This is the story of Shannon Hudson Terry and what might have been had she had this information earlier.
Chiari is a highly undiagnosed condition and is widely unknown and misunderstood. The longer you delay a Chiari diagnosis, the greater chance of additional neurological damage. Shannon’s Chiari diagnosis was over 5 years after the onset of symptoms, which initially included severe headaches and anxiety, later followed by depression. After she began to lose the use of her hand and leg, Shannon was finally diagnosed and underwent Chiari decompression surgery. Unfortunately, it did not succeed for her although it apparently does relieve symptoms in about 80% of attempted procedures. During the remainder of her life, Shannon lived in excruciating pain with extreme insomnia plus about 6 other Chiari symptoms. Unfortunately, Shannon was unable to survive with the condition and left us earlier this year (2011). Chiari is not a rare disease, just rarely diagnosed on a timely basis!!! Chiari affects 1 in 1000 people, and 3 of 4 of those are women.
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After reviewing the available literature on the internet, it appears that if any of the common symptoms or combination of symptoms applies to you or a loved one, it is critical that you immediately talk to your physician about Chiari.
There are many symptoms associated with Chiari that are also associated with other conditions. The table to the right (from the Conquer Chari Site shows many of the symptoms of Chiari and the percentage of Chiari sufferers that experience each of those symptoms. What is important to understand is that 95% OF CHIARI SUFFERERS EXPERIENCE 5 OR MORE OF THESE SYMPTOMS. If you or someone you know are experiencing any of these symptoms, it is crucial you investigate the possibility of Chiari further.
Please view the following set of slides from http://www.conquerchiari.com which will describe in more detail the symptomatology that is associated with Chiari.
(click the box icon in the upper right corner of the slide viewer to view full page)[googleapps domain=”docs” dir=”viewer” query=”url=http%3A%2F%2Fwww.conquerchiari.org%2FSYMPTOMS%2520Presentation%25202011.pdf&embedded=true” width=”600″ height=”400″ /]
Additional Chiari symptomatology can be seen from this excerpt from “The Wisconson Chiari Center”.
Chiari malformation is a congenital (i.e. present at birth) structural problem at the base of the skull that affects the brain and spinal cord. In fact it is not a malformation of the brain at all. More accurately, the lower portion of the skull, the portion known as the posterior fossa, is too small to accomodate the lower portion of the brain. As a result, a portion of the cerebellum called the tonsils is forced to protrude into the spinal column. This results in compression of the brain stem and spinal cord and interferes with the flow of spinal fluid. It is the compression of the brain stem and spinal cord that is primarily responsible for the symptoms. We have learned that compression that is sufficient to cause symptoms can occur even when the cerebellar tonsils protrude by as little as 1 millimeter.
- Types of Chiari Malformation
- Testing and Diagnosis
- Incidence of Chiari Malformation
Despite the fact that Chiari malformation is usually present at birth, the symptoms of the malformation are usually not experienced until adulthood, usually when the patient is in their 20s or 30s. Symptoms can develop sooner or later than this. Chiari malformation is often difficult to diagnose because the symptoms can be vague and so numerous that many doctors just can’t make sense of them.
The symptoms of Chiari malformation include:
- Headache – This is the most common symptom of Chiari malformation. The headache is often pressure like and usually begins in the back of the head often radiates behind the eyes. Coughing, laughing, bending forward, sneezing or looking up can worsen the headache. Headache may be confused with migraine.
- Neck Pain – often pressure like and radiating down the spine and across the shoulders.
- Dizziness/vertigo – often worsened by extension of the neck
- Vague pains throughout the body
- Impaired balance
- Chronic nausea
- Foggy thinking, poor memory and concentration
- Frequent or urgent urination
- Irritable bowel syndrome
- Auditory Symptoms – ringing in the ears, decrease in the ability to hear or sensitivity to sounds
- Difficulty swallowing
- Changes in the voice – This can include hoarseness or the inability to regulate the voice when shouting or singing.
- Visual Symptoms – including double vision, sensitivity to light, blind spots.
Types of Chiari Malformation (CM)
Type I involves the extension of the cerebellar tonsils (the lower part of the cerebellum) into the foramen magnum.There are usually no other associated malformations of the brain. This is the only form of Chiari malformation to present in adult life.
Type II involves the extension of both cerebellum and brainstem tissue into the foramen magnum. Type II is always accompanied by a myelomeningocele — a severe form of spina bifida- and therefore is recognized at birth. We do not treat Chiari II malformation.
Type III is the most serious form of CM. It is quite rare and involves the protrusion or herniation of the hind part of the brain through a defect in the skull. This diagnosis is also evident at birth. We do not treat Chiari III malformation.
Some people describe a Chiari 0 malformation. This is simply a Chiari I malformation in which the cerebellar tonsils do not protrude by the standard 5 mm. These are the patients that are frequently misdiagnosed because of the failure of the current diagnostic criteria to accurately reflect the underlying neurological problem. Many of our patients would fall into this category.
Testing and Diagnosis
The diagnostic process begins with a complete medical history and physical examination. These are followed by a complete neurological evaluation that will assess your specific symptoms and neurological functioning. The patient with a Chiari 1 malformation may have an entirely normal examination. In such cases, it becomes more difficult to determine if Chiari is indeed the problem and if surgery is indeed the best solution. In such cases, the judgment of an experienced neurosurgeon becomes very important. When the neurological examination is abnormal, the pattern of abnormal findings can make it very easy to pinpoint the problem to the cervical spinal cord and brain stem.
Patients may have difficulty with coordination, balance, eye movements, sensation and strength. The reflexes may be too active or not active enough. Reflexes that are never present except when the spinal cord is not functioning normally- pathological reflexes- may also be detected.
Magnetic Resonance Imaging (MRI) is best diagnostic tool for detecting Chiari malformation. This test provides the doctor with detailed anatomical information regarding the structures at the base of the brain. The criteria for diagnosing a Chiari malformation however have been very arbitrarily determined. This results in some doctors adhering to rigid standards – 5mm of tonsil herniation- before making the diagnosis of Chiari, even in the face of typical patient symptoms and obvious neurological findings. Hence many patients with Chiari go undiagnosed and untreated for years. We have developed some MRI imaging strategies that demonstrate the critical problem – brain stem compression- more clearly. These MRI techniques allow us to identify some Chiari patients who would otherwise go undiagnosed. Our imaging techniques involve the use of 3-D imaging of the brain and cine imaging – a movie of the brain- that allows us to determine if the brain stem is being compressed by the pulsating arteries that surround it. Examples of this imaging can be found on our web site.
Incidence of Chiari Malformation
The incidence of Chiari 1 malformation is not known because we have yet to fully understand the problem and arrived at a meaningful MRI standard for making the diagnosis If the 5mm standard of tonsil herniation is used, the incidence would seem to be less than 1% of the population. Brain stem compression due to tonsil herniation less than 5 mm – “Chiari 0”- is likely to be very much more common than that. For reasons not yet understood, Chiari malformation is much more common in women than in men. (3:1 women:men)
Some people with Chiari malformation have no symptoms and do not require treatment.
Some symptoms of Chiari malformation such as headache and dizziness may be effectively treated by medication. Medication alone, however, will not correct the root cause of the symptoms: the brain stem compression. As a rule, when the Chiari malformation causes symptoms that seriously impair the patients quality of life, it is best to consider surgery.
Surgery for Chiari malformation is basically about creating more room for the brain. Surgery relieves compression of the brain stem and spinal cord and improves circulation of the spinal fluid.
This information is only a small portion of the Chiari information that is available on these and other websites. Please visit these and the other sites you can find in our link list on our home page (in the right column) for more extensive details of this disease. Use this information to help yourself or someone you know to get the proper diagnosis.
The following is a recent story about a Chiari patient from the Ft. Worth Star-Telegram dated June 15, 2011…
BY ELIZABETH CAMPBELL
BURLESON — Clyde Walker spends most of his time propped up in a recliner, unable to do much for himself.
Standing, walking or even lying in bed sends pain shooting through his body. He has difficulty breathing and swallowing food, and his arms are becoming weaker by the day.
He can’t work as a mechanic anymore, he’s been forced to move out of his home, and he delayed seeking treatment because of his inability to pay.
“I’m dying an excruciating death,” Walker said, describing his rare condition, Chiari malformation, in which the brainstem doesn’t form properly. That prevents spinal fluid from circulating properly, causing nerve damage and other complications.
“I am losing my business because of this. I can’t keep up in the office anymore,” he said.
But some relief may be on the way.
Walker, 50, was recently chosen to participate in a five-year study at the National Institute of Neurological Disorders and Stroke, which is part of the National Institutes of Health in Bethesda, Md.
On Sunday, Walker and his brother Tony flew to Maryland, and he is scheduled to undergo surgery Friday.
Dr. John Heiss, a neurosurgeon who will perform the operation, said Chiari malformation affects 1 in 10,000 people. The surgery lasts several hours and involves removing the bone from the base of the skull, creating more space for spinal fluid to circulate. The surgery is not a cure, but doctors hope it will improve Walker’s quality of life.
The milder form of Chiari malformation causes headaches, but Walker’s symptoms are more severe, he said. Walker’s spinal cord is injured because of the malformation, and his upper body is starting to weaken, Heiss said.
“Our main reason for doing the surgery is to keep Mr. Walker from getting worse,” Heiss said. “Now, he is having pain and weakness in his arms. We don’t want things to progress to the point where he can’t walk or do things for himself.”
Paralysis can lead to other complications, such as infections and blood clots.
The study will track patients’ progress after the surgery. In many cases, Heiss said, people have surgery but no follow-up takes place.
Dr. Gregory Skie, who practices in Mansfield, said he diagnosed Walker’s Chiari malformation. The cerebellum extends below the head into the spinal column, putting pressure on it.
“Mr. Walker was more complex than I imagined,” Skie said. “He’s typical of my male patients. He didn’t get in to the doctor like he should have.”
Skie said another doctor referred Walker to him because he could not figure out what was wrong. He said Walker’s situation had grown worse in the past couple of years because he was too weak to repair cars and had no medical insurance. Skie said he negotiated with a radiologist to do MRIs at a price that Walker could afford, which is how he discovered the Chiari malformation.
Local neurosurgeons can perform the surgery, but it would cost Walker at least $150,000, Skie said.
Walker said he learned of the NIH study while researching his condition online. Skie was a fellow at the institute during the 1980s and knew of its research capabilities. Skie sent in Walker’s records, including the MRI scans.
“This is a person who inhibited himself because he couldn’t pay the bills, but we found resources available for him to get a diagnosis,” he said. “We then got him into our only national research hospital. I am thrilled with that. This surgery will not only save his life, but it will prevent him from having paralysis of his upper-left extremities.”
Walker said things have gradually worsened. At first, he had severe headaches if he coughed or sneezed, and his condition continued to deteriorate.
He is also concerned that his daughter, Nikki Walker, could have the disease, as she has some of the symptoms.
Because of his weakness, Walker can no longer repair cars at his garage, East Renfro Automotive Center, and his daughter is working without pay to keep the business going. Because of his illness, Walker also lost his home and is living in a run-down trailer that he said was destroyed by renters.
Walker, who will be in Maryland for about three weeks to recuperate, said he is grateful that he was chosen for the study.
Tony Walker, who will stay at the NIH with his brother, said he has no Chiari malformation symptoms. Studies show that 10 percent of people diagnosed with the malformation also have a relative with the disease.
Tony said he worries about his brother’s well-being and hopes that the surgery will bring relief. Learning about the surgery “scared the hell out of me, but it scared the hell out of me for him not to have it.
“It’s a risky surgery, but Clyde has no quality of life without it.”
Elizabeth Campbell, 817-390-7696
Give yourself a better chance against Chiari with good information and a properly educated diagnosis from your physician. The medical profession is not generally well informed about this disease, but Chiari is the REAL DEAL!!! I am not a doctor, just someone who lost a beautiful, loving daughter to Chiari. Shannon would be delighted if her story could help another Chiari patient in their struggle with this terrible disease. Shannon was also an organ donor. She gave the gift of life to those suffering other diseases. Help us keep her memory alive by working together to Conquer Chiari!!