Clinical Outcome in Patients With Syringomyelia（COPSM）
Study Details
Study Description
Brief Summary
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.
| Condition or Disease | Intervention/Treatment | Phase |
|---|---|---|
|
Detailed Description
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 Design
Arms and Interventions
| Arm | Intervention/Treatment |
|---|---|
| syringomyelia group
|
Diagnostic Test: high throughput sequencing and electromyography
Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics
|
| Other neurodegenerative diseases such as hydrocephalus |
Diagnostic Test: high throughput sequencing and electromyography
Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics
|
| Normal group
|
Diagnostic Test: high throughput sequencing and electromyography
Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics
|
Outcome Measures
Primary Outcome Measures
- Change of the spinal cord function [1 day before operation and 3 days, 3 months, 12 months postoperation]
American Spinal Injury Association(ASIA) Score for evaluating the spinal cord function
Secondary Outcome Measures
- molecular profiling results [1 day before operation and 3 days, 3 months postoperation]
Change From Baseline in molecular at postoperation
- Electrophysiology results [1 day before operation and 3 days, 3 months postoperation]
included：electromyography and evoked potential; Change From Baseline in Electrophysiology at postoperation
- the rates of syrinx reduction [3 days, 3 months postoperation]
defined as a reduction of the syrinx diameter or length in MRI
- Chicago Chiari outcome scale (CCOS) [3 days, 3 months, 12 months postoperation]
Pain symptoms,Nonpain symptoms,Functionality,Complications, 4-16, higher scores mean a better outcome.
- Visual Analog Scale (VAS) [1 day before operation and 3 days, 3 months, 12 months postoperation]
degree of the pain, 1-10, higher scores mean a worse outcome
- Klekamp and Sammi syringomyelia scale [1 day before operation and 3 days, 3 months, 12 months postoperation]
for evaluating the spinal cord function, higher scores mean a better outcome
- modified Japanese Orthopaedic Association Scores (mJOA) [1 day before operation and 3 days, 3 months, 12 months postoperation]
Motor function， sensory， bladder function；for evaluating the spinal cord function；0-17， higher scores mean a better outcome
- Incidence of perioperative complications [1 week]
Eligibility Criteria
Criteria
Inclusion Criteria:
-
patients who was diagnosed as syringomyelia including: Chiari malformation， Basilar Impression， subarachnoid obstruction， patient not received surgical or interventional treatment before， patient willing and able to participate in the registry，
-
Hydrocephalus or other neurodegenerative disease and normal subjects.
Exclusion Criteria:
-
patient received surgical treatment or interventional treatment before
-
patient is pregnant
-
patient unable to complete follow-up
-
patient with other spinal lesions
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other nervous system diseases
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | Xuanwu Hospital | Beijing | Beijing | China | 100032 |
Sponsors and Collaborators
- Xuanwu Hospital, Beijing
Investigators
- Study Director: Fengzeng Jian, M.D., Xuanwu Hospital, Beijing
Study Documents (Full-Text)
None provided.More Information
Publications
- CreveCoeur TS, Yahanda AT, Maher CO, Johnson GW, Ackerman LL, Adelson PD, Ahmed R, Albert GW, Aldana PR, Alden TD, Anderson RCE, Baird L, Bauer DF, Bierbrauer KS, Brockmeyer DL, Chern JJ, Couture DE, Daniels DJ, Dauser RC, Durham SR, Ellenbogen RG, Eskandari R, Fuchs HE, George TM, Grant GA, Graupman PC, Greene S, Greenfield JP, Gross NL, Guillaume DJ, Haller G, Hankinson TC, Heuer GG, Iantosca M, Iskandar BJ, Jackson EM, Jea AH, Johnston JM, Keating RF, Kelly MP, Khan N, Krieger MD, Leonard JR, Mangano FT, Mapstone TB, McComb JG, Menezes AH, Muhlbauer M, Oakes WJ, Olavarria G, O'Neill BR, Park TS, Ragheb J, Selden NR, Shah MN, Shannon C, Shimony JS, Smith J, Smyth MD, Stone SSD, Strahle JM, Tamber MS, Torner JC, Tuite GF, Wait SD, Wellons JC, Whitehead WE, Limbrick DD. Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium. Neurosurgery. 2021 Jan 13;88(2):332-341. doi: 10.1093/neuros/nyaa460.
- Guan J, Yuan C, Zhang C, Ma L, Yao Q, Cheng L, Liu Z, Wang K, Duan W, Wang X, Wang Z, Wu H, Chen Z, Jian F. A novel classification and its clinical significance in Chiari I malformation with syringomyelia based on high-resolution MRI. Eur Spine J. 2021 Jun;30(6):1623-1634. doi: 10.1007/s00586-021-06746-y. Epub 2021 Feb 5. Erratum in: Eur Spine J. 2021 Apr 10;:.
- Klekamp J. Surgical treatment of Chiari I malformation--analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. Neurosurgery. 2012 Aug;71(2):365-80; discussion 380. doi: 10.1227/NEU.0b013e31825c3426.
- Nakajima M, Rauramaa T, Mäkinen PM, Hiltunen M, Herukka SK, Kokki M, Musialowicz T, Jyrkkänen HK, Danner N, Junkkari A, Koivisto AM, Jääskeläinen JE, Miyajima M, Ogino I, Furuta A, Akiba C, Kawamura K, Kamohara C, Sugano H, Tange Y, Karagiozov K, Leinonen V, Arai H. Protein tyrosine phosphatase receptor type Q in cerebrospinal fluid reflects ependymal cell dysfunction and is a potential biomarker for adult chronic hydrocephalus. Eur J Neurol. 2021 Feb;28(2):389-400. doi: 10.1111/ene.14575. Epub 2020 Nov 1.
- Roser F, Ebner FH, Liebsch M, Dietz K, Tatagiba M. A new concept in the electrophysiological evaluation of syringomyelia. J Neurosurg Spine. 2008 Jun;8(6):517-23. doi: 10.3171/SPI/2008/8/6/517.
- Sadler B, Wilborn J, Antunes L, Kuensting T, Hale AT, Gannon SR, McCall K, Cruchaga C, Harms M, Voisin N, Reymond A, Cappuccio G, Brunetti-Pierri N, Tartaglia M, Niceta M, Leoni C, Zampino G, Ashley-Koch A, Urbizu A, Garrett ME, Soldano K, Macaya A, Conrad D, Strahle J, Dobbs MB, Turner TN, Shannon CN, Brockmeyer D, Limbrick DD, Gurnett CA, Haller G. Rare and de novo coding variants in chromodomain genes in Chiari I malformation. Am J Hum Genet. 2021 Mar 4;108(3):530-531. doi: 10.1016/j.ajhg.2021.01.014.
- XWCOPSM