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Multicentric Osteolysis Nodulosis and Arthropathy (MONA) is a rare skeletal disorder driven by mutations in the MMP2 gene, leading to bone and joint degradation. This case series presents three unique MONA cases, highlighting clinical, radiological, and genetic aspects. These insights shed light on the complexities of MONA, aiding early diagnosis and multidisciplinary management. Case 1 is a 13-year-old male, born to consanguineous parents, presented with a 5-year history of progressive joint deformities, pain, and difficulty walking. Initially diagnosed as juvenile idiopathic arthritis (JIA), despite treatment, his symptoms persisted. Examination revealed multiple clinical findings, including joint contractures and nodules. Genetic analysis identified a pathogenic variant in the MMP2 gene, confirming MONA. Case 2 and Case 3 were two siblings, aged 12 and 17 years respectively, who presented progressive joint contractures in their hands and feet since early childhood. Clinical examinations revealed contractures and subcutaneous nodules. Genetic analysis confirmed MONA with a shared homozygous pathogenic MMP2 variant, emphasising the genetic basis of this rare disorder.

Objectives This study aims to assess the predictive value of preoperative parameters on recovery outcomes in patients with myelopathy caused by cervical ossification of the posterior longitudinal ligament (COPLL) undergoing laminoplasty (LAMP). Methods A retrospective analysis was performed on myelopathy patients caused by COPLL who underwent LAMP between 2017 and 2020. Preoperative variables, including basic epidemiological characteristics, comorbidities, functional scores, K-line-related parameters, Torg-Pavlov ratio, maximal SCOR and COPLL shape, were analyzed for their predictive influence on postoperative outcomes in cervical spine function, upper and lower extremity function, bladder function, and quality of life (QOL). Binary logistic regression model analyses were used to evaluate predictive accuracy. Results A total of 84 patients were included in the study. Preoperative parameters were significant predictors of postoperative improvement following LAMP surgery for myelopathy caused by COPLL. K-line-related factors, including K-line (-) (AUC = 0.80) and K-line on sagittal T1WI (-) (AUC = 0.76), were important predictors of cervical spine function improvement. Preoperative QOL scores (AUC = 0.78) also played a significant role in predicting cervical spine function improvement. For upper extremity function, preoperative upper extremity scores were a key predictor (AUC = 0.79), while C4-C6 K-line (-) (AUC = 0.81) was also a relevant factor. Similarly, preoperative lower extremity scores were crucial for predicting lower extremity function improvement (AUC = 0.85), and preoperative QOL scores were significant predictors of QOL improvement (AUC = 0.78). Other parameters, such as the Torg-Pavlov ratio, maximal SCOR, and the shape of COPLL, provided supplementary predictive value, though their influence was secondary to JOACMEQ scores and K-line parameters. Bladder function showed minimal postoperative improvement, with preoperative bladder status and the Torg-Pavlov ratio at C5 being the primary predictors for bladder improvement. Overall, preoperative K-line findings, JOACMEQ scores, and spinal canal measurements provided valuable guidance for postoperative expectations and surgical planning. Conclusions Preoperative K-line parameters and JOACMEQ scores are robust predictors of functional recovery in myelopathy patients caused by COPLL undergoing LAMP. While Torg-Pavlov ratio, maximal SCOR, and the shape of COPLL offer additional predictive value for overall recovery, they remain useful for preoperative surgical planning. These findings emphasize the importance of comprehensive preoperative assessment to optimize outcomes.

•The prognosis of rapid progressive CSM without a history of major trauma is worse than that of common chronic CSM.•The rapid neurological deterioration can be identified in some extent by TPR MRI (< 0.4), compression ratio (≥50 %), sagittal diameter of ISI (≥50 % of spinal canal diameter).•Early intervention was recommended for the high-risk people for rapid progressive CSM. Cervical spondylotic myelopathy (CSM) is a major cause of cervical spinal cord dysfunction in people over 55 years of age. Most patients with CSM usually present with chronic and phased compression, however, some patients with CSM develop rapid severe neurological dysfunction without any trauma. To our knowledge, markers that can be used for early identification of patients with potential to develop rapid neurological deterioration have not been totally identified. Here, we evaluate epidemiological, clinical and radiographic features associated with the development and prognosis of rapid progressive cervical spondylotic myelopathy (rp-CSM). A retrospective study was carried out for 175 patients diagnosed with CSM between March 2011 and January 2017 at West China Hospital. Patients were divided into rp-CSM group and chronic CSM (c-CSM) group based on the time taken for neurological deterioration to occur and the severity of preoperative neurological dysfunction. The clinical outcomes were assessed using the Modified Japanese Orthopaedic Association (mJOA) score, and imaging parameters such as Torg-Pavlov Ratio (TPR), intervertebral disc level compression ratio and increased signal intensity (ISI) on T2W1. Multivariate analysis was used to compare the outcomes between the two groups and identify potential predictors for rapid neurological dysfunction in CSM patients. Out of the 175 patients enrolled in the study, 25 developed rp-CSM (18 males; median age 59.04 ± 12.81 years) and the remaining 75 (54 males; median age 56.88 ± 12.31 years) were used as controls for the study (c-CSM group). The average time taken to develop severe neurological deterioration was 0.8 month in rp-CSM group and 24 month in c-CSM group (p = 0.001), while the preoperative mJOA scores were 6 in rp-CSM patients and 12 in c-CSM patients (p = 0.014). In addition, rp-CSM patients demonstrated worse outcomes than the controls in one year after surgery (mJOA improvement rate 54.5 % and 80 %, respectively, p = 0.021). There were no differences in the clinical parameters evaluated between the two groups except for the history of diabetes and smoking. Analysis of radiographic parameters indicated that TPR MRI, intervertebral disc level compression ratio and increased signal intensity (ISI) on T2W1 were poor in rp-CSM patients compared to c-CSM patients. Regression analysis also showed that the history of diabetes, TPR MRI < 0.4, compression ratio ≥50 %, and the sagittal diameter of ISI ≥ 50 % of spinal canal diameter on T2W1 were strongly associated with the rapid progressive neurological dysfunction in patients with CSM. The prognosis of rapid progressive CSM is worse than that of common chronic CSM. The rapid neurological deterioration can be identified by TPR MRI (<0.4), compression ratio (≥50 %), sagittal diameter of ISI (≥50 % of spinal canal diameter). Besides, a history of diabetes is a risk factor for the development of rp-CSM.

Purpose The purpose of this study was to develop a simple and clinically useful morphological classification system for congenital lumbar spinal stenosis using sagittal MRI, allowing clinicians to recognize patterns of lumbar congenital stenosis quickly and be able to screen these patients for tandem cervical stenosis. Methods Forty-four subjects with an MRI of both the cervical and lumbar spine were included. On the lumbar spine MRI, the sagittal canal morphology was classified as one of three types: Type I normal, Type II partially narrow, Type III globally narrow. For the cervical spine, the Torg-Pavlov ratio on X-ray and the cervical spinal canal width on MRI were measured. Kruskal–Wallis analysis was done to determine if there was a relationship between the sagittal morphology of the lumbar spinal canal and the presence of cervical spinal stenosis. Results Subjects with a type III globally narrow lumbar spinal canal had a significantly lower cervical Torg-Pavlov ratio and smaller cervical spinal canal width than those with a type I normal lumbar spinal canal. Conclusion A type III lumbar spinal canal is a globally narrow canal characterized by a lack of spinal fluid around the conus. This was defined as “functional lumbar spinal stenosis” and is associated with an increased incidence of tandem cervical spinal stenosis.

This compelling book provides a vivid firsthand account of the student demonstrations and massacre in Tiananmen Square in 1989.Uniquely placed as a Western observer drawn into active participation through Chinese friends in the uprising, Philip J Cunningham offers a remarkable day-by-day account of Beijing students desperately trying to secure.

ObjectiveTo analyze cervical spondylotic myelopathy (CSM) predictive factors in patients with lumbar spinal stenosis (LSS).MethodsTwo hundred thirty-seven patients who visited for low back pain, lower limb pain and/or lower limb numbness and who were diagnosed with LSS were enrolled in this study. The ratio of males to females was 117–120, and the mean age was 68.8 years (range 45–87 years). LSS and CSM were diagnosed by characteristic symptoms, physical findings and MRI. We examined gender, age, Torg-Pavlov ratio (TPR), spondylolisthesis or spondylosis, LSS symptom types and number of stenosis segments with LSS to clarify predictive factors for CSM.ResultsThere were 21 (8.86%) patients with coexistent CSM among 237 LSS patients. CSM morbidity was significantly more common among males compared with females. TPR was 0.71 ± 0.09 in the CSM patients and 0.81 ± 0.10 in the non-CSM patients. TPR of the CSM patients was significantly smaller than that of the non-CSM patients. We analyzed to determine the predictive factors of CSM and TPR was identified. The predictive value of TPR for CSM was 0.78.ConclusionTorg-Pavlov ratio was the most important predictive factor of CSM in patients with LSS.

Until now, archaeological and historical studies of Mesoamerican plazas have been scarce compared to studies of the surrounding monumental architecture such as pyramidal temples and palaces. Many scholars have assumed that ancient Mesoamericans invested their labor, wealth, and symbolic value in pyramids and other prominent buildings, viewing plazas as by-products of these buildings. Even when researchers have recognized the potential significance of plazas, they have thought that plazas as vacant spaces could offer few clues about their cultural and political roles.Mesoamerican Plazaschallenges both of these assumptions.The primary question that has motivated the contributors is how Mesoamerican plazas became arenas for the creation and negotiation of social relations and values in a community. The thirteen contributions stress the significance of interplay between power relations and embodied practices set in specific historical and material settings, as outlined by practice theory and performance theory. This approach allows the contributors to explore broader anthropological issues, such as the negotiation of power relations, community making, and the constitution of political authorities.Overall, the contributions establish that physical interactions among people in communal events were not the outcomes of political machinations held behind the scenes, but were the actual political processes through which people created, negotiated, and subverted social realities. If so, spacious plazas that were arguably designed for interactions among a large number of individuals must have also provided critical arenas for the constitution and transformation of society.

Torg–Winchester syndrome (OMIM 259600) is an autosomal recessive multicentric osteolysis disorder. Mutations in the gene for matrix metalloproteinase 2 ( MMP2 ) are involved in its pathogenesis. This is the first report of Torg–Winchester syndrome in east Asians. A 31-year-old female Korean patient had the typical clinical phenotypes of the syndrome, including shortening of trunk and limbs and severe osteolysis resulting in extremely small hands and feet. In addition, she had cord compression at the cervico-medullary junction, as well as lumbar dural ectasia. Molecular analysis revealed a novel homozygous missense mutation of MMP2 , c.1217G>A (p.G406D). Gelatin zymography demonstrated a complete loss of the MMP2 activity of the mutation. Our results provide insights into the clinical and radiological features and pathogenic mechanisms of the syndrome.

Background A cervical Torg ratio of 0.8 has been used as a screening tool to determine the presence of cervical spinal stenosis. However, there have been no studies done to define the Torg ratio in the lumbar spine for predicting lumbar spinal stenosis (LSS). Torg ratios have never been correlated with the actual calculated canal area as derived from anatomic specimens. The aim of this study was to provide an analysis of the utility of the lumbar Torg ratio for predicting LSS based on objective measurements of skeletal specimens. Materials and methods 420 adult skeletal specimens from the Hamann Todd Collection in the Cleveland Museum of Natural History were selected. Digital calipers were used to measure the sagittal diameter (SCD), interpedicular distance, pedicle length, and vertebral body diameter. The canal area at each level was calculated using a geometric formula. A standard distribution curve for canal area and Torg ratio was created, and values that were that is less than the mean minus two standard deviations (SD) below the mean were considered stenotic. Regression analysis was performed to determine if the Torg ratio was correlated with canal area, and if a “below normal” Torg ratio was predictive of LSS. Results The Torg ratio for 2SD below the mean was defined as 0.43 at L1, 0.43 at L2, 0.41 at L3, 0.38 at L4, 0.37 at L5. Regression analysis revealed a significant association of the Torg ratio with canal area ( p  < 0.01). A Torg ratio that was less than the mean − 2SD predicted canal stenosis at L2, L3, L4, and L5 ( p  < 0.01). Using a Torg ratio of <0.5 predicted stenosis with a sensitivity of 86 % and specificity of 52 % at all lumbar levels. Conclusions Based on the results of our study, we have defined the lower limit of the normal Torg ratio at each level. A Torg ratio of <0.5 predicts LSS and could be a useful radiological tool for LSS screening.