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1.
J Neurosurg Spine ; : 1-9, 2024 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-38848604

RESUMO

OBJECTIVE: Intraspinal cysts are uncommon, and the success rate of complete resection is still low for spinal neurenteric cysts (NCs). The aim of this study was to evaluate the efficacies of an anterior microscopic surgical approach in the treatment of ventral and ventrolateral subaxial cervical NCs (SCNCs). METHODS: Between 2019 and 2022, 9 patients with NCs of the subaxial spine underwent an anterior microsurgical approach. Their clinical presentations, radiological features, operative findings, and follow-up data were retrospectively reviewed and analyzed. RESULTS: All spinal cysts were intradural and extramedullary in origin. Five patients were first-time cases while 4 patients with recurrence underwent revision surgery. The most common clinical manifestation was pain (77.78%). One patient was found to have a concomitant disorder of Klippel-Feil syndrome. Microscopically confirmed gross-total resection was achieved in 8 patients (88.89%) based on clinical comparisons between pre- and postoperative MRI and intraoperative video. One patient had symptom recurrence 1 year after subtotal resection, while there was no evidence of recurrence during follow-up for the other patients. Dense adhesions within the spinal cord were observed in 8 patients (88.89%) intraoperatively. Most importantly, the surgical outcome was significantly improved in all patients, and the mean (± SE) Japanese Orthopaedic Association score increased from 11.33 ± 0.91 preoperatively to 16.22 ± 0.32 postoperatively (p = 0.008). CONCLUSIONS: An anterior surgical approach was proven to be both safe and effective in treating the ventral or ventrolateral SCNCs. The authors believe that an anterior microsurgical approach should be considered as a useful approach especially in patients with ventral recurrent SCNCs. Its clinical efficacy compared with a posterior approach in ventral spinal cyst may be better as most of the neurenteric cysts are ventrally or ventrolaterally located.

2.
Clin Neurol Neurosurg ; 242: 108315, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38749356

RESUMO

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.


Assuntos
Vértebras Cervicais , Descompressão Cirúrgica , Dura-Máter , Ossificação do Ligamento Longitudinal Posterior , Tomografia Computadorizada por Raios X , Humanos , Ossificação do Ligamento Longitudinal Posterior/cirurgia , Ossificação do Ligamento Longitudinal Posterior/diagnóstico por imagem , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Tomografia Computadorizada por Raios X/métodos , Dura-Máter/cirurgia , Dura-Máter/diagnóstico por imagem , Descompressão Cirúrgica/métodos , Estudos Retrospectivos , Vértebras Cervicais/cirurgia , Vértebras Cervicais/diagnóstico por imagem , Adulto , Fatores de Risco
3.
Neurosurgery ; 2024 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-38619238

RESUMO

BACKGROUND AND OBJECTIVES: Venous hypertensive myelopathy (VHM), mainly induced by the spinal dural arteriovenous fistula, is a congestive spinal cord injury that currently has no appropriate animal model available in preclinical research. METHODS: Sprague Dawley rats (280-320 g) were used. The rats were divided into 3 groups: (1) Group 1, which underwent renal artery-dorsal spinal venous bypass (AVB group); (2) Group 2, which underwent renal artery-dorsal spinal venous bypass and drainage vein stenosis (AVB/VS group); and (3) Control group, with T13 dorsal vein ligation. The success of the model was assessed using Doppler ultrasound and 7.0-T magnetic resonance imaging. Transmission electron microscopy, histochemistry, proteomics, and western blot analysis were used to evaluate ultrastructural, pathological, and molecular features in the spinal cord and cerebrospinal fluid (CSF). RESULTS: The success rate of the arteriovenous bypass was 100% at 5 days and 83% at 2 weeks. The locomotor assessment showed decreased lower extremity strength in the AVB/VS group (P = .0067), whereas unremarkable changes were found in the AVB and Control groups. Histochemical staining suggested a 2-fold expansion of the dorsal spinal vein in the AVB/VS group, which was lower than that in the AVB group (P < .05); however, the former displayed greater myelin and neuronal damage (P < .05) and slight dilatation of the central canal (P > .05). Proteomics analysis revealed that the complement and coagulation cascade pathways were upregulated in the CSF of AVB/VS rats, whereas the C3 level was elevated both in the CSF and bilateral spinal cord. Furthermore, overexpression of C3, ITGB2, and CD9 in the spinal cord was confirmed by immunoblotting. CONCLUSION: These findings suggest that the AVB/VS model can effectively mimic the clinical and molecular characteristics of VHM. Furthermore, they suggest that impaired deep intramedullary venous drainage is the key reason for the VHM.

4.
Br J Cancer ; 130(10): 1609-1620, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38605247

RESUMO

BACKGROUND: Chordomas are rare osseous neoplasms with a dismal prognosis when they recur. Here we identified cell surface proteins that could potentially serve as novel immunotherapeutic targets in patients with chordoma. METHODS: Fourteen chordoma samples from patients attending Xuanwu Hospital Capital Medical University were subjected to single-cell RNA sequencing. Target molecules were identified on chordoma cells and cancer metastasis-related signalling pathways characterised. VEGFR-targeting CAR-T cells and VEGFR CAR-T cells with an additional TGF-ß scFv were synthesised and their in vitro antitumor activities were evaluated, including in a primary chordoma organoid model. RESULTS: Single-cell transcriptome sequencing identified the chordoma-specific antigen VEGFR and TGF-ß as therapeutic targets. VRGFR CAR-T cells and VEGFR/TGF-ß scFv CAR-T cells recognised antigen-positive cells and exhibited significant antitumor effects through CAR-T cell activation and cytokine secretion. Furthermore, VEGFR/TGF-ß scFv CAR-T cells showed enhanced and sustained cytotoxicity of chordoma cell lines in vitro compared with VRGFR CAR-T cells. CONCLUSIONS: This study provides a comprehensive single-cell landscape of human chordoma and highlights its heterogeneity and the role played by TGF-ß in chordoma progression. Our findings substantiate the potential of VEGFR as a target for CAR-T cell therapies in chordoma which, together with modulated TGF-ß signalling, may augment the efficacy of CAR-T cells.


Assuntos
Cordoma , Imunoterapia Adotiva , Análise de Célula Única , Humanos , Cordoma/terapia , Cordoma/genética , Cordoma/patologia , Cordoma/imunologia , Imunoterapia Adotiva/métodos , Feminino , Masculino , Fator de Crescimento Transformador beta/metabolismo , Linhagem Celular Tumoral , Receptores de Antígenos Quiméricos/imunologia , Receptores de Antígenos Quiméricos/genética , Pessoa de Meia-Idade , Adulto , Neoplasias Ósseas/terapia , Neoplasias Ósseas/patologia , Neoplasias Ósseas/genética , Neoplasias Ósseas/imunologia
5.
Childs Nerv Syst ; 40(7): 2153-2160, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38498172

RESUMO

PURPOSE: Surgical treatment for atlantoaxial instability in pediatric patients is challenging. We report our experience with posterior intra-articular distraction technique in treating this disorder. METHODS: This is a retrospective descriptive study which included 15 patients of atlantoaxial instability whose age was less than 16 years at the time of clinical presentation. All patients underwent anterior soft tissue released through a posterior-only approach, followed by intra-facet cage implantation, cantilever correction, and instrumentation. Clinical results were measured using the Japanese Orthopedic Association (JOA) scale and radiographic measurements including the atlantodental interval (ADI), posterior atlantodental interval (pADI), the distance of odontoid tip above Chamberlain's line, clivuscanal angle (CCA), and triangular area (TA) of craniovertebral junction. RESULTS: The follow-up period ranged from 18 to 72 months, with an average of 41.2 ± 15.2 months. The JOA score increased from 13.6 ± 2.3 to 16.6 ± 0.8. ADI decreased from 4.31 ± 2.37 to 1.85 ± 1.09 mm, and TA decreased from 261.96 ± 107.99 to 197.12 ± 72.37 mm2. pADI increased from 12.89 ± 3.52 to 18.25 ± 3.89 mm, and CCA improved from 132.19 ± 16.34 to 144.35 ± 13.91°. All changes in measurements showed statistically significant. There were no evidence of surgery-related complications or iatrogenic secondary cervical deformity during follow-up. Radiological evaluation showed satisfactory corrections and bony fusions of C1-2 facet joint in all cases. CONCLUSION: Posterior intra-articular distraction followed by cage implantation and cantilever correction can be one of the safe and effective ways to solve atlantoaxial instability in pediatric patients.


Assuntos
Articulação Atlantoaxial , Instabilidade Articular , Humanos , Articulação Atlantoaxial/cirurgia , Articulação Atlantoaxial/diagnóstico por imagem , Instabilidade Articular/cirurgia , Instabilidade Articular/diagnóstico por imagem , Feminino , Masculino , Criança , Adolescente , Estudos Retrospectivos , Resultado do Tratamento , Fusão Vertebral/métodos , Pré-Escolar
6.
World Neurosurg ; 185: e1361-e1371, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38522787

RESUMO

OBJECTIVE: Recent years have witnessed a rapidly growing interest in CVJ bony abnormalities, and a qualitative and quantitative analysis of relevant literatures is necessary. This study aims to identify and summarize the published articles related to craniovertebral junction bony abnormalities, to analyze and visualize the current research trends and major contributors. METHODS: We collected data from Web of Science, excluding certain article types. Two researchers screened articles for relevance. Data were organized with EndnoteX9, and analyzed using VOSviewer and CiteSpace for co-authorship, co-occurrence, keyword burst, and co-citation analyses to identify research trends and collaborations. RESULTS: A total of 2,776 articles were included, revealing an increasing trend in annual publications of CVJ bony abnormalities. The USA was the leading country. King Edward Memorial Hospital was the most prolific institution, and Seth GS Medical College had the most citations. The Spine is the most popular journal with the highest number of publications and citations. Professor Goel Atul from India emerged as the most influential pioneer in this field. Keyword analysis highlighted surgical techniques, diagnosis, and anatomy as the primary research hotspots and Fixation, Placement, and Basilar invagination gradually become the new research trend. However, there is a relative weakness in basic research and epidemiology. CONCLUSIONS: This study provides valuable insights into the current research trends and critical contributors in CVJ bony abnormalities, guiding evidence-based decisions and fostering international collaborations to advance knowledge in this field.


Assuntos
Bibliometria , Humanos , Articulação Atlantoccipital/anormalidades , Articulação Atlantoaxial/anormalidades
7.
World Neurosurg ; 185: 45-54, 2024 05.
Artigo em Inglês | MEDLINE | ID: mdl-38320651

RESUMO

Spinal cord injury (SCI) results in neurological deficits below the level of injury, causing motor dysfunction and various severe multisystem complications. Rehabilitative training plays a crucial role in the recovery of individuals with SCI, and exoskeleton serves as an emerging and promising tool for rehabilitation, especially in promoting neuroplasticity and alleviating SCI-related complications. This article reviews the classifications and research progresses of medical exoskeletons designed for SCI patients and describes their performances in practical application separately. Meanwhile, we discuss their mechanisms for enhancing neuroplasticity and functional remodeling, as well as their palliative impacts on secondary complications. The potential trends in exoskeleton design are raised according to current progress and requirements on SCI rehabilitation.


Assuntos
Exoesqueleto Energizado , Plasticidade Neuronal , Traumatismos da Medula Espinal , Humanos , Traumatismos da Medula Espinal/reabilitação , Traumatismos da Medula Espinal/fisiopatologia , Plasticidade Neuronal/fisiologia , Reabilitação Neurológica/métodos , Reabilitação Neurológica/instrumentação , Recuperação de Função Fisiológica/fisiologia
8.
Neurospine ; 21(1): 212-222, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38317552

RESUMO

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.

9.
J Neuroinflammation ; 21(1): 60, 2024 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-38419042

RESUMO

BACKGROUND: The spinal inflammatory signal often spreads to distant segments, accompanied by widespread pain symptom under neuropathological conditions. Multiple cytokines are released into the cerebrospinal fluid (CSF), potentially inducing the activation of an inflammatory cascade at remote segments through CSF flow. However, the detailed alteration of CSF in neuropathic pain and its specific role in widespread pain remain obscure. METHODS: A chronic constriction injury of the infraorbital nerve (CCI-ION) model was constructed, and pain-related behavior was observed on the 7th, 14th, 21st, and 28th days post surgery, in both vibrissa pads and hind paws. CSF from CCI-ION rats was transplanted to naïve rats through intracisternal injection, and thermal and mechanical allodynia were measured in hind paws. The alteration of inflammatory cytokines in CCI-ION's CSF was detected using an antibody array and bioinformatic analysis. Pharmacological intervention targeting the changed cytokine in the CSF and downstream signaling was performed to evaluate its role in widespread pain. RESULTS: CCI-ION induced local pain in vibrissa pads together with widespread pain in hind paws. CCI-ION's CSF transplantation, compared with sham CSF, contributed to vibrissa pad pain and hind paw pain in recipient rats. Among the measured cytokines, interleukin-6 (IL-6) and leptin were increased in CCI-ION's CSF, while interleukin-13 (IL-13) was significantly reduced. Furthermore, the concentration of CSF IL-6 was correlated with nerve injury extent, which gated the occurrence of widespread pain. Both astrocytes and microglia were increased in remote segments of the CCI-ION model, while the inhibition of astrocytes in remote segments, but not microglia, significantly alleviated widespread pain. Mechanically, astroglial signal transducer and activator of transcription 3 (STAT3) in remote segments were activated by CSF IL-6, the inhibition of which significantly mitigated widespread pain in CCI-ION. CONCLUSION: IL-6 was induced in the CSF of the CCI-ION model, triggering widespread pain via activating astrocyte STAT3 signal in remote segments. Therapies targeting IL-6/STAT3 signaling might serve as a promising strategy for the widespread pain symptom under neuropathological conditions.


Assuntos
Interleucina-6 , Neuralgia , Ratos , Animais , Interleucina-6/metabolismo , Ratos Sprague-Dawley , Fator de Transcrição STAT3/metabolismo , Gliose/complicações , Constrição , Hiperalgesia/etiologia , Hiperalgesia/tratamento farmacológico , Neuralgia/tratamento farmacológico , Citocinas
10.
Spine (Phila Pa 1976) ; 49(6): E62-E71, 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38014747

RESUMO

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.


Assuntos
Traumatismos da Medula Espinal , Siringomielia , Humanos , Siringomielia/diagnóstico por imagem , Siringomielia/etiologia , Siringomielia/cirurgia , Estudos de Coortes , Traumatismos da Medula Espinal/complicações , Prognóstico , Imunoglobulinas
11.
J Neuroimmunol ; 383: 578191, 2023 10 15.
Artigo em Inglês | MEDLINE | ID: mdl-37660537

RESUMO

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.


Assuntos
Meningite , Humanos , Dura-Máter/diagnóstico por imagem , Dura-Máter/cirurgia , Dura-Máter/patologia , Hipertrofia , Meningite/complicações , Meningite/diagnóstico por imagem , Medula Espinal/patologia
12.
Neurospine ; 20(2): 498-506, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37401068

RESUMO

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.

13.
Neurospine ; 20(2): 701-708, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37401089

RESUMO

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.

14.
Front Neurol ; 14: 1178651, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37305741

RESUMO

Malignant peripheral nerve sheath tumors (MPNSTs) are commonly associated with poor prognosis and are primarily caused by germline mutations in the SMARCB1/INI-1 gene. However, these tumors are rarely found in the spine. This case report presents the case of a 3-year-old boy diagnosed with a lumbosacral dumbbell-shaped epithelioid MPNST, an extremely uncommon manifestation. Immunohistochemistry revealed the complete absence of the SMARCB1/INI-1 protein, and genetic testing identified a novel germline mutation in the SMARCB1/INI-1 gene in both the patient and his father, suggesting a "second-hit loss." One year of follow-up after the tumor's radical resection revealed no suspected metastasis. This case report offers novel genetic research results regarding spinal dumbbell-shaped MPNSTs. Six studies, including 13 cases associated with spinal dumbbell MPNST, were included in the literature. The range of age of these patients varied from 2 to 71 years. Of the 12 known patients diagnosed with spinal dumbbell MPNST, only one received radiation therapy, while the rest underwent surgery. Two patients who underwent partial resection had metastases after surgery, while one of the five patients who underwent complete surgical resection alone had no distant metastases and a good prognosis, indicating that radical resection is more likely to be effective in inhibiting distant metastasis and improving the prognosis.

15.
Clin Nucl Med ; 48(8): 702-703, 2023 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-37276463

RESUMO

ABSTRACT: A 60-year-old woman presented with left upper limb weakness. Spinal MRI revealed C2-C3 heterogeneous mass with no hypermetabolic foci on 18 F-FDG PET. Total resection was performed, and the pathology showed gliosis. Her limb weakness worsened 3 months after surgery. Spinal MRI revealed long intramedullary mass from medulla oblongata to C5, which showed obvious hypermetabolism under 18 F-FDG PET with an SUV max of 7.11, but 18 F-FET PET indicated mild hypermetabolism with an SUV max of 2.29. Malignancy was suspected, and C2-C4 biopsy was performed. The pathology results confirmed the diagnosis of gliosis, and the postoperative spinal 18 F-FDG PET results were considered pseudoprogression.


Assuntos
Fluordesoxiglucose F18 , Neoplasias , Feminino , Humanos , Pessoa de Meia-Idade , Gliose , Coluna Vertebral , Diagnóstico Diferencial
16.
Int J Surg ; 109(8): 2276-2285, 2023 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-37204435

RESUMO

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.


Assuntos
Infecção da Ferida Cirúrgica , Vancomicina , Humanos , Infecção da Ferida Cirúrgica/epidemiologia , Infecção da Ferida Cirúrgica/etiologia , Estudos de Coortes , Fatores de Risco , Albuminas , Esteroides , Estudos Retrospectivos
17.
J Pathol ; 260(3): 317-328, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37114614

RESUMO

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.


Assuntos
Astrocitoma , Glioma , Humanos , Fosfatidilinositol 3-Quinases , Mutação , Glioma/genética , Medula Espinal/patologia , Proteínas de Ligação a RNA/genética
18.
Acta Neurol Belg ; 123(3): 807-814, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37046133

RESUMO

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.


Assuntos
Malformação de Arnold-Chiari , Siringomielia , Humanos , Adulto , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/cirurgia , Siringomielia/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Descompressão Cirúrgica/métodos , Forame Magno , Imageamento por Ressonância Magnética
19.
Oper Neurosurg (Hagerstown) ; 25(2): 125-135, 2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-37083634

RESUMO

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.


Assuntos
Articulação Atlantoaxial , Luxações Articulares , Platibasia , Siringomielia , Humanos , Siringomielia/diagnóstico por imagem , Siringomielia/cirurgia , Estudos Retrospectivos , Articulação Atlantoaxial/diagnóstico por imagem , Articulação Atlantoaxial/cirurgia , Luxações Articulares/diagnóstico por imagem , Luxações Articulares/cirurgia , Platibasia/diagnóstico por imagem , Platibasia/cirurgia , Resultado do Tratamento
20.
World Neurosurg ; 174: e131-e143, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36898626

RESUMO

OBJECTIVE: We used topology optimization technology to explore the new theory and method of interbody fusion cage design and realized an innovative design of interbody cages. METHODS: The lumbar spine of a normal healthy volunteer was scanned to perform reverse modeling. Based on the scan data for the L1-L2 segments of the lumbar spine, a three dimensional model was reconstructed to obtain the complete simulation model of the L1-L2 segment. The boundary inversion method was used to obtain approximately isotropic material parameters that can effectively characterize the mechanical behavior of vertebrae, thereby reducing the computational complexity. The topology description function was used to model the clinically used traditional fusion cage to obtain Cage A. The moving morphable void-based topology optimization method was used for the integrated design of size, shape, and topology to obtain the optimized fusion cage, Cage B. RESULTS: The volume fraction of the bone graft window in Cage B was 74.02%, which was 60.67% higher than that (46.07%) in Cage A. Additionally, the structural strain energy in the design domain of Cage B was 1.48 mJ, which was lower than that of Cage A (satisfying the constraints). The maximum stress in the design domain of Cage B was 5.336 Mpa, which was 35.6% lower than that (8.286 Mpa) of Cage A. In addition, the surface stress distribution of Cage B was more uniform than that of Cage A. CONCLUSIONS: This study proposed a new innovative design method for interbody fusion cages, which not only provides new insights into the innovative design of interbody fusion cages but may also guide the customized design of interbody fusion cages in different pathological environments.


Assuntos
Vértebras Lombares , Fusão Vertebral , Humanos , Vértebras Lombares/diagnóstico por imagem , Vértebras Lombares/cirurgia , Fusão Vertebral/métodos , Fenômenos Biomecânicos
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