gredig, two of Arnold's students were the first to describe the "kink" at the base of the brain in the Type H malformation. They called the cerebellar malformation Arnold's malformation and the medullar malformation Chiari. This uncertainty of what to call the malformation still exists today. Some authors prefer to use the term Arnold Chiari for the Type II, and other like to use the classification of Chiari Type I and Chiari Type II.
The only way to positively detect an Arnold Chiari malformation is by having an MRI (magnetic resonance imaging). This being a fairly new piece of technology has caused many people to be misdiagnosed with other things, most commonly multiple sclerosis. In the past, people have died from not being treated. The symptoms of Chiari are typically subtle and come on over a period of time. Because of this, people go months and sometimes even years living daily with symptoms that are caused by Chiari. Some of the major symptoms are: blurred vision, burning/shooting pain in head or neck, decreased sensation in limbs, mouth and throat, difficulty adjusting vision to changes to light, difficulty focusing vision on printed materials, difficulty walking down stairs or on uneven surfaces, dizziness or vertigo (blackout feeling when standing or changing positions), double vision, feeling pressure behind eyes, foot drop, general loss of balance, head pain when getting up, changing positions, coughing or sneezing, frequent headaches, heaviness on chest when lying flat, loss of gag reflex, sense of smell, feeling on skin surface and taste, muscle spasms, restricted movement in neck, pain along eye, jaw and ear on same side of face, pressure in neck, ringing in ears, shallow breathing, swallowing problems, and swaying back and forth when sitting or standing. These symptoms may not sound like a big deal, but when you suffer from them, not only do they affect your every day life, but they also can be very scary
The treatment for Chiari malformation is neurosurgery. Most surgeries for Chiari report a 70% success rate in helping with symptoms. There are four categories of surgery. There are: 1) decompression of the back of the brain by removing a portion of bone in the skull at the base of the brain (suboccipital) with a craniotomy which is the opening of the skull and upper cervical area. Also, a laminectomy which is the removal of the portion of the bone to gain access to the spinal nerves and opening the tissue covering the base of the brain with the placement of a patch to cover the area where the bone was removed. 2) draining the syrinx which is a cavity of fluid in the spinal cord and opening the spinal cord to drain the area with possible placement of a catheter leading from the syrinx to another space to create a pathway for constant draining. 3) making a pathway for fluid to drain by cutting off the lower tip of the spinal cord. This procedure is called terminal ventriculostomy. 4) suctioning out the fluid from the syrinx. There is a 30% failure rate in all of these procedures. Some doctors like to treat the Chiari malformation at the same time the syrinx (if one exists) is treated. Syrinxes are not always present.
Most neurosurgeons agree that treatment should include the first procedure that was explained. When there is syringomyelia, which is the condition of having a syrinx, along with the Chiari, there is much less agreement on what procedure should be used. After treatment, patients with Arnold Chiari malformation alone have a better prognosis than patients with syringomyelia and Arnold Chiari. Although treatment may prevent the symptoms from getting worse, there is seldom an improvement. Therefore any disabilities or symptoms that have occurred before the diagnosis and treatment are likely to be permanent.
Chiari malformation was first thought to be found only in teenage years and beyond. We now know that there are just as many pediatric cases as adult cases. Symptomatic Chiari malformation with and without a syrinx have been increasing in recognition in children. The MRI machine has contributed to this fact. It is hoped that earlier diagnosis and treatment of Chiari before it advances will result in better long-term results.
Survey
1. Name
2. Sex
3. Date of birth
4. Age at diagnosis
5. Age at surgery
6. City and state or country that you reside in
7. What type Chiari malformation do you have? (Type I, II, or
III)
8. What other diagnoses do you have related to Chiari? (ex.
scoliosis, syringomyelia, or
hydrocephalus)
9. Please list ten of the most important symptoms in order of
what you feel is most
important/troublesome.
10. How do you relieve these symptoms?
11. What ONE symptom bothers you the most and why?
12. How do you deal with Chiari as a whole?
13. Did you have any symptoms pre-op that you felt were very
strange? How about
post-op?
14. What improvement in symptoms did you get from surgery?
15. Is there any particular skill that is better?
16. What is something you had to give up that you miss the most?
Bibliography
Abbott, R. Arnold Chiari Malformation; Diagnostic Evaluation. Beth Israel Medical Center, North Division. New York City.
Breger, M. & Haines, 5. (1991). Current Treatment of Chiari Malformations. Department of Neurosurgery, University of Minnesota Hospital and Clinics.
Chiari Malformation. (1996, May). National Institutes of Health National Institute of Neurological Disorders and Strokes.
Chiari Malformation: Facts Sheet. Center for the Study of Inherited and Neurological Disorders, Duke University Medical Center.
Feldstein, N. Pediatric Neurosurgery: Chiari Malformation Columbia-Presbyterian Medical Center, New York City.
Guin, P. & MacDonald, J. (1997, January 5). Arnold Chiari Malformation, A Close Look. Shands Hospital, Gainesville, Florida.