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1.
Clin Neurol Neurosurg ; 242: 108315, 2024 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-38749356

RESUMEN

OBJECTIVE: To develop and validate a computed tomography (CT)-based scoring system for evaluating the risk of dural defects (DDs) in anterior surgery for cervical ossification of the posterior longitudinal ligament (OPLL). METHODS: We retrospectively analyzed CT imaging features of 114 OPLL patients in our institute who received anterior decompression surgery. Intraoperative DDs were found in 16 patients. A multivariable logistic regression was used to evaluate the predictors. According to the odd ratio of the included risk factors, we developed a CT scoring system for evaluating the risk of DDs in anterior OPLL surgery. The system was further validated in an independent group of 39 OPLL patients. RESULTS: We developed a CT scoring system as follows: hook sign (2 points), K-line (-) (1 point) and broad base (1 point). Thus, the system comprised 4 total points, and patients were at high risks of dural defects when the score ≥3 points. The operating characteristics of a score ≥3 for predicting DDs in the validation group were: sensitivity of 0.83, specificity of 0.94, LR positive of 13.75, LR negative of 0.18 and AUC of 0.886. The discriminatory ability of the proposed score could be demonstrated in the validation cohort. CONCLUSIONS: The relatively simple and easy-to-use scoring system we propose integrates the 3 most reliable spinal CT findings observed in patients with OPLL and a DD. The likelihood to identify the underlying risks of spinal CSF leaks may be useful to triage patients who may benefit from indirect decompression techniques.

2.
Tissue Eng Regen Med ; 21(4): 625-639, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38578425

RESUMEN

BACKGROUND: Syringomyelia is a progressive chronic disease that leads to nerve pain, sensory dissociation, and dyskinesia. Symptoms often do not improve after surgery. Stem cells have been widely explored for the treatment of nervous system diseases due to their immunoregulatory and neural replacement abilities. METHODS: In this study, we used a rat model of syringomyelia characterized by focal dilatation of the central canal to explore an effective transplantation scheme and evaluate the effect of mesenchymal stem cells and induced neural stem cells for the treatment of syringomyelia. RESULTS: The results showed that cell transplantation could not only promote syrinx shrinkage but also stimulate the proliferation of ependymal cells, and the effect of this result was related to the transplantation location. These reactions appeared only when the cells were transplanted into the cavity. Additionally, we discovered that cell transplantation transformed activated microglia into the M2 phenotype. IGF1-expressing M2 microglia may play a significant role in the repair of nerve pain. CONCLUSION: Cell transplantation can promote cavity shrinkage and regulate the local inflammatory environment. Moreover, the proliferation of ependymal cells may indicate the activation of endogenous stem cells, which is important for the regeneration and repair of spinal cord injury.


Asunto(s)
Trasplante de Células Madre Mesenquimatosas , Células Madre Mesenquimatosas , Células-Madre Neurales , Ratas Sprague-Dawley , Siringomielia , Animales , Células-Madre Neurales/metabolismo , Células-Madre Neurales/citología , Trasplante de Células Madre Mesenquimatosas/métodos , Células Madre Mesenquimatosas/metabolismo , Células Madre Mesenquimatosas/citología , Siringomielia/terapia , Ratas , Proliferación Celular , Epéndimo , Masculino , Microglía/metabolismo , Modelos Animales de Enfermedad
3.
Neurospine ; 21(1): 212-222, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38317552

RESUMEN

OBJECTIVE: Surgical procedures for patients with posttraumatic syringomyelia (PTS) remain controversial. Until now, there have been no effective quantitative evaluation methods to assist in selecting appropriate surgical plans before surgery. METHODS: We consecutively enrolled PTS patients (arachnoid lysis group, n = 42; shunting group, n = 14) from 2003 to 2023. Additionally, 19 intrathecal anesthesia patients were included in the control group. All patients with PTS underwent physical and neurological examinations and spinal magnetic resonance imaging preoperatively, 3-12 months postoperatively and during the last follow-up. Preoperative lumbar puncture was performed and blood-spinal cord barrier disruption was detected by quotient of albumin (Qalb, cerebrospinal fluid/serum). RESULTS: The ages (p = 0.324) and sex (p = 0.065) of the PTS and control groups did not differ significantly. There were also no significant differences in age (p = 0.216), routine blood data and prognosis (p = 0.399) between the arachnoid lysis and shunting groups. But the QAlb level of PTS patients was significantly higher than that of the control group (p < 0.001), and the shunting group had a significantly higher QAlb (p < 0.001) than the arachnoid lysis group. A high preoperative QAlb (odds ratio, 1.091; 95% confidence interval, 1.004-1.187; p = 0.041) was identified as the predictive factor for the shunting procedure, with the receiver operating characteristic curve showing 100% specificity and 80.95% sensitivity for patients with a QAlb > 12.67. CONCLUSION: Preoperative QAlb is a significant predictive factor for the types of surgery. For PTS patients with a QAlb > 12.67, shunting represents the final recourse, necessitating the exploration and development of novel treatments for these patients.

4.
Neurospine ; 2024 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-38317543

RESUMEN

Objective: To investigate the developmental defects caused by knockdown of best1 gene in zebrafish as a model for a subtype of craniovertebral junction (CVJ) malformation. Methods: Two antisense morpholinos were designed targeting zebrafish best1 to block translation (ATG-MO) or to disrupt splicing (I3E4-MO). Morpholinos were microinjected into fertilized one-cell embryos. Efficacy of splicing morpholino was confirmed by RT-PCR. Phenotypes were analyzed and quantified by microscopy at multiple developmental stages. Neuronal outgrowth was assessed in transgenic zebrafish expressing GFP in neurons. Skeletal ossification was visualized by calcein staining. Results: Knockdown of best1 resulted in zebrafish embryos with shorter body length, curved axis, low survival rate, microcephaly, reduced eye size, smaller head and brain, impaired neuronal outgrowth, and reduced ossification of craniofacial and vertebral bone. Conclusion: Best1 gene plays critical roles in ophthalmologic, neurological and skeletal development in zebrafish. A patient with a premature stop codon in BEST1 gene exhibited simillar phenotypes, implying a subtype of CVJ malformation.

5.
Spine (Phila Pa 1976) ; 49(6): E62-E71, 2024 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-38014747

RESUMEN

STUDY DESIGN: Cohort study. OBJECTIVE: The aim of this study was to explore the association between blood-spinal cord barrier (BSCB) markers and other factors associated with an unfavorable outcome among patients with post-traumatic syringomyelia (PTS) who achieved successful intradural adhesion lysis (IAL). SUMMARY OF BACKGROUND DATA: Only approximately half of PTS patients receiving IAL have a favorable outcome. PATIENTS AND METHODS: Forty-six consecutive patients with PTS and 19 controls (CTRL) were enrolled. All PTS patients underwent physical and neurological examinations and spinal magnetic resonance imaging before and 3 to 12 months after IAL. All patients underwent myelography before surgery. BSCB disruption was detected by increased intrathecal and serum concentrations of albumin, immunoglobulin (Ig)G, IgA, and IgM. A multivariable analysis was performed with a logistic regression model to identify factors associated with unfavorable outcomes. Receiver operating characteristic curves were calculated to investigate the diagnostic value of biomarkers. RESULTS: The ages and general health of the PTS and CTRL groups did not differ significantly. QAlb, IGAQ, IGGQ, and IGMQ was significantly higher in PTS patients than in controls ( P =<0.001). The degree of intradural adhesion was significantly higher in the unfavorable outcome group than in the favorable outcome group ( P <0.0001). QAlb, immunoglobulin (Ig)AQ, IGGQ, and IGMQ was significantly correlated with clinical status ( R =-0.38, P <0.01; R =-0.47, P =0.03; R =-0.56, P =0.01; R =-0.43, P =0.05, respectively). Higher QAlb before surgery (odds ratio=2.66; 95% CI: 1.134-6.248) was significantly associated with an unfavorable outcome. The receiver operating characteristic curve analysis demonstrated a cutoff for QAlb higher than 10.62 with a specificity of 100% and sensitivity of 96.3%. CONCLUSION: This study is the first to detect increased permeability and BSCB disruption in PTS patients. QAlb>10.62 was significantly associated with unfavorable clinical outcomes following intradural decompression. LEVEL OF EVIDENCE: Level III-prognostic.


Asunto(s)
Traumatismos de la Médula Espinal , Siringomielia , Humanos , Siringomielia/diagnóstico por imagen , Siringomielia/etiología , Siringomielia/cirugía , Estudios de Cohortes , Traumatismos de la Médula Espinal/complicaciones , Pronóstico , Inmunoglobulinas
7.
J Neuroimmunol ; 383: 578191, 2023 10 15.
Artículo en Inglés | MEDLINE | ID: mdl-37660537

RESUMEN

Hypertrophic pachymeningitis (HP) is a relatively rare disease of the central nervous system characterized by local or diffuse fibrous thickening of the dura mater. At present, there is still insufficient research on the pathogenesis and treatment strategies of this disease. We reported a continuous case series of seven patients with idiopathic HP (IHP), and also details one case of immunoglobulin G4-related HP requiring surgical intervention. Early diagnosis and appropriate surgical intervention for IHP could prevent the progression of permanent neurological damage and spinal cord paraplegia.


Asunto(s)
Meningitis , Humanos , Duramadre/diagnóstico por imagen , Duramadre/cirugía , Duramadre/patología , Hipertrofia , Meningitis/complicaciones , Meningitis/diagnóstico por imagen , Médula Espinal/patología
8.
Cell Death Differ ; 30(9): 2053-2065, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37553426

RESUMEN

Embryonic neurogenesis is tightly regulated by multiple factors to ensure the precise development of the cortex. Deficiency in neurogenesis may result in behavioral abnormalities. Pd1 is a well-known inhibitory immune molecule, but its function in brain development remains unknown. Here, we find brain specific deletion of Pd1 results in abnormal cortical neurogenesis, including enhanced proliferation of neural progenitors and reduced neuronal differentiation. In addition, neurons in Pd1 knockout mice exhibit abnormal morphology, both the total length and the number of primary dendrites were reduced. Moreover, Pd1cKO mice exhibit depressive-like behaviors, including immobility, despair, and anhedonia. Mechanistically, Pd1 regulates embryonic neurogenesis by targeting Pax3 through the ß-catenin signaling pathway. The constitutive expression of Pax3 partly rescues the deficiency of neurogenesis in the Pd1 deleted embryonic brain. Besides, the administration of ß-catenin inhibitor, XAV939, not only rescues abnormal brain development but also ameliorates depressive-like behaviors in Pd1cKO mice. Simultaneously, Pd1 plays a similar role in human neural progenitor cells (hNPCs) proliferation and differentiation. Taken together, our findings reveal the critical role and regulatory mechanism of Pd1 in embryonic neurogenesis and behavioral modulation, which could contribute to understanding immune molecules in brain development.


Asunto(s)
Neuronas , beta Catenina , Animales , Humanos , Ratones , beta Catenina/metabolismo , Encéfalo/metabolismo , Ratones Noqueados , Neurogénesis , Neuronas/metabolismo , Factores de Transcripción/metabolismo
9.
Neurospine ; 20(2): 498-506, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37401068

RESUMEN

OBJECTIVE: The surgical management of basilar invagination without atlantoaxial dislocation (type B basilar invagination) remains controversial. Hence, we have reported the use of posterior intra-articular C1-2 facet distraction, fixation, and cantilever technique versus foramen magnum decompression in treating type B basilar invagination as well as the results and surgical indications for this procedure. METHODS: This was a single-center retrospective cohort study. Fifty-four patients who underwent intra-articular distraction, fixation, and cantilever reduction (experimental group) and foramen magnum decompression (control group) were enrolled in this study. Distance from odontoid tip to Chamberlain's line, clivus-canal angle, cervicomedullary angle, craniovertebral junction (CVJ) triangle area, width of subarachnoid space and syrinx were used for radiographic assessment. Japanese Orthopedic Association (JOA) scores and 12-item Short Form health survey (SF-12) scores were used for clinical assessment. RESULTS: All patients in the experimental group had a better reduction of basilar invagination and better relief of pressure on nerves. JOA scores and SF-12 scores also had better improvements in the experimental group postoperation. SF-12 score improvement was associated with preoperative CVJ triangle area (Pearson index, 0.515; p = 0.004), cutoff value of 2.00 cm2 indicating the surgical indication of our technique. No severe complications or infections occurred. CONCLUSION: Posterior intra-articular C1-2 facet distraction, fixation, and cantilever reduction technique is an effective treatment for type B basilar invagination. As various factors involved, other treatment strategies should also be investigated.

10.
Neurospine ; 20(2): 701-708, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37401089

RESUMEN

OBJECTIVE: Although cerebrospinal fluid (CSF)-based liquid biopsy was proved to be practical in molecular analysis of intracranial gliomas, liquid biopsy of primary intramedullary astrocytoma was rarely reported. Given the distinct genomic profiles between primary intramedullary glioma and intracranial astrocytoma, whether the feasibility of CSF-based molecular analysis of intracranial gliomas can be replicated in primary spinal cord astrocytoma needs to be investigated. The aim of this pilot study is to evaluate the feasibility of molecular analysis of primary intramedullary astrocytoma through sequencing CSF-derived circulating tumor DNA (ctDNA). METHODS: Two grade IV diffuse midline gliomas, 1 grade II, and 1 grade I astrocytoma were included. Intraoperative collection of peripheral blood and CSF samples was conducted, along with postoperative collection of matched tumor tissues. A panel covering the 1,021 most common driver genes of solid tumors was used for targeted DNA sequencing. RESULTS: CSF-derived ctDNA was detected in 3 CSF samples (2 grade IV diffuse midline gliomas and 1 grade I astrocytoma), 5 mutations were found in both tumor tissues and CSF samples, while 11 mutations and 20 mutations were detected exclusively in tumor tissues and CSF samples, respectively. Importantly, hotspot genetic alterations, including H3F3A K28M, TP53, and ATRX, were identified in CSF and the average mutant allele frequency was often higher in CSF than in tumor tissues. CONCLUSION: CSF-based liquid biopsy showed potential feasibility for molecular analysis of primary intramedullary astrocytoma through sequencing of ctDNA. This approach may assist in diagnosis and prognostic evaluation of this rare spinal cord tumor.

11.
iScience ; 26(6): 106850, 2023 Jun 16.
Artículo en Inglés | MEDLINE | ID: mdl-37275526

RESUMEN

Syringomyelia is a common clinical lesion associated with cerebrospinal fluid flow abnormalities. By a reversible model with chronic extradural compression to mimic human canalicular syringomyelia, we explored the spatiotemporal pathological alterations during syrinx development. The most dynamic alterations were observed in ependymal cells (EPCs), oligodendrocyte lineage, and microglia, as a response to neuroinflammation. Among different cell types, EPC subtypes experienced obvious dynamic alterations, which were accompanied by ultrastructural changes involving the ependymal cytoskeleton, cilia, and dynamic injury in parenchyma primarily around the central canal, corresponding to the single-cell transcripts. After effective decompression, the syrinx resolved with the recovery of pathological damage and overall neurological function, implying that for syringomyelia in the early stage, there was still endogenous repair potential coexisting with immune microenvironment imbalance. Ependymal remodeling and cilia restoration might be important for better resolution of syringomyelia and parenchymal injury recovery.

12.
World Neurosurg ; 178: e34-e41, 2023 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-37356485

RESUMEN

OBJECTIVE: Tracheal traction exercise (TTE) has been proposed as a preventative measure for laryngopharyngeal complications following anterior cervical discectomy and fusion. However, the exact effects of TTE remain controversial. Therefore, we conducted a systematic review and meta-analysis of randomized controlled trials to evaluate the efficacy of TTE. METHODS: We systematically searched PubMed, Web of Science, Embase, Cochrane, ClinicalTrials.gov, China National Knowledge Infrastructure, WANFANG DATA, VIP citation databases, and Chinese BioMedical Literature Database for randomized controlled trials (published between January 1, 2000, and January 23, 2023, without language restrictions) comparing the TTE group to the control group (non-TTE group). We assessed the risk of bias using Cochrane risk of bias assessment tool. Our primary end points were operation duration, the number of patients with postoperative dysphagia, and Visual Analog Scale (VAS) for laryngopharyngeal pain. We used a fixed-effects model to assess the pooled data. RESULTS: Of the 823 identified studies, 5 were eligible and included in our analysis (N = 542 participants). Compared with the control group, TTE reduced the incidence of postoperative dysphagia (relative risk = 0.41, 95% confidence interval [CI]: 0.28, 0.61, P < 0.05) and operation duration (weighted mean difference = -10.24, 95% CI: -14.48, -6.00, P < 0.05). However, no significant difference was observed in postoperative VAS between the 2 groups (weighted mean difference = -0.11, 95% CI: -0.23, 0.11, P = 0.08 > 0.05). CONCLUSIONS: TTE can effectively reduce operation duration and postoperative dysphagia. However, it does not result in a significant difference in postoperative VAS.


Asunto(s)
Trastornos de Deglución , Humanos , Trastornos de Deglución/etiología , Trastornos de Deglución/prevención & control , Tracción/efectos adversos , Ensayos Clínicos Controlados Aleatorios como Asunto , Discectomía/efectos adversos , Tráquea
13.
Cell Biosci ; 13(1): 98, 2023 May 29.
Artículo en Inglés | MEDLINE | ID: mdl-37248485

RESUMEN

BACKGROUND: Syringomyelia is a cerebrospinal fluid (CSF) disorder resulted in separation of pain and temperature, dilation of central canal and formation of syrinx in central canal. It is unclear about mechanisms of the dilation and syrinx formation. We aimed to investigate roles of ependymal cells lining central canal on the dilation, trying to reduce syrinx formation in central canal. METHODS: We employed 78 Sprague-Dawley (SD) rats totally with syringomyelia to detect the contribution of ependymal cells to the dilation of central canal. Immunofluorescence was used to examine the activation of ependymal cells in 54 syringomyelia rat models. BrdU was used to indicate the proliferation of ependymal cells through intraperitoneal administration in 6 syringomyelia rat models. 18 rats with syringomyelia were injected with SIS3, an inhibitor of TGFßR-Smad3, and rats injected with DMSO  were used as control. Among the 18 rats, 12 rats were used for observation of syrinx following SIS3 or DMSO administration by using magnetic resonance imaging (MRI) on day 14 and day 30 under syringomyelia without decompression. All the data were expressed as mean ± standard deviation (mean ± SD). Differences between groups were compared using the two-tailed Student's t-test or ANOVA. Differences were considered significant when *p < 0.05. RESULTS: Our study showed the dilation and protrusions of central canal on day 5 and enlargement from day 14 after syringomyelia induction in rats with activation of ependymal cells lining central canal. Moreover, the ependymal cells contributed to protrusion formation possibly through migration along with central canal. Furthermore, suppression of TGFßR-Smad3 which was crucial for migration reversed the size of syrnix in central canal without treatment of decompression, suggesting TGFßR-Smad3 signal might be key for dilation of central canal and formation of syrinx. CONCLUSIONS: The size of syrinx was decreased after SIS3 administration without decompression. Our study depicted the mechanisms of syrinx formation and suggested TGFßR-Smad3 signal might be key for dilation of central canal and formation of syrinx.

14.
Int J Surg ; 109(8): 2276-2285, 2023 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-37204435

RESUMEN

BACKGROUND: To develop a practical prediction model to predict the risk of deep surgical site infection (SSI) in patients receiving open posterior instrumented thoracolumbar surgery. METHODS: Data of 3419 patients in four hospitals from 1 January 2012 to 30 December 2021 were evaluated. The authors used clinical knowledge-driven, data-driven, and decision tree model to identify predictive variables of deep SSI. Forty-three candidate variables were collected, including 5 demographics, 29 preoperative, 5 intraoperative, and 4 postoperative variables. According to model performance and clinical practicability, the best model was chosen to develop a risk score. Internal validation was performed by using bootstrapping methods. RESULTS: After open posterior instrumented thoracolumbar surgery, 158 patients (4.6%) developed deep SSI. The clinical knowledge-driven model yielded 12 predictors of deep SSI, while the data-driven and decision tree model produced 11 and 6 predictors, respectively. A knowledge-driven model, which had the best C-statistics [0.81 (95% CI: 0.78-0.85)] and superior calibration, was chosen due to its favorable model performance and clinical practicality. Moreover, 12 variables were identified in the clinical knowledge-driven model, including age, BMI, diabetes, steroid use, albumin, duration of operation, blood loss, instrumented segments, powdered vancomycin administration, duration of drainage, postoperative cerebrospinal fluid leakage, and early postoperative activities. In bootstrap internal validation, the knowledge-driven model still showed optimal C-statistics (0.79, 95% CI: 0.75-0.83) and calibration. Based on these identified predictors, a risk score for deep SSI incidence was created: the A-DOUBLE-SSI (Age, D [Diabetes, Drainage], O [duration of Operation, vancOmycin], albUmin, B [BMI, Blood loss], cerebrospinal fluid Leakage, Early activities, Steroid use, and Segmental Instrumentation) risk score. Based on the A-DOUBLE-SSI score system, the incidence of deep SSI increased in a graded fashion from 1.06% (A-DOUBLE-SSIs score ≤8) to 40.6% (A-DOUBLE-SSIs score>15). CONCLUSIONS: The authors developed a novel and practical model, the A-DOUBLE-SSIs risk score, that integrated easily accessible demographics, preoperative, intraoperative, and postoperative variables and could be used to predict individual risk of deep SSI in patients receiving open posterior instrumented thoracolumbar surgery.


Asunto(s)
Infección de la Herida Quirúrgica , Vancomicina , Humanos , Infección de la Herida Quirúrgica/epidemiología , Infección de la Herida Quirúrgica/etiología , Estudios de Cohortes , Factores de Riesgo , Albúminas , Esteroides , Estudios Retrospectivos
15.
Oper Neurosurg (Hagerstown) ; 25(2): 125-135, 2023 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-37083634

RESUMEN

BACKGROUND: The correlation among syrinx resolution, occipitoaxial sagittal alignment, and surgical outcome in long-term follow-up seems to have not been clarified. OBJECTIVE: To further explore the relationship between the syrinx resolution and occipitoaxial realignment after posterior reduction and fixation in basilar invagination (BI)-atlantoaxial dislocation (AAD) patients with syringomyelia. METHODS: A continuous series of 32 patients with BI-AAD and syringomyelia who received direct posterior reduction met the inclusion criteria of this study. Their clinical and imaging data were analyzed retrospectively. Before surgery and at the last follow-up, we used the Japanese Orthopedic Association (JOA) score and the Neck Disability Index (NDI) to assess the neurological status, respectively. The Pearson correlation coefficient and multiple stepwise regression analysis were used to explore the relevant factors that may affect surgical outcomes. RESULTS: There were significant differences in atlantodental interval, clivus-axial angle, occiput-C2 angle (Oc-C2A), cervicomedullary angle (CMA), subarachnoid space (SAS) at the foramen magnum (FM), syrinx size, NDI, and JOA score after surgery compared with those before surgery. ΔCMA and the resolution rate of syrinx/cord as relevant factors were correlated with the recovery rate of JOA (R 2 = 0.578, P < .001) and NDI (R 2 = 0.369, P < .01). What's more, ΔSAS/FMD (SAS/FM diameter) and ΔOc-C2A were positively correlated with the resolution rate of syrinx/cord (R 2 = 0.643, P < .001). CONCLUSION: With medulla decompression and occipital-cervical sagittal realignment after posterior reduction and fusion for BI-AAD patients with syringomyelia, the structural remodeling of the craniovertebral junction and occipitoaxial realignment could contribute to syringomyelia resolution.


Asunto(s)
Articulación Atlantoaxoidea , Luxaciones Articulares , Platibasia , Siringomielia , Humanos , Siringomielia/diagnóstico por imagen , Siringomielia/cirugía , Estudios Retrospectivos , Articulación Atlantoaxoidea/diagnóstico por imagen , Articulación Atlantoaxoidea/cirugía , Luxaciones Articulares/diagnóstico por imagen , Luxaciones Articulares/cirugía , Platibasia/diagnóstico por imagen , Platibasia/cirugía , Resultado del Tratamiento
16.
J Pathol ; 260(3): 317-328, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37114614

RESUMEN

Primary spinal cord astrocytoma (SCA) is a rare disease. Knowledge about the molecular profiles of SCAs mostly comes from intracranial glioma; the pattern of genetic alterations of SCAs is not well understood. Herein, we describe genome-sequencing analyses of primary SCAs, aiming to characterize the mutational landscape of primary SCAs. We utilized whole exome sequencing (WES) to analyze somatic nucleotide variants (SNVs) and copy number variants (CNVs) among 51 primary SCAs. Driver genes were searched using four algorithms. GISTIC2 was used to detect significant CNVs. Additionally, recurrently mutated pathways were also summarized. A total of 12 driver genes were identified. Of those, H3F3A (47.1%), TP53 (29.4%), NF1 (19.6%), ATRX (17.6%), and PPM1D (17.6%) were the most frequently mutated genes. Furthermore, three novel driver genes seldom reported in glioma were identified: HNRNPC, SYNE1, and RBM10. Several germline mutations, including three variants (SLC16A8 rs2235573, LMF1 rs3751667, FAM20C rs774848096) that were associated with risk of brain glioma, were frequently observed in SCAs. Moreover, 12q14.1 (13.7%) encompassing the oncogene CDK4 was recurrently amplified and negatively affected patient prognosis. Besides frequently mutated RTK/RAS pathway and PI3K pathway, the cell cycle pathway controlling the phosphorylation of retinoblastoma protein (RB) was mutated in 39.2% of patients. Overall, a considerable degree of the somatic mutation landscape is shared between SCAs and brainstem glioma. Our work provides a key insight into the molecular profiling of primary SCAs, which might represent candidate drug targets and complement the molecular atlas of glioma. © 2023 The Pathological Society of Great Britain and Ireland.


Asunto(s)
Astrocitoma , Glioma , Humanos , Fosfatidilinositol 3-Quinasas , Mutación , Glioma/genética , Médula Espinal/patología , Proteínas de Unión al ARN/genética
17.
Acta Neurol Belg ; 123(3): 807-814, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37046133

RESUMEN

BACKGROUND: A specific scoring system for syringomyelia is lacking. Our objective was to investigate the value of a novel scoring system (Syringomyelia Outcome Scale of Xuanwu hospital, SOS-XW) in assessing surgical outcomes in the treatment of syringomyelia (SM) associated with Chiari malformation type I (CM I). METHODS: A quantitative evaluation system (SOS-XW) of SM includes 4 parameters: pain (P), sensation (S), movement (M), and syringomyelia tension index (STI). The clinical data of 88 patients with CM I-related syringomyelia treated by foramen magnum and Magendie dredging (FMMD) from January 2018 to January 2019 were retrospectively analysed with a mean follow-up of 14.3 months, and the SOS-XW score was used to assess the efficacy. RESULTS: The higher the SOS-XW score, the more severe was the SM and related symptoms. The mean preoperative score was 5.97, and the postoperative score was 2.66. The symptom improvement rates were 77.78% for P, 69.01% for S, 31.82% for M, and 95.06% for the syringomyelia tension index (STI). The symptom improvement rate of the PSM score was weakly correlated with the improvement rate of STI, R2 = 0.0016. The percentage of PSM (P + S + M) improvement was lower in patients with an STI of 0. The postoperative SOS-XW score was positively correlated with the postoperative JOA score, R2 = 0.8314. The positive detection rate of SOS-XW was higher than that of the JOA score. CONCLUSIONS: To evaluate the surgical procedure efficacy in the treatment of syringomyelia, the SOS-XW score can provide a more objective, detailed, and comprehensive analysis, especially STI. A reduction in STI is the practical standard for assessing the effectiveness of surgery.


Asunto(s)
Malformación de Arnold-Chiari , Siringomielia , Humanos , Adulto , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/cirugía , Siringomielia/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Descompresión Quirúrgica/métodos , Foramen Magno , Imagen por Resonancia Magnética
18.
Exp Neurol ; 365: 114430, 2023 07.
Artículo en Inglés | MEDLINE | ID: mdl-37121428

RESUMEN

Ependymal cilia, which are maintained by the Connexin 43 (Cx43) and protected by the actin network, play an essential role in regulating cerebrospinal fluid (CSF) circulation. The decline of ependymal cilia has been reported in syringomyelia, but the underlying mechanism remains unclear. In this study, we used an extradural compression-induced syringomyelia rat model to investigate the changes in cilia and related pathologies during the formation of syringomyelia. We divided rats into control and syringomyelia groups and sacrificed them at three time points, 7, 14, and 28 days postoperative (dpo). Scanning electron microscopy (SEM) and immunofluorescence (IF) were used to illustrate the number and morphology of ependymal cilia. IF was also used to show the status of centrioles, actin network, and Cx43 (the main component of the gap junction). Transmission electron microscopy (TEM) was used to observe the structure of the gap junction. The results showed that most syringomyelia were located at segments (T10-12) rostral to the compression site (T13). SEM images showed that the number of cilia in the central canal (CC) declined in two phases during the development of syringomyelia (early stage, 7 dpo; later stage, 14 and 28 dpo). The number of cilia showed a significant difference between the early and later stages of syringomyelia development. Additionally, TEM showed the absence of gap junction and IF illustrated less Cx43 expression in ependymal cells (ECs) at the compression site in both the early and later stages. Actin network disruption and centrioles reduction at adjacent segments rostral to the compression site were found in the later stage. These findings indicate that the loss of Cx43 at the compression site may be related to cilia detachment at rostral adjacent segments by disrupting intercellular communication in the early stage of syringomyelia development. This early cilia decline then causes actin network disorganization, further aggravating cilia decline by exposing centrioles to CSF shear stress in the later stage. These findings suggest a potential mechanism of ependymal cilia decline in the development of syringomyelia and may provide a novel perspective for future research in this area.


Asunto(s)
Siringomielia , Animales , Ratas , Actinas/metabolismo , Cilios/metabolismo , Cilios/patología , Conexina 43 , Uniones Comunicantes/metabolismo , Uniones Comunicantes/patología , Siringomielia/metabolismo , Siringomielia/patología
19.
Clin Neurol Neurosurg ; 227: 107637, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36857885

RESUMEN

OBJECTIVES: To analyze the prognostic and risk factors related to surgical treatment of central spinal cord syndrome (CSS) and to find out the optimal timing of operative management. METHODS: From January 2011 to January 2019, a consecutive series of 128 patients with CSS confirmed by magnetic resonance imaging (MRI) were retrospectively analyzed including their clinical records and radiologic data from a prospectively maintained database in a single center. RESULTS: According to the prognosis evaluated by the modified Japanese Orthopedic Association (mJOA), American Spinal Injury Association (ASIA) motor score (AMS), and ASIA impairment scale (AIS) grade, the overall postoperative outcome was good. Finally, it was found that surgical timing, presence of myelopathy or not at baseline, AMS at admission, and compression ratio were independent factors affecting the prognosis. Surgery as soon as possible after the occurrence of CSS is still advocated. CONCLUSION: Cervical myelopathy at baseline, compression ratio, and AMS score on admission were independent prognostic factors for the surgical treatment of CSS. If surgical indications are clear, early surgical intervention should be actively considered.


Asunto(s)
Síndrome del Cordón Central , Compresión de la Médula Espinal , Enfermedades de la Médula Espinal , Osteofitosis Vertebral , Humanos , Pronóstico , Compresión de la Médula Espinal/diagnóstico por imagen , Compresión de la Médula Espinal/etiología , Compresión de la Médula Espinal/cirugía , Síndrome del Cordón Central/diagnóstico por imagen , Síndrome del Cordón Central/cirugía , Resultado del Tratamiento , Estudios Retrospectivos , Enfermedades de la Médula Espinal/cirugía , Vértebras Cervicales/diagnóstico por imagen , Vértebras Cervicales/cirugía , Vértebras Cervicales/patología , Imagen por Resonancia Magnética/métodos , Factores de Riesgo , Médula Espinal/diagnóstico por imagen , Médula Espinal/cirugía
20.
Front Surg ; 10: 1130489, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36950057

RESUMEN

Study design: This is a retrospective study. Objective: To demonstrate a modified oblique lumbar interbody fusion (OILF) technique for L1-L5. Methods: The modified technique splits anterior portion of psoas belly to access the oblique corridor (OC) anteroinferior to psoas, minimizing psoas manipulation and retraction and avoiding nerve injury while offering excellent microscopic visualization. Psoas weakness and neurovascular complication rates in patients treated with traditional OLIF (T-OLIF) or anteroinferior psoas OLIF (AP-OLIF) were retrospectively reviewed. Clinical outcomes were also reviewed. Results: A total of 162 cases treated with T-OLIF (n = 73) and AP-OLIF (n = 89) for degenerative lumbar disease were included. The mean operative time and blood loss were less with AP-OLIF (P < 0.01). Approach related complications were 14 (19.1%) with T-OLIF and 4 (4.5%) with AP-OLIF. Postoperative visual analog scale (VAS) and Oswestry Disability Index (ODI) scores improved in both T-OIF and AP-OIF groups (P < 0.01). Conclusion: The modified OLIF technique (AP-OLIF) is characterized by an easy exposure of the lumbar spine under direct microscopic vision, resulting in less psoas weakness and neurovascular injury.

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