A Multi-Disciplinary Approach to the Study of Type I Chiari Malformation


Winter Conference on Brain Research 2017


Type I Chiari malformation (CM) is a structural defect where part of the cerebellum is located below the foramen magnum. Characterized by NIH, individuals with CM may complain of neck pain, balance problems, muscle weakness, numbness in the arms or legs, dizziness or headache made worse by coughing or straining. Symptoms may change for some individuals, depending on the buildup of CSF and resulting pressure on the tissues and nerves. Thus, a basic understanding of the pathogenesis of CM has been elusive. Our group at the Conquer Chiari Research Center has been working towards a better understanding of CM through a multidisciplinary approach. The four areas of investigation are imaging, cognitive processes, psycho-social function, and assessments of the inflammatory response. We have developed projects to examine these four areas by creating a web-accessed, secure database called Chiari1000. Chiari1000 collects health-related, neuropsychological, and MR scans voluntarily provided by Chiari patients. Cognitive processes and psycho-social function are assessed through a series of web surveys. To assess the inflammatory response, blood assays are obtained through local Quest Diagnostics clinics, and saliva samples are provided through the mail. The Chiari1000 registry launched in the Fall 2015. Presently, 708 CMI participants have completed surveys (with 314 MR images). We hope to obtain 1000 CMI subjects with completed surveys and corresponding MRIs and the inflammatory response on 100 of these subjects. We have developed custom software for automated morphological measurements, data visualization, and analysis of these multidisciplinary results. This information may help to stratify CM patients which will help in the development of new hypotheses for this complex condition. The overall goal is to identify novel objective measures that are predictive of symptom severity to help in the diagnosis of CM and the evaluation of various treatment options.


Francis Loth, James R. Houston, Dipankar Biswas, Soroush Pahlavian, Maggie Eppelheimer, Dorothy Loth, Richard Labuda, Philip A. Allen