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 Chiari Malformation/Syringomyelia |
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(Pronounced sir-RING-o-my- EE-lia)
The University of Missouri-Columbia Chiari Clinic website explains that syringomyelia was first discovered by Antione Portal in 1803 after doing an autopsy on a late patient. Syringomyelia is a neurological disorder that occurs when a cyst forms in the spinal cord. The cyst is called a syrinx (pronounced see-rinx). Over time the syrinx damages the nerves inside the spinal cord and may expand and elongate.
The Chiari Institute’s (of Long Island Jewish Hospital) website cites that about 80% of syringomyelia cases were caused by a related disorder called Chiari malformation. Some of the other causes of syringomyelia are trauma, hemorrhage, infection and spinal cord tethering.
As described on the University of Missouri-Columbia Chiari Clinic website, there are many symptoms caused by syringomyelia. Some of these include pain, weakness, stiffness in the back, shoulders, arms, or legs, headaches, inability to feel hot and cold.
According to a study performed at Duke University by Dr. Marcy Speer in 2004, Chiari Type I Malformation With or Without Syringomyelia: Prevalence and Genetics, between 182,075 and 234,631 Americans have syringomyelia.
Syringomyelia is usually diagnosed through Magnetic Resonance Imaging, also known as an MRI. The MRI is able to take pictures of different parts of the body like the brain, spine and spinal cord. The MRI helps doctors find abnormalities and diagnose many disorders such as syringomyelia.
To treat syringomyelia, oftentimes surgery is recommended to fix the cause of the syrinx. For instance, a brain decompression may be recommended if the patient has Chiari malformation. By correcting the Chiari, doctors hope that the syringomyelia will be corrected as well.
If the cause of the syringomyelia cannot be corrected or if the Chiari surgery did not correct the syringomyelia, spinal cord surgery may be recommended. One possible surgical method is to remove one or more laminas (this is called a laminectomy) in the area of the syrinx, to create more space around the spinal cord (subarachnoid space). Another surgical route is to put a tube in the syrinx called a shunt. However this method carries significantly more risks. Due to this, strong communication between the physician and patient as well as research performed by the patient is imperative. Lastly, some syringomyelia patients have few or no symptoms. In this case, physicians often choose not to operate. A physician may also choose not to operate if the patient’s symptoms are not progressing.
The University of Missouri-Columbia Chiari Clinic website explains that Chiari I malformation was discovered by Dr. Chiari in 1891. Normally the brain is in a cavity inside the skull. The brainstem normally sits in a cone-shaped shaft, called the foramen magnum, right above the spinal cord. However in the case of Chiari I malformation, the back of the skull, called the posterior fossa, is not formed properly. When this happens there is not enough room in the cavity for the brain to exist in the skull properly. This forces tonsils on the cerebellum to elongate into the foramen magnum. This elongation blocks the flow of the fluid, called cerebral spinal fluid that flows around the brain and spinal cord. Chiari I malformation is generally diagnosed through an MRI (magnetic resonance imaging).
As described by the Chiari Institute’s website, Chiari I malformation is generally a congenital disorder, however acquired cases do exist. According to the University of Missouri-Columbia Chiari Clinic website, while the actual cause of the disorder is unknown it is believed that the abnormality forms when the spinal cord and brain stem are forming in the embryo.
Many times patients with the disorder do not experience symptoms. However when symptoms are present, they usually occur during adolescence or early adulthood and less often, during childhood.
The University of Missouri-Columbia Chiari Clinic website lists the following as symptoms of Chiari I Malformation: headaches, dysphagia (difficulty swallowing), pain, hoarseness, numbness, visual disturbance, ataxia (gait disturbance), syncope (episodes of passing out or loss of consciousness), nausea, vomiting, dizziness, disequilibrium (problems with balance), nosebleeds, fast or slow heart beat, high or low blood pressure, ringing in the ears, hearing loss, depression, shortness of breath and fatigue. Although this is a comprehensive list, more symptoms can occur.
Chiari I malformation is generally treated through surgery. The goal of the surgery is to decompress the posterior fossa and the foramen magnum areas. This is done by removing a small section of the skull in the area of the posterior fossa. Other procedures may be performed during surgery but the method depends on the surgeon and the patient.
According to Dr. Steven Richeimer on the Spine Universe website, neuropathic pain occurs when there is a malfunction in the part of the nervous system that tells the brain that tissue is injured. While there may be no injury to the tissue where the pain exists, the nervous system tells the brain that there is damage, causing pain. This is the case because the pain is caused by the damage or pathological change in the central or peripheral nervous system.
Neuropathic pain is generally described by patients as being burning, electric, tingling and shooting pain. The pain can be constant or come in sudden attack.
Dr. Steven Richeimer, on the University of South California Pain Center website, lists the following examples of neuropathic pain: ÒExamples include post herpetic (or post-shingles) neuralgia, reflex sympathetic dystrophy / causalgia (nerve trauma), components of cancer pain, phantom limb pain, entrapment neuropathy (e.g., carpal tunnel syndrome), and peripheral neuropathy (widespread nerve damage). Among the many causes of peripheral neuropathy, diabetes is the most common, but the condition can also be caused by chronic alcohol use, exposure to other toxins (including many chemotherapies), vitamin deficiencies, and a large variety of other medical conditions--it is not unusual for the cause of the condition to go undiagnosed.
As described by Dr. Richeimer on the Spine Universe website, medications used to treat neuropathic pain are as follows, tricyclic antidepressants, anticonvulsants and topical therapy that contain substance P depletors, autonomic drugs and NMDA receptor antagonists. Invasive procedures can also be performed like implanting a morphine pump or nerve blocks. Dr. Richeimer highlights the importance of having a multidisciplinary approach to treating neuropathic pain. Aside from medication and procedures it may be pertinent for the patient to make use of physical modalities, an example being physical rehabilitation. It also may be important to make use of psychological modalities like behavior modification or relaxation training.
When there is a traumatic blow to the spine, it can cause to the vertebrae to fracture or dislocate. A spinal cord injury occurs when the dislocated vertebrae, bone fragments, disc material, or ligaments bruise or damage the spinal cord. Most spinal cord injuries are caused by bruising or damage to the disc or vertebrae material, as opposed to being completely severed.
Vertebrae are the bones that surround the spinal cord. Each bone is shaped like a ring and is named in relation to their location. The cervical vertebrae are in the neck, the thoracic are in the back, the lumbar and sacral vertebrae are in the lower back.
As described by the Spinal Cord Injury Resource Center website, the level on the spinal cord where the injury occurs can determine how the body will be affected by the injury. Injuries that occur in the cervical spine tend to cause quadriplegia (loss of function in the arms and legs). Injuries that occur at the thoracic spine and lower usually cause paraplegia (the arms are not affected).
The NINDS website points out that there are two types of spinal cord injury; complete and incomplete. An incomplete injury occurs when the spinal cord’s ability to send information to the brain is still partially intact. This means that people with this type of spinal cord injury still have some sensory and motor function below the point of injury. A complete injury occurs when the patient retains no sensory or motor function.
According to the NINDS website, people with spinal cord injuries often have complications. For instance, they can experience chronic pain and bladder and bowl problems. There is also higher chance for spinal cord injury patients to experience respiratory and heart problems than the general public.
The SCI-Info-Pages website list the following statistics on spinal cord injury:
| Number of Americans with Spinal Cord Injuries |
250,000 |
| 52% |
| 47% |
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| Demographics |
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| 82% |
| 18% |
| 63% |
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| Costs |
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| $428,000 (age of injury, 25) |
| $1,350,000 (age of injury, 25) |
| 52% |
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