The aim of this study is to determine the clinical spectrum and natural progression of Syringomyelia (SM) and related disorders in a prospective single center study, identify digital, imaging and molecular biomarkers that can assist in diagnosis and therapy development and study the etiology and molecular mechanisms of these diseases.
Syringomyelia is a chronic central spinal cord injury, which is characterized by dilation of the central canal of the spinal cord. At present, the treatment of syringomyelia is mainly through surgical decompression to restore the disturbance of cerebrospinal fluid circulation. Due to the heterogeneity of the etiology of syringomyelia, almost all published studies on the clinical outcome and prognostic factors of syringomyelia are relatively limited, and most of them are retrospective. It is not clear which is the most reliable predictor of clinical outcome. Therefore, the researchers conducted this prospective cohort study to identify the occurrence, development and outcome of syringomyelia and determine the main prognostic factors through clinical scales, biomarkers and electrophysiology. At study visits a standardized clinical examination will be performed including application of clinical rating scales. At all study visits, patients will be asked to donate biosamples; biomaterial collection is optional and participants can elect to participate in sampling of blood, urine, CSF, and/or a muscle biopsy. Optionally, additional examinations may be performed including imaging, neurophysiological examination, analysis of patient or observer reported outcomes and analysis to characterize molecular biomarkers.
Study Type
OBSERVATIONAL
Enrollment
200
Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics
Xuanwu Hospital
Beijing, Beijing Municipality, China
RECRUITINGChange of the spinal cord function
American Spinal Injury Association(ASIA) Score for evaluating the spinal cord function
Time frame: 1 day before operation and 3 days, 3 months, 12 months postoperation
molecular profiling results
Change From Baseline in molecular at postoperation
Time frame: 1 day before operation and 3 days, 3 months postoperation
Electrophysiology results
included:electromyography and evoked potential; Change From Baseline in Electrophysiology at postoperation
Time frame: 1 day before operation and 3 days, 3 months postoperation
the rates of syrinx reduction
defined as a reduction of the syrinx diameter or length in MRI
Time frame: 3 days, 3 months postoperation
Chicago Chiari outcome scale (CCOS)
Pain symptoms,Nonpain symptoms,Functionality,Complications, 4-16, higher scores mean a better outcome.
Time frame: 3 days, 3 months, 12 months postoperation
Visual Analog Scale (VAS)
degree of the pain, 1-10, higher scores mean a worse outcome
Time frame: 1 day before operation and 3 days, 3 months, 12 months postoperation
Klekamp and Sammi syringomyelia scale
for evaluating the spinal cord function, higher scores mean a better outcome
Time frame: 1 day before operation and 3 days, 3 months, 12 months postoperation
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modified Japanese Orthopaedic Association Scores (mJOA)
Motor function, sensory, bladder function;for evaluating the spinal cord function;0-17, higher scores mean a better outcome
Time frame: 1 day before operation and 3 days, 3 months, 12 months postoperation
Incidence of perioperative complications
Time frame: 1 week