Syringomyelia/Hydromyelia
Conditions
Keywords
Syringomyelia, Biomarker，Electrophysiology
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.
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.
Interventions
DIAGNOSTIC_TESThigh throughput sequencing and electromyography
Whole Genome Sequencing, Whole Exome Sequencing, Transcriptomics, Proteomics, Metabolomics
Sponsors
Xuanwu Hospital, Beijing
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 80 Years
Healthy volunteers
Yes
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 * other nervous system diseases
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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
| Measure | Time frame | Description |
|---|---|---|
| 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. |
| molecular profiling results | 1 day before operation and 3 days, 3 months postoperation | Change From Baseline in molecular at postoperation |
| 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 | — |
| 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 |
Countries
China
Contacts
Primary ContactFengzeng Jian, M.D.
Backup Contactchenghua yuan
Outcome results
None listed