Imaging Assessment of Nontraumatic Pathologic Conditions at the Craniovertebral Junction: A Comprehensive Review.

Nontraumatic pathologic conditions of the craniovertebral junction encompass a range of conditions affecting the complex anatomy of this region without direct physical injury. These conditions include congenital syndromes that predispose individuals to ligamentous laxity, potentially leading to instability. Additionally, rare but noteworthy cases such as Grisel syndrome, a cause of pediatric torticollis, may arise without a traumatic trigger. Inflammatory diseases, including rheumatoid arthritis, ankylosing spondylitis, and crystal deposition, can lead to cervical instability and spinal cord compression. Infections at the upper cervical spine are dominated by tuberculosis, typically transmitted through hematologic or lymphatic routes with characteristic imaging findings. On the other hand, purulent bacterial infections in this area are rare. Furthermore, although tumors involving the structures of the craniovertebral junction are infrequent, they can lead to significant complications, albeit less frequently through cord compression and more commonly via pathologic fractures or subluxation. The craniocervical junction is a complex anatomic region comprising ligaments, bones, joints, and muscles that support the head's weight and enable its wide range of motion. Accurate recognition and understanding of the complex anatomy and the various nontraumatic pathologic conditions at the craniovertebral junction are pivotal for initiating timely and appropriate treatment strategies. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Non-traumatic complete cervical spine dislocation with severe fixed kyphosis: successful multidisciplinary approach to a challenging case.

BACKGROUND: To our knowledge, there is no previous report in the literature of non-traumatic neglected complete cervical spine dislocation characterized by anterior spondyloptosis of C4, extreme head drop, and irreducible cervicothoracic kyphosis. CASE PRESENTATION: We report the case of a 33-year-old Caucasian man with a 17-year history of severe immune polymyositis and regular physiotherapy who presented with severe non-reducible kyphosis of the cervicothoracic junction and progressive tetraparesia for several weeks after a physiotherapy session. Radiographs, computed tomography, and magnetic resonance imaging revealed a complete dislocation at the C4-C5 level, with C4 spondyloptosis, kyphotic angulation, spinal cord compression, and severe myelopathy. Due to recent worsening of neurological symptoms, an invasive treatment strategy was indicated. The patient's neurological status and spinal deformity greatly complicated the anesthetic and surgical management, which was planned after extensive multidisciplinary discussion and relied on close collaboration between the orthopedic surgeon and the anesthetist. Regarding anesthesia, difficult airway access was expected due to severe cervical angulation, limited mouth opening, and thyromental distance, with high risk of difficult ventilation and intubation. Patient management was further complicated by a theoretical risk of neurogenic shock, motor and sensory deterioration, instability due to position changes during surgery, and postoperative respiratory failure. Regarding surgery, a multistage approach was carefully planned. After a failed attempt at closed reduction, a three-stage surgical procedure was performed to reduce displacement and stabilize the spine, resulting in correct spinal realignment and fixation. Progressive complete neurological recovery was observed. CONCLUSION: This case illustrates the successful management of a critical situation based on a multidisciplinary collaboration involving radiologists, anesthesiologists, and spine surgeons.

Incidence of skeletal-related events in patients with Ewing sarcoma: An observational retrospective study in Japan.

BACKGROUND: Skeletal-related events (SREs), including the pathological fracture, surgical treatment or radiation of bone lesions, malignant spinal cord compression, hypercalcemia, are important considerations when managing metastatic bone tumors; however, owing to their rarity, the incidence of SREs in patients with Ewing sarcoma remains unknown. METHODS: We retrospectively reviewed the clinical data from 146 patients with Ewing sarcoma treated at a single institution from 2005 to 2019. The median age at diagnosis was 22.7 years. Fifty patients (34.2%) had metastatic disease at diagnosis. The primary outcome was the SRE-free rate among patients with Ewing sarcoma. Moreover, we identified the risk factors for SREs using univariate or multivariate analyses. RESULTS: During the observational period (median, 2.6 years), SREs occurred in 23 patients. Radiation to the bone, malignant spinal cord compression, and hypercalcemia were documented as the initial SREs in 12 patients (52.2%), 10 patients (43.5%), and one patient (4.3%), respectively. The SRE-free rate was 94.2 ± 2.0, 87.3 ± 3.0, and 79.6 ± 3.8% at 1, 2, and 3 years after the initial visit, respectively. Multivariate analysis revealed bone metastasis at diagnosis (hazard ratio [HR] = 4.41, p = 0.007), bone marrow invasion (HR = 34.08, p < 0.001), and local progression or recurrence after definitive treatment (HR = 3.98, p = 0.012) as independent risk factors for SREs. CONCLUSIONS: SREs are non-rare events that can occur during the treatment course for Ewing sarcoma, with an especially high incidence of malignant spinal cord compression. Patients with metastatic disease at diagnosis, especially in the bone or bone marrow, or with local progression or recurrence after definitive treatment, should be carefully monitored for the occurrence of SREs. The most effective methods to monitor the occurrence of SREs and new preventative therapies for SREs should be investigated in the future.

Evaluation of the structural integrity of different spinal cord tracts with magnetization transfer ratio in degenerative cervical myelopathy.

PURPOSE: Degenerative cervical myelopathy (DCM) is a common cause of spinal cord dysfunction. In this study, we explored the potential of magnetization transfer ratio (MTR) for evaluating the structural integrity of spinal cord tracts in patients with clinically significant DCM. METHODS: Fifty-three patients with DCM and 41 patients with cervical radiculopathy were evaluated using high-resolution cervical spinal cord magnetic resonance imaging (MRI), which included the magnetization transfer technique. MRI data were analyzed with the Spinal Cord Toolbox (v5.5); MTR values in each spinal tract were calculated and compared between groups after correction for patient age and sex. Correlations between MTR values and patients' clinical disability rate were also evaluated. RESULTS: A statistically significant reduction in the average MTR of the spinal cord white matter, as well as the MTR of the ventral columns and lateral funiculi, was revealed in the DCM group (adjusted p < 0.01 for all comparisons). Furthermore, reductions in MTR values in the fasciculus cuneatus, spinocerebellar, rubrospinal, and reticulospinal tracts were found in patients with DCM (adjusted p < 0.01 for all comparisons). Positive correlations between the JOA score and the MTR within the ventral columns of the spinal cord (R = 0.38, adjusted p < 0.05) and the ventral spinocerebellar tract (R = 0.41, adjusted p < 0.05) were revealed. CONCLUSION: The findings of our study indicate that demyelination in patients with DCM primarily affects the spinal tracts of the extrapyramidal system, and the extent of these changes is related to the severity of the condition.

Isolated Rosai-Dorfman disease of the spine: A systematic literature review.

INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis involving the central nervous system in 5% of cases. Spinal location occurs in less than 1% of extranodal RDD and can be responsible for neurological manifestations. We present a systematic review of cases of isolated spinal RDD. We also report a new case of isolated spinal RDD revealed by spinal cord compression. MATERIALS AND METHODS: The systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline using the MEDLINE and SCOPUS databases and included case reports and case series describing isolated RDD of the spine. RESULTS: There were 53 patients with isolated spinal RDD (including our case). The mean age was 35.85±16.48 years. Neurological deficit was the most frequent clinical presentation (89%). RDD lesions were mainly located in the thoracic spine (51%), then the cervical spine (32%). The lesion was reported to be extradural (57%), intradural extramedullary (26%), intramedullary (7%), and in the vertebral body (10%). Histological examination showed emperipolesis in 73%. Histocytes were positive for S-100 protein in 83%. Treatment was based on surgery 96%), radiotherapy, chemotherapy, and adjunctive steroid therapy were indicated in four, one, and eight cases. After a mean follow-up period of 14.84±13.00 months, recurrence of RDD was noted in 15%. CONCLUSION: Spinal RDD is a rare condition, requiring meticulous histological examination for accurate diagnosis. Complete surgical resection is the treatment of choice. Adjuvant chemotherapy and radiotherapy can also be indicated in patients demonstrating partial improvement following surgery.

Post-operative complications affect survival in surgically treated metastatic spinal cord compression.

PURPOSE: The prevalence of metastatic epidural spinal cord compression (MESCC) is increasing globally due to advancements in cancer diagnosis and treatment. Whilst surgery can benefit specific patients, the complication rate can reach up to 34%, with limited reporting on their impact in the literature. This study aims to analyse the influence of major complications on the survival of surgically treated MESCC patients. METHODS: Consecutive MESCC patients undergoing surgery and meeting inclusion criteria were selected. Survival duration from decompressive surgery to death was recorded. Perioperative factors influencing survival were documented and analysed. Kaplan-Meier survival analysis at one year compared these factors. Univariate and multivariate Cox proportional hazard regression analyses were performed. Additionally, univariate analysis compared complicated and uncomplicated groups. RESULTS: Seventy-five patients were analysed. Median survival for this cohort was 229 days (95% CI 174-365). Surgical complications, low patient performance, and rapid primary tumour growth were significant perioperative variables for survival in multivariate analyses (p < 0.001, p = 0.003, and p = 0.02, respectively) with a hazard ratio of 3.2, 3.6, and 2.1, respectively. Univariate analysis showed no variables associated with complication occurrence. CONCLUSION: In this cohort, major surgical complications, patient performance, and primary tumour growth rate were found to be independent factors affecting one year survival. Thus, prioritizing complication prevention and appropriate patient selection is crucial for optimizing survival in this population.

Evaluation of computed tomography artefacts of carbon-fiber and titanium implants in patients with spinal oligometastatic disease undergoing stereotactic ablative radiotherapy.

This study evaluated artefacts on computed tomography (CT) images using Hounsfield units (HU) in patients with spinal oligometastatic disease who received carbon-fiber (CF; n = 11) or titanium (n = 11) spine implants and underwent stereotactic ablative radiotherapy (SABR). Pre- and postoperative HU were measured at the vertebral body, pedicle, and spinal cord at three different levels: the lower instrumented vertebra, the level of metastatic spinal cord compression, and an uninvolved level. Areas measured at each level were delicately matched pre- and postoperatively. Significant differences in HU were observed at the vertebral body, the pedicle, and the spinal cord at the lowest instrumented vertebra level for both CF and titanium (average increase 1.54-fold and 5.11-fold respectively). At the metastatic spinal cord compression level, a trend towards a higher HU-increase was observed in titanium compared with CF treated patients (average increase 2.51-fold and 1.43-fold respectively). The relatively high postoperative HU-increase after insertion of titanium implants indicated CT artefacts, while the relatively low HU-increase of CF implants was not associated with artefacts. Less CT artefacts could facilitate an easier contouring phase in radiotherapy planning. In addition, we propose a CT artefact grading system based on postoperative HU-increase. This system could serve as a valuable tool in future research to assess if less CT artefacts lead to time savings during radiotherapy treatment planning and, potentially, to better tumoricidal effects and less adverse effects if particle therapy would be administered.

Network pharmacology analysis and animal experiment validation of neuroinflammation inhibition by total ginsenoside in treating CSM.

BACKGROUND: Cervical spondylotic myelopathy (CSM) is a degenerative pathology that affects both upper and lower extremity mobility and sensory function, causing significant pressure on patients and society. Prior research has suggested that ginsenosides may have neuroprotective properties in central nervous system diseases. However, the efficacy and mechanism of ginsenosides for CSM have yet to be investigated. PURPOSE: This study aims to analyze the composition of ginsenosides using UPLC-MS, identify the underlying mechanism of ginsenosides in treating CSM using network pharmacology, and subsequently confirm the efficacy and mechanism of ginsenosides in rats with chronic spinal cord compression. METHODS: UPLC-Q-TOF-MS was utilized to obtain mass spectrum data of ginsenoside samples. The chemical constituents of the samples were analyzed by consulting literature reports and relevant databases. Ginsenoside and CSM targets were obtained from the TCMSP, OMIM, and GeneCards databases. GO and KEGG analyses were conducted, and a visualization network of ginsenosides-compounds-key targets-pathways-CSM was constructed, along with molecular docking of key bioactive compounds and targets, to identify the signaling pathways and proteins associated with the therapeutic effects of ginsenosides on CSM. Chronic spinal cord compression rats were intraperitoneally injected with ginsenosides (50 mg/kg and 150 mg/kg) and methylprednisolone for 28 days, and motor function was assessed to investigate the therapeutic efficacy of ginsenosides for CSM. The expression of proteins associated with TNF, IL-17, TLR4/MyD88/NF-κB, and NLRP3 signaling pathways was assessed by immunofluorescence staining and western blotting. RESULTS: Using UPLC-Q-TOF-MS, 37 compounds were identified from ginsenoside samples. Furthermore, ginsenosides-compounds-key targets-pathways-CSM visualization network indicated that ginsenosides may modulate the PI3K-Akt signaling pathway, TNF signaling pathway, MAPK signaling pathway, IL-17 signaling pathway, Toll-like receptor signaling pathway and Apoptosis by targeting AKT1, TNF, MAPK1, CASP3, IL6, and IL1B, exerting a therapeutic effect on CSM. By attenuating neuroinflammation through the TNF, IL-17, TLR4/MyD88/NF-κB, and MAPK signaling pathways, ginsenosides restored the motor function of rats with CSM, and ginsenosides 150 mg/kg showed better effect. This was achieved by reducing the phosphorylation of NF-κB and the activation of the NLRP3 inflammasome. CONCLUSIONS: The results of network pharmacology indicate that ginsenosides can inhibit neuroinflammation resulting from spinal cord compression through multiple pathways and targets. This finding was validated through in vivo tests, which demonstrated that ginsenosides can reduce neuroinflammation by inhibiting NLRP3 inflammasomes via multiple signaling pathways, additionally, it should be noted that 150 mg/kg was a relatively superior dose. This study is the first to verify the intrinsic molecular mechanism of ginsenosides in treating CSM by combining pharmacokinetics, network pharmacology, and animal experiments. The findings can provide evidence for subsequent clinical research and drug development.

Hirayama's disease associated with cervical deformity and spinal cord compression: a case report from Sweden.

BACKGROUND: Hirayama's disease (HD) is most common in young males, and previous studies are predominantly from Asian countries. The cause of HD is unknown but the most common theory about the pathology speculates on forward bending that causes a compression of the dura mater and the anterior horn of the spinal cord against the vertebra during an overstretch flexion that may result in myelopathy. Both anterior and posterior cervical surgical approaches have been shown to be effective in stopping the disease and improving function; however, HD is also reported to be a self-limited disease, and treatment with a cervical collar may be an alternative for these patients. CASE REPORT: We report HD in a 17-year-old male from Sweden who underwent surgical treatment with a 2 level anterior cervical discectomy and fusion (ACDF) due to neurological progression from HD after conservative treatment. CONCLUSION: HD is rare and is easily overlooked. Surgical intervention shows promising results for neurological progression, but HD is also reported to be a self-limited disease.

Thoracic Vertebral Canal Stenosis and Vertebral Instability in a Young Minuet Cat.

This report describes a unique case of thoracic vertebral canal stenosis and vertebral instability in a 1 yr old Minuet cat. The cat presented with a history of chronic progressive nonambulatory paraparesis. Myelography with neutral and stress positions revealed dynamic compression at T1-4. Computed tomography and MRI revealed multiple sites of vertebral endplate osteolysis, adjacent bone sclerosis, intervertebral disk space narrowing, and spondylotic bridging within the cervical and cranial thoracic vertebral bodies and pedicles, particularly at C6-T4. The cat underwent a right-sided T1-4 hemilaminectomy and C7-T4 vertebral stabilization using positively threaded profile pins and polymethylmethacrylate. The cat fully recovered without any complication. The case highlights the potential for young cats, especially those with a chondrodysplastic condition, to develop vertebral canal stenosis and vertebral instability. The surgical treatment described herein resulted in an excellent outcome.

Resting-state functional magnetic resonance imaging indices are related to electrophysiological dysfunction in degenerative cervical myelopathy.

The age-related degenerative pathologies of the cervical spinal column that comprise degenerative cervical myelopathy (DCM) cause myelopathy due spinal cord compression. Functional neurological assessment of DCM can potentially reveal the severity and pathological mechanism of DCM. However, functional assessment by conventional MRI remains difficult. This study used resting-state functional MRI (rs-fMRI) to investigate the relationship between functional connectivity (FC) strength and neurophysiological indices and examined the feasibility of functional assessment by FC for DCM. Preoperatively, 34 patients with DCM underwent rs-fMRI scans. Preoperative central motor conduction time (CMCT) reflecting motor functional disability and intraoperative somatosensory evoked potentials (SEP) reflecting sensory functional disability were recorded as electrophysiological indices of severity of the cervical spinal cord impairment. We performed seed-to-voxel FC analysis and correlation analyses between FC strength and the two electrophysiological indices. We found that FC strength between the primary motor cortex and the precuneus correlated significantly positively with CMCT, and that between the lateral part of the sensorimotor cortex and the lateral occipital cortex also showed a significantly positive correlation with SEP amplitudes. These results suggest that we can evaluate neurological and electrophysiological severity in patients with DCM by analyzing FC strengths between certain brain regions.

Spinal cord schistosomiasis in a 6-year-old child with complete recovery after spine surgery and medical treatment: case report and discussion.

Spinal cord schistosomiasis is a rare and severe form of schistosomiasis. The prognosis is largely conditioned by early diagnosis and treatment. The authors present a case of spinal cord schistosomiasis complicated by spinal cord compression syndrome. This is the case of a 6-year-old patient who presented with febrile gastroenteritis followed by complete paralysis of both lower limbs of sudden onset following a brief stay in a village setting with notion of multiple baths at a stream. Spinal cord MRI revealed an enlarged spinal cord spanning D10 to D12 with heterogeneous contrast enhancement and a syrinx cavity above the lesion. Biological workup revealed an inflammatory syndrome. Treatment consisted of decompressive laminectomy with biopsy of the lesion and a syringo-subarachnoid shunt. Pathological analysis revealed fragments of central nervous system tissues with an infiltrate composed of lymphocytes, plasmocytes, and macrophages producing granulomatous foci lined with areas of necrosis in addition to a large contingent of polynuclear eosinophils, agglutinating around or covering in some places elongated ovoid structures, with relatively thick eosinophilic shells and presenting a terminal spur. Adjuvant treatment consisted of praziquantel and corticotherapy for 1 month. The evolution showed marked improvement in the neurological deficits. She now walks unassisted and has good sphincter control. Spinal cord schistosomiasis is rare in our context; its diagnosis is difficult. The treatment is both medical and surgical.

Respiratory dysfunction in degenerative cervical myelopathy: A systematic review.

INTRODUCTION: Degenerative cervical myelopathy is a condition of symptomatic cervical spinal cord compression secondary to a range of degenerative spinal pathology. Respiratory symptoms such as shortness of breath are not uncommonly reported by people with DCM and respiratory dysfunction has been described in several DCM studies. The objective of this review was therefore to systematically synthesise the current evidence on the relationship between DCM and respiratory function. METHODS: The review was registered on PROSPERO and adhered to PRISMA guidelines. Ovid MEDLINE and Embase were searched from inception to 14th March 2023. DCM studies reporting on any measure or outcome relating to respiratory function or disease were eligible. Reference lists of included studies and relevant reviews articles were hand searched. Title, abstract and full text screening, risk of bias and GRADE assessments were completed in duplicate. A quantitative synthesis is presented. RESULTS: Of 1991 studies identified by literature searching, 13 met inclusion criteria: 3 cohort studies, 5 case-control studies, 1 case series and 4 case studies. Forced vital capacity (FVC), peak expiratory flow rate (PEFR) and maximal voluntary ventilation (MVV) were reported to be lower in DCM patients than controls; there was inconsistency in comparisons of forced expiratory volume in 1 s (FEV1). There was conflicting evidence on whether surgical decompression was associated with improvements in respiratory parameters and on the relationship between level of spinal cord compression and respiratory dysfunction. CONCLUSION: DCM may be associated with respiratory dysfunction. However, consistency and quality of evidence is currently low. Further work should characterise respiratory dysfunction in DCM patients more rigorously and investigate putative mechanisms such as disruption to cervical nerve roots responsible for diaphragmatic innervation and damage to descending spinal projections from brainstem respiratory centres.