View clinical trials related to Arnold Chiari Deformity.
Filter by:Background: - Syringomyelia is a disorder in which a cyst (syrinx) forms within the spinal cord and causes spinal cord injury, with symptoms worsening over many years, including paralysis, loss of sensation, and chronic pain. Researchers are interested in obtaining more knowledge about how a syrinx forms in order to develop safer and more effective treatments for syringomyelia and related conditions. - The goal of surgical treatment of syringomyelia is to eliminate the syrinx and prevent further spinal cord injury. In most patients, surgery results in the syrinx becoming smaller, but the effect of surgery on a patient s muscle strength, pain level, and overall function has not been studied over time. In addition, some individuals with syringomyelia or related conditions are not considered to be good candidates for surgery, and more information is needed about potential alternative treatments for these individuals. - By recording more than 5 years of symptoms, muscle strength, general level of functioning, and magnetic resonance imaging (MRI) scan findings from individuals who receive standard treatment for syringomyelia, researchers can obtain more information about factors that influence its development, progression, and relief of symptoms. Objectives: - To conduct a 5-year natural history study of individuals with syringomyelia and related conditions. Eligibility: - Individuals at least 18 years of age who have syringomyelia or related conditions (including pre-syringomyelia or Chiari I malformation without syringomyelia). Design: - This study requires 7 outpatient visits to the National Institutes of Health Clinical Center: an initial visit; a visit 3 months later; and visits 1, 2, 3, 4, and 5 years after the initial visit. An additional 10 days of inpatient treatment and testing will be required if surgery is needed during the study. - The following tests will be performed during this study: - Medical history and physical examination, which may also determine eligibility for surgery - Detailed neurological history and examination - Blood and urine samples - MRI scans: Participants will have 2 scans at the initial evaluation, 2 scans at the 3-month visit, and 1 scan every year for the following 5 years. - Additional neurological and imaging tests if needed, including a lumbar puncture to collect spinal fluid, a myelogram (imaging study) of the spinal fluid, and a computed tomography scan of the skull and spine. - Participants who are surgical candidates will have additional tests along with the surgery, including diagnostic studies (electrocardiogram and chest X-ray) before surgery and an MRI scan 1 week after surgery.
The brain and spinal cord are surrounded by fluid called cerebrospinal fluid (CSF). The CSF flows through channels in the brain and around the spinal cord. Occasionally, people are born with malformations of these channels. Syringomyelia is a pocket within the CSF channels that results from abnormal CSF flow. Syringomyelia is associated with problems in the nervous system. Patients with syringomyelia may be unable to detect sensations of pain and heat. If the condition is not treated it can worsen. Treatment of this condition is surgical. It requires that the flow of CSF is returns to normal. There are many different treatment options, but no one procedure has been shown to be significantly better than any other. In this study, researchers would like to learn more about how the CSF pressure and flow contribute to the progression of syringomyelia. Ultrasounds and magnetic resonance imaging (MRI) will be used to evaluate the anatomy of the brain. Researchers hope that information gathered about anatomy and measures of CSF pressure and flow can be used later to develop an optimal surgical treatment for syringomyelia.