Compressive Myelopathy Research Articles

Studies in the Falls Efficacy Scale-International for patients with cervical compressive myelopathy: Reliability, validity, and minimum clinically important difference

Context Patients with cervical compressive myelopathy (CCM) often complain of body balance problems, such as fear of falling and bodily unsteadiness. However, no accepted patient-reported outcome measures (PROMs) for this symptomatology exist. The Falls Efficacy Scale-International (FES-I) is one of the most widely used PROMs for evaluating impaired body balance in various clinical fields. Objective To examine reliability, validity, and minimum clinically important difference (MCID) of the FES-I for the evaluation of impaired body balance in patients with CCM. Methods Patients who underwent surgery for CCM were retrospectively reviewed. The FES-I was administered preoperatively and at 1 year postoperatively. Further, cJOA-LE score (subscore for lower extremities in the Japanese Orthopaedic Association score for cervical myelopathy) and stabilometric data, obtained at the same time points of the FES-I administration, were analyzed. Reliability was examined through internal consistency with Cronbach’s alpha. Convergent validity was studied using correlation analysis. The MCID was estimated using anchor- and distribution-based methods. Results Overall, 151 patients were included for analysis. Cronbach’s alpha coefficient was the acceptable value of 0.97 at both baseline and 1 year postoperatively. As for convergent validity, the FES-I had significant correlations with the cJOA-LE score and stabilometric parameters both at baseline and 1 year postoperatively. The MCID calculated using anchor- and distribution-based methods was 5.5 and 10, respectively. Conclusion FES-I is a reliable and valid PROM to evaluate body balance problems for the CCM population. The established thresholds of MCID can help clinicians recognize the clinical significance of changes in patient status.

Comparing SSEPs and MRI as Diagnostic Tools for Evaluating Compressive Myelopathies Caused by Disc Herniation

Compressive myelopathy is a neurological deficit that results from compression of the spinal cord and leads to a variety of symptoms and potentially serious consequences. Magnetic resonance imaging (MRI) is the gold standard for diagnosing compressive myelopathy as it provides high-resolution anatomical images and is the preferred method for evaluating the underlying pathology, but somatosensory evoked potentials (SSEPs) have also been used as a diagnostic tool as it provides information about the functional integrity of the spinal cord and assess the severity and extent of the damage. This article presents a comparative analysis of MRI and SSEPs in the diagnosis of compressive myelopathy and discusses the complementary role they can play in diagnosis and monitoring of the disease. Seventy four subjects were involved in the study, divided into controls (35) and cases (39) of myelopathies due to disc herniation. Severity of myelopathy for cases group were classified according to modified Japanese Assessment scale (mJOA) into; mild, moderate and severe. SSEPs showed more correlation with severity of myelopathy more than MRI (r=0.948, 0.599 respectively) and higher sensitivity of SSEPs for detecting severe myelopathies (91.7%) while MRI sensitivity was only (16.7%) sensitive.

The ferroptosis activity is associated with neurological recovery following chronic compressive spinal cord injury.

Chronic compressive spinal cord injury in compressive cervical myelopathy conditions can lead to rapid neurological deterioration in the early phase, followed by partial self-recovery, and ultimately an equilibrium state of neurological dysfunction. Ferroptosis is a crucial pathological process in many neurodegenerative diseases; however, its role in chronic compressive spinal cord injury remains unclear. In this study, we established a chronic compressive spinal cord injury rat model, which displayed its most severe behavioral and electrophysiological dysfunction at 4 weeks and partial recovery at 8 weeks after compression. Bulk RNA sequencing data identified enriched functional pathways, including ferroptosis, presynapse, and postsynaptic membrane activity at both 4 and 8 weeks following chronic compressive spinal cord injury. Transmission electron microscopy and malondialdehyde quantification assay confirmed that ferroptosis activity peaked at 4 weeks and was attenuated at 8 weeks after chronic compression. Ferroptosis activity was negatively correlated with behavioral score. Immunofluorescence, quantitative polymerase chain reaction, and western blotting showed that expression of the anti-ferroptosis molecules, glutathione peroxidase 4 (GPX4) and MAF BZIP transcription factor G (MafG), in neurons was suppressed at 4 weeks and upregulated at 8 weeks following spinal cord compression. There was a positive correlation between the expression of these two molecules, suggesting that they may work together to contribute to functional recovery following chronic compressive spinal cord injury. In conclusion, our study determined the genome-wide expression profile and ferroptosis activity of a consistently compressed spinal cord at different time points. The results showed that anti-ferroptosis genes, specifically GPX4 and MafG, may be involved in spontaneous neurological recovery at 8 weeks of chronic compressive spinal cord injury. These findings contribute to a better understanding of the mechanisms underlying chronic compressive spinal cord injury and may help identify new therapeutic targets for compressive cervical myelopathy.

Robotic rehabilitation therapy using Hybrid Assistive Limb (HAL) for patients with spinal cord lesions: a narrative review

The Hybrid Assistive Limb (HAL) is a rehabilitation device that utilizes the "interactive biofeedback" hypothesis to facilitate the motion of the device according to the user's motion intention and appropriate sensory input evoked by HAL-supported motion. HAL has been studied extensively for its potential to promote walking function in patients with spinal cord lesions, including spinal cord injury. We performed a narrative review of HAL rehabilitation for spinal cord lesions. Several reports have shown the effectiveness of HAL rehabilitation in the recovery of walking ability in patients with gait disturbance caused by compressive myelopathy. Clinical studies have also demonstrated potential mechanisms of action leading to clinical findings, including normalization of cortical excitability, improvement of muscle synergy, attenuation of difficulties in voluntarily initiating joint movement, and gait coordination changes. However, further investigation with more sophisticated study designs is necessary to prove the true efficacy of HAL walking rehabilitation. HAL remains one of the most promising rehabilitation devices for promoting walking function in patients with spinal cord lesions.

Differentiation Between Compressive Cervical and Thoracic Myelopathy Using the Central Motor Conduction Time Ratio.

Thoracic myelopathy is a rare condition whose diagnosis is often missed or delayed. This study aimed to differentiate between cervical and thoracic myelopathy using motor-evoked potential testing. The authors included 835 patients with compressive cervical myelopathy and 94 patients with compressive thoracic myelopathy. Myelopathy using motor-evoked potentials were recorded from the bilateral abductor digiti minimi and abductor hallucis muscles through transcranial magnetic stimulation. The peripheral conduction time was measured through electrical stimulation of the ulnar and tibial nerves; moreover, the central motor conduction time (CMCT) was calculated by subtracting the peripheral conduction time from the myelopathy using motor-evoked potential latency. The most accurate differentiation between compressive cervical myelopathy and compressive thoracic myelopathy was achieved by the CMCT ratios (CMCT-ADM:CMCT-AH; cutoff value of 0.490, sensitivity of 83.0%, and specificity of 80.5%). After excluding patients with compressive cervical myelopathy who had spinal cord compression at C6-7, the cutoff value was 0.490, with a sensitivity of 83.0% and specificity of 87.3%. Determining the CMCT ratio (cutoff value of 0.490) through motor-evoked potential testing could facilitate differentiation between compressive cervical myelopathy and compressive thoracic myelopathy.

Acute Thoracic Compressive Myelopathy as a Rare Manifestation of Thalassemia: A Case Report

Acute Thoracic Compressive Myelopathy as a Rare Manifestation of Thalassemia: A Case Report

Cerebrospinal fluid and serum proteomic profiles accurately distinguish neuroaxonal dystrophy from cervical vertebral compressive myelopathy in horses

Cervical vertebral compressive myelopathy (CVCM) and equine neuroaxonal dystrophy/degenerative myeloencephalopathy (eNAD/EDM) are leading causes of spinal ataxia in horses. The conditions can be difficult to differentiate, and there is currently no diagnostic modality that offers a definitive antemortem diagnosis. Evaluate novel proteomic techniques and machine learning algorithms to predict biomarkers that can aid in the antemortem diagnosis of noninfectious spinal ataxia in horses. Banked serum and cerebrospinal fluid (CSF) samples from necropsy-confirmed adult eNAD/EDM (n = 47) and CVCM (n = 25) horses and neurologically normal adult horses (n = 45). . A subset of serum and CSF samples from eNAD/EDM (n=5) and normal (n=5) horses was used to evaluate the proximity extension assay (PEA). All samples were assayed by PEA for 368 neurologically relevant proteins. Data were analyzed using machine learning algorithms to define potential diagnostic biomarkers. Of the 368 proteins, 84 were detected in CSF and 146 in serum. Eighteen of 84 proteins in CSF and 30/146 in serum were differentially abundant among the 3 groups, after correction for multiple testing. Modeling indicated that a 2-protein test using CSF had the highest accuracy for discriminating among all 3 groups. Cerebrospinal fluid R-spondin 1 (RSPO1) and neurofilament-light (NEFL), in parallel, predicted normal horses with an accuracy of 87.18%, CVCM with 84.62%, and eNAD/EDM with 73.5%. Cross-species platform. Uneven sample size. Proximity extension assay technology allows for rapid screening of equine biologic matrices for potential protein biomarkers. Machine learning analysis allows for unbiased selection of highly accurate biomarkers from high-dimensional data.

Brain and craniovertebral junction in patients with achondroplasia using low dose dynamic computed tomography

Purpose: To describe the brain and craniovertebral junction (CVJ) in achondroplasia by dynamic low dose computed tomography (CT). Methods: This paper documents a cross-sectional retrospective study performed between 1998 and 2013. The Ethics and Research Committee approved the project. The investigators reviewed the medical records of 440 patients with skeletal dysplasia and performed flexion-extension CT for 24 patients with achondroplasia confirmed by molecular testing. They were divided in two groups: children younger than eight years old and group 2 with older individuals. Normal scans were selected as controls in proportion 1:4. Three measurements were made by two radiologists using the following parameters: Evans Ratio, cerebroventricular index of the anterior horns and maximum width of the third ventricle; diameters of the foramen magnum (FM), width of the spinal canal, clivus size, basion-axial interval, atlantooccipital interval and Powers ratio, atlantodental interval, distance of the dens tip position above McGregor line and Welcher angle. T-test, Kruskal-Wallis and Fisher statistics were used. P-value<0.05 threshold was considered for statistical significance. Results: The ventricles were greater, and the FM was smaller in achondroplastic patients than in controls scans (p<0.01). Other findings were: ventriculomegaly (30%), hydrocephalus (4%), narrowing of FM (between 96% and 100%), spinal stenosis (92%), clivus hypoplasia (88%), atlantooccipital instability (25%), atlantoaxial instability (13 %), basilar invagination (13%) and platybasia (8%). There was no upper cervical compressive myelopathy. There was no difference of CVJ changes between the groups 1 and 2 (p>0.09). Conclusion: The craniometry showed the most frequent cerebral and CVJ changes in achondroplasia.

Floating Laminoplasty for the Treatment of Compressive Myelopathy Accompanied by C1 Posterior Ring Hypoplasia: A Technical Note and Case Series

Posterior ring hypoplasia of the atlas (C1) is not common and in most cases is found by chance without symptoms. This disorder is sometimes accompanied by a mild C1-C2 instability or the presence of a space-occupying lesion such as ossification of posterior longitudinal ligament, pannus, or retro-odontoid pseudo-tumor, which are indications for surgery if symptomatic cord compression is present. A C1 posterior arch resection has been the procedure of choice but is associated with several fatal complications. We here present a novel posterior arch decompression technique that is relatively safer and easier and that involves mobilization and posterior translation of the posterior arch as a whole, similar to a vertebral body sliding osteotomy.

A Longer Duration of Myelopathy Symptoms is Associated With the Lack of Intraoperative Motor Evoked Potential Improvement During Decompressive Cervical Myelopathy Surgery.

Retrospective cohort study. We aim to investigate the relationship between the intraoperative motor evoked potential (MEP) signal changes during surgical treatment of cervical myelopathy with postoperative functional outcomes and determine what factors correlate with MEP signal changes. Intraoperative neurophysiologic monitoring with MEP for cervical cord decompression can potentially predict postoperative neurological complications. We prospectively collected data from 114 consecutive cervical compressive myelopathy patients who underwent decompressive cervical spine surgery. Functional outcomes were measured preoperatively and postoperatively at the 6-month mark, using the modified Japanese Orthopedic Association score. Among the 114 patients, 87 patients showed significant MEP improvement, 1 patient with MEP degeneration, 3 patients with no change in MEP, and 23 patients with MEP change, but which eventually returned to baseline. Univariate analysis showed that patients with MEP improvement had similar 6-month functional and Japanese Orthopedic Association scores compared with patients who did not have MEP improvement. Critically, a longer duration of symptoms was shown to have a statistically significant relationship with patients who did not have MEP improvement on univariate analysis (49.2wk in patients with no MEP improvement compared with 34.59wk in patients with MEP improvement, P = 0.03) but this did not translate to differences in functional outcomes. There was also no statistically significant association between the functional outcome scores and demographics, surgical, or radiologic factors. Our study shows that the duration of symptoms is not attributed to lower functional outcomes but is associated with a lack of MEP improvement. Level III.

Are Functional Gains Durable After Decompressive Surgery for Cervical Myelopathy?: Commentary on an article by Victor Hin Ting Yick, MBBS, et al.: "Neurological Survivorship Following Surgery for Degenerative Cervical Myelopathy. A Longitudinal Study on 195 Patients".

Commentary In degenerative cervical myelopathy, spine surgeons generally accept the concept that patients will show clinically meaningful improvement after spinal cord decompression. At a minimum, the spine literature and common sense support the notion that cord decompression will at least halt ongoing neurological deterioration. Surgeons may debate the best method of decompression (anterior or posterior) and whether decompression with fusion is necessary, but there is little dissension with regard to the need for a surgical procedure in the setting of moderate to severe myelopathy1. The extent to which early neurological improvement after the surgical treatment of degenerative cervical myelopathy is sustained in the long term is unknown. Using the concept of neurological survivorship and Kaplan-Meier curves, Yick et al. demonstrate that, although most patients improve after decompressive surgery, not all of these good early results are durable. The authors found that as many as 17.9% of patients will see a recurrent decline in neurological function by 10 years, and most of these patients who underwent failed treatment (74.3%) underwent a revision surgical procedure. Given the published high reoperation rates in cervical spine surgery, these results in degenerative cervical myelopathy are not particularly surprising. In a recent minimum 10-year follow-up of anterior cervical fusion in 159 patients, Buttermann reported a 31% reoperation rate2. In the current study, Yick et al. confirm what smaller, short-term studies have suggested: improvement after decompression for degenerative cervical myelopathy is fleeting in many patients and reoperation is common. Such knowledge is valuable when counseling patients both before and after the surgical procedure. In addition to describing the neurological survivorship of these patients, Yick et al. drilled down on the risk factors for failure. Some of these significant risk factors are patient-specific and are not controllable by the surgeon. These factors include renal failure and spinal cord signal change on T2-weighted magnetic resonance imaging sequences. A cord signal change, associated with cellular changes in the cord secondary to severe cord compression, is often irreversible3 and, as seen in the current study, is associated with ongoing declines in neurological function. Other significant risk factors are modifiable. Surgeons can use these factors to modify their surgical management of cervical myelopathy and, hopefully, improve future neurological survivorship. First, and most importantly, surgeons should not be performing suture laminoplasty. Patients treated with this technique had the worst neurological survivorship (31.2%) and tended to experience treatment failure early after the surgical procedure. Second, patients with ossification of the posterior longitudinal ligament did best when decompression was combined with fusion. Surgeons should give strong consideration to including fusion in patients with ossification of the posterior longitudinal ligament to improve outcomes. Conversely, surgeons might consider avoidance of fusion in younger patients with cervical compressive myelopathy. The top risk factor for late neurological decline was adjacent-level stenosis, proximal or distal to a fusion construct. In these patients, perhaps a posterior decompression without fusion or a laminoplasty with plating will result in better neurological survivorship. Yick et al. should be commended for their efforts to publish longer-term neurological outcomes for this complex group of patients. The strengths of the study include a large cohort of 195 patients and a mean follow-up of 76 months. The weaknesses of the study include a retrospective design with many patients (49) excluded because of incomplete follow-up. Furthermore, many of the included patients (37) had a follow-up period shorter than 2 years. Nevertheless, Yick et al. identify important predictive factors for neurological survivorship after surgical treatment of degenerative cervical myelopathy and give surgeons specific pearls to help with surgical planning. A long-term prospective study with a minimum 10-year follow-up is still necessary to define optimal surgical approaches for degenerative cervical myelopathy.

Natural history of pituitary carcinoma with metastasis to the cervical spine: illustrative case

Pituitary carcinoma is a rare tumor of the adenohypophysis with noncontiguous craniospinal dissemination and/or systemic metastases. Given the rarity of this malignancy, there is limited knowledge and consensus regarding its natural history, prognosis, and optimal treatment. The authors present the case of a 46-year-old woman initially treated with invasive prolactin-secreting pituitary macroadenoma who developed metastatic disease of the cervical spine 6 years later. The patient presented with acutely worsening compressive cervical myelopathy and required posterior cervical decompression, tumor resection, and instrumented arthrodesis for posterolateral fusion. This case underscores the importance of long-term monitoring of hormone levels and having a high clinical suspicion for metastatic disease to the spine in patients presenting with acute myelopathy or radiculopathy in the setting of previously treated invasive secreting pituitary adenoma.

Compressive Myelopathy, Spinal Shock, and a Complicated Neurological Exam

Compressive Myelopathy, Spinal Shock, and a Complicated Neurological Exam

Bladder and Bowel Dysfunction in Patients with Spinal Compressive Myelopathy

Bladder and Bowel Dysfunction in Patients with Spinal Compressive Myelopathy

Post-Traumatic holocord syrinx – A case report and review of literature

Posttraumatic holocord syrinx is a rare entity with only few cases reported in the literature. We hereby discuss one such case of posttraumatic holocord syrinx presented to us with features of compressive myelopathy and bladder dysfunction, 7 years after sustaining a traumatic D12 fracture which was managed with stabilization. A syringopleural shunt was done for the patient and significant clinico-radiological improvement was noted.

PATTERN RECOGNITION ,APPROACH AND CLINICO-RADIOLOGIC EVALUATION OF MYELOPATHIES

INTRODUCTION Myelopathy, the rapid or insidious onset of motor and sensory abnormalities referable to the spinal cord, occurs as a result of a variety of causes that may be classied on the basis of their location of origin (intramedullary, intradural-extramedullary, and extradural). Osteophytic spurring and disc herniation may also produce myelopathy localised to the thoracic spine, though less commonly. Degenerative cervical myelopathy (DCM) is a progressive spine disease and the most common cause of spinal cord dysfunction in adults worldwide. AIM OF THE STUDY: 1. To study the clinical prole of cases of Myelopathy 2. To study the pattern of spinal cord involvement 3. To compare the clinical diagnosis with MRI diagnosis MATERIALS AND METHODS Ÿ Study setting: The study was conducted on patients admitted in the departments of neurology and neurosurgery with clinical suspicion of spinal cord disease Ÿ Study design: The current study was a prospective stud Ÿ Study period: The study was conducted between august 2021 to July 2022 Ÿ Sample size: The present prospective study included 30 patients admitted in the departments of neurology and neurosurgery with clinical suspicion of spinal cord disease. INCLUSION CRITERIA: Inclusion criteria include patients with clinical suspicion of spinal cord disease of age group 20-80 years were included as study participants in the present study. EXCLUSION CRITERIA: The current study excluded patients with seizures, abnormal higher mental functions, and children. RESULTS Ÿ In the present series, the patient's age ranged from 20 years to 80 years Ÿ The majority of 53.33% of participants were aged between 51 to 60 years, followed by 61 to 70 years was 13.33%, 21 to 30 years and 41 to 50 was 10% for each Ÿ Distribution of cases according to etiology: Among the study population, 17 (57%) participants had cervical spondylosis, 4 (13%) participants had caries spine, 3 (10%) participants had a vertebral fracture and others for each, 2 (7%) participants had uorosis and only 1 (3%) participant had spinal tumor Ÿ Clinico-radiological patterns of spinal cord syndromes: The majority of 50% of participants had Anterior + Posterior Cord involvement. The proportion of posterior cord, pan cord, and the central cord was 20%, 13.33%, and 10% respectively CONCLUSION: Ÿ The present study results have analyzed the prole of myelopathies and found more male preponderance Ÿ The commonest cause of compressive myelopathy was Cervical spondylosis Ÿ Anterior plus posterior cord syndrome was the commonest pattern seen which was followed by posterior cord syndrome and the least observed pattern was anterior cord syndrome. Hence, it can be concluded that MRI could suggest the location of the lesion and correlated well with clinical diagnosis.

Extensive Neuromusculoskeletal Complications—Including Multi-Level Vertebral Osteomyelitis, Myelopathy, Polyradiculopathy, and Multiple Muscle Abscesses—After Lumbar Transforaminal Epidural Block: A Case Report

Vertebral osteomyelitis (VO) is a rare, slowly progressing disease that often causes neuromuscular complications. Epidural block is a non-surgical treatment used in patients with radicular pain to deliver drugs to the epidural space; it is generally known to be a safe method, but it can occasionally cause infection. Herein, we present a rare case of VO with severe neuromuscular complications that developed in a patient who underwent lumbar transforaminal epidural block for back pain. Imaging studies showed VO, multiple pyogenic abscesses, and compressive cervical myelopathy. Electrodiagnostic studies showed clear evidence of cervical myelopathy and polyradiculopathy. With early treatment using a multidisciplinary approach, including medical treatment, surgery, and comprehensive rehabilitation, recovery of motor weakness and functional improvement were achieved after 2 months of treatment. Electrodiagnostic studies are advantageous for localizing and determining the degree of neuromuscular damage following VO. A multidisciplinary approach to the diagnosis and treatment of VO could improve patients’ prognosis, functional ability, and quality of life.

ABORDAGEM FISIOTERAPÊUTICA NA MIELOPATIA COMPRESSIVA SECUNDÁRIA À AMILOIDOSE: RELATO DE CASO

Myelopathy is an umbrella term used to refer to disorders that affect the spinal cord. There are several mechanisms that can cause it, however, secondary spinal cord compression, amyloidosis, is one of the rarest forms. The aim of the present study is to report the physiotherapy approach in compressive myelopathy secondary to amyloidosis. This is a descriptive study with a mixed approach, of the case report type, carried out during the supervised internship of neurofunctional physiotherapy, of the graduation course in physiotherapy, at the Community University of the Chapecó Region (UNOCHAPECÓ). Data collection was carried out through the neurofunctional assessment script and specific scales on the patient's case, a study carried out from May to July 2022, totaling nine visits, between assessment, treatment and reassessment. The patient under study is 55 years old, male, with a diagnosis of compressive myelopathy secondary to amyloidosis. It was possible to observe objective improvement through the Romberg and Timed Get Up And Go tests in the variables of balance and risk of falls, and subjective improvements, through the patient's report in relation to psychological aspects such as a feeling of well-being and disposition after the consultations. . It was noted that the physiotherapeutic approach adopted in this patient resulted in an improvement in the functional framework and brought psychological benefits such as a feeling of well being and disposition after the consultations. In addition, it made it possible to better understand and understand a rare condition with few reports in the literature.

Amyloidoma Presenting as Compressive Myelopathy.

Spinal cord compression is a common problem in clinical practice. Sometimes puzzling symptoms may mislead the clinician in the initial days. We present a 59 year old lady who presented with progressive numbness of both feet and abdomen, walking difficulties for which the initial evaluation did not yield any result. The work up at our facility showed collapsed D3 vertebra, prevertebral collection and cord compression. She was tentatively started on antituberculous treatment, based on the radiologist opinion. Two months later, the patient had further progression of symptoms and had to undergo D3 laminectomy and surgical decompression. She also developed hypertension and diabetes in the meantime. Histopathology did not favour tuberculous process, but showed amyloid deposition. Antituberculous treatment was halted and she was evaluated for plasma cell dyscrasias. The serum protein electrophoresis with immunofixation was in favour of IgG Kappa light chain monoclonal gammopathy. She was further subjected to bone marrow aspiration and biopsy at a Cancer institute. The result was negative for plasma cell malignancy, and was suggestive of only reactive lymphocytosis. On the advice of oncologist, antituberculous treatment was started again and continued as RH- two drug regimen for 9 months along with supportive care. She improved symptomatically, able to walk independently and attended regular follow up. Three years later, a repeat MRI scan showed enlargement of the lesion to involve the posterior mediastinum. The oncology service decided to irradiate the lesion, only if she develops new symptoms. This outcome suggests the unusual presentation of the disease, and the so litary amyloidoma is probably a primary disease in itself or secondary to a neoplastic process.

Cranio-cervical decompression associated with non-instrumented occipito-C2 fusion in children with mucopolysaccharidoses: Report of twenty-one cases

BackgroundMucopolysaccharidosis (MPS) is a multisystemic storage disorder of glycosaminoglycan deposits. Infiltration of the dura mater and supporting ligaments caused spinal cord compression and consecutive myelopathy, especially at the cranio-cervical junction (CCJ). Craniocervical instability and posterior decompression often raise the problem of fixation in children. The main purpose of this paper was to report the result of an original technique of occipito-cervical arthrodesis using a cranial halo-cast system in pediatric population. MethodsWe recorded 21 patients with cervical myelopathy. All of them had spinal cord decompression by enlargement of the foramen magnum, C1 laminectomy, and occipito-C2 fusion using corticocancellous bone graft. Only one child has an extended laminectomy from C1 to C3. The occiput-C2 arthrodesis was stabilized by the cranial halo-cast system. This immobilization was performed preoperatively and kept for three months then switched to rigid cervical collar. Clinical assessment, including the Goel grade and mJOA, radiographs and magnetic resonance imaging were performed before surgery. The occipito-cervical arthrodesis was controlled by standard X-rays and CT scan. ResultsAccording to the type of mucopolysaccharidosis, the patients were divided into MPS type I: n= 3, II: n=7, IV: n=11. The mean age of patients at surgery was 6.76 years. All mucopolysaccharidoses cases required a foramen magnum decompression by craniectomy, C1 laminectomy and occipito-C2 arthrodesis. As major complications, a child had immediate post-operative paraplegia due to spinal cord ischemia. The postoperative follow-up ranged from 1.5 to 4 years, with an average of 3.3 years. The average preoperative mJOA score was 8.9, and it improved to 14 points at the last follow-up. ConclusionsSatisfactory fusion and good clinical results were obtained with the 2-stage approach to CCJ anomalies.