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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| syringomyelia group |
| ||
| Other neurodegenerative diseases | such as hydrocephalus |
| |
| Normal group |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| high throughput sequencing and electromyography | Diagnostic Test | Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics |
|
| Measure | Description | Time Frame |
|---|---|---|
| Change of the spinal cord function | American Spinal Injury Association(ASIA) Score for evaluating the spinal cord function | 1 day before operation and 3 days, 3 months, 12 months postoperation |
| Measure | Description | Time Frame |
|---|---|---|
| molecular profiling results | Change From Baseline in molecular at postoperation | 1 day before operation and 3 days, 3 months postoperation |
| Electrophysiology results | included：electromyography and evoked potential; Change From Baseline in Electrophysiology at postoperation |
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Inclusion Criteria:
Exclusion Criteria:
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patients who was diagnosed as syringomyelia including: Chiari malformation、Basilar Impression、subarachnoid obstruction
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Fengzeng Jian, M.D. | Contact | +8613552067268 | jianfengzeng@xwh.ccmu.edu.cn | |
| chenghua yuan | Contact | +8617777784396 | yuanchenghua2@163.com |
| Name | Affiliation | Role |
|---|---|---|
| Fengzeng Jian, M.D. | Xuanwu Hospital, Beijing | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Xuanwu Hospital | Recruiting | Beijing | Beijing Municipality | 100032 | China |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 33313928 | Background | 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. | |
| 33035386 |
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| ID | Term |
|---|---|
| D013595 | Syringomyelia |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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| ID | Term |
|---|---|
| D004576 | Electromyography |
| ID | Term |
|---|---|
| D004568 | Electrodiagnosis |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D009213 | Myography |
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Blood, cerebral spinal fluid, saliva, urine, biopsy and autopsy
| 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 | 3 days, 3 months postoperation |
| Chicago Chiari outcome scale (CCOS) | Pain symptoms,Nonpain symptoms,Functionality,Complications, 4-16, higher scores mean a better outcome. | 3 days, 3 months, 12 months postoperation |
| Visual Analog Scale (VAS) | degree of the pain, 1-10, higher scores mean a worse outcome | 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 | 1 day before operation and 3 days, 3 months, 12 months postoperation |
| 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 | 1 day before operation and 3 days, 3 months, 12 months postoperation |
| Incidence of perioperative complications | 1 week |
| Background |
| Nakajima M, Rauramaa T, Makinen PM, Hiltunen M, Herukka SK, Kokki M, Musialowicz T, Jyrkkanen HK, Danner N, Junkkari A, Koivisto AM, Jaaskelainen 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. |
| 22569058 | Background | 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. |
| 18518671 | Background | 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. |
| 33667397 | Background | 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. No abstract available. |
| 33544223 | Background | 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. |