Biportal endoscopic cervical open-door laminoplasty to treat cervical spondylotic myelopathy.

BACKGROUND: Although cervical laminoplasty is a frequently utilized surgical intervention for cervical spondylotic myelopathy, it is primarily performed using conventional open surgical techniques. We attempted the minimally invasive cervical laminoplasty using biportal endoscopic approach. METHODS: Contralateral lamina access is facilitated by creating space through spinous process drilling, followed by lamina hinge formation. Subsequently, the incised lamina is elevated from ipsilateral aspect, and secure metal plate fixation is performed. CONCLUSION: We successfully performed the cervical open door laminoplasty using biportal endoscopic approach. Biportal endoscopic cervical open-door laminoplasty may be a minimally invasive technique that can prevent complications related with open surgery.

Full endoscopic surgery for calcium pyrophosphate deposition disease (CPPD) in the cervical ligamentum flavum: report of two cervical myelopathy cases.

Calcium pyrophosphate deposition disease (CPPD), known as pseudogout, is characterized by the accumulation of calcium pyrophosphate crystals in musculoskeletal structures, primarily joints. While CPPD commonly affects various joints, involvement in the cervical spine leading to myelopathy is rare. Surgical intervention becomes necessary when conservative measures fail, but reports on full endoscopic surgeries are extremely rare. We present two successful cases where full endoscopic systems were used for CPPD removal in the cervical spine. The surgical technique involved a full endoscopic approach, adapting the previously reported technique for unilateral laminotomy bilateral decompression. Full-endoscopic removal of cervical CPPD inducing myelopathy were successfully removed with good clinical and radiologic outcomes. The scarcity of endoscopic cases for cervical ligamentum flavum CPPD is attributed to the condition's rarity. However, our successful cases advocate for endoscopic surgery as a potential primary treatment option for CPPD-induced cervical myelopathy, especially in elderly patients or those with previous cervical operation histories. This experience encourages the consideration of endoscopic surgery for managing cervical ligamentum flavum CPPD as a viable alternative.

Single-cell RNA sequencing reveals peripheral blood leukocyte responses to spinal cord injury in mice with humanised immune systems.

Next-generation humanised mouse models and single-cell RNA sequencing (scRNAseq) approaches enable in-depth studies into human immune cell biology. Here we used NSG-SGM3 mice engrafted with human umbilical cord haematopoietic stem cells to investigate how human immune cells respond to and/or are changed by traumatic spinal cord injury (SCI). We hypothesised that the use of such mice could help advance our understanding of spinal cord injury-induced immune depression syndrome (SCI-IDS), and also how human leukocytes change as they migrate from the circulation into the lesion site. Our scRNAseq experiments, supplemented by flow cytometry, demonstrate the existence of up to 11 human immune cell (sub-) types and/or states across the blood and injured spinal cord (7 days post-SCI) of humanised NSG-SGM3 mice. Further comparisons of human immune cell transcriptomes between naïve, sham-operated and SCI mice identified a total of 579 differentially expressed genes, 190 of which were 'SCI-specific' (that is, genes regulated only in response to SCI but not sham surgery). Gene ontology analysis showed a prominent downregulation of immune cell function under SCI conditions, including for T cell receptor signalling and antigen presentation, confirming the presence of SCI-IDS and the transcriptional signature of human leukocytes in association with this phenomenon. We also highlight the activating influence of the local spinal cord lesion microenvironment by comparing the transcriptomes of circulating versus infiltrated human immune cells; those isolated from the lesion site were enriched for genes relating to both immune cell activity and function (e.g., oxidative phosphorylation, T cell proliferation and antigen presentation). We lastly applied an integrated bioinformatics approach to determine where immune responses in humanised NSG-SGM3 mice appear congruent to the native responses of human SCI patients, and where they diverge. Collectively, our study provides a valuable resource and methodological framework for the use of these mice in translational research.

Biportal endoscopic transforaminal thoracic interbody fusion for the treatment of thoracic myelopathy.

BACKGROUND: Biportal endoscopic spine surgery independently controls two hands, similar to microscopic surgery, and utilizes a broader working space that is not disturbed by retractors under clear-magnified endoscopic vision. These advantages facilitate successful neural decompression and safe transforaminal interbody fusion, even in patients with thoracic spondylotic myelopathy. METHODS: A wide laminectomy and precise total facetectomy, in conjunction with partial pediculotomy, establish a secure transforaminal space for cage insertion. Endplate preparation and cage insertion were performed without retracting the spinal cord under direct endoscopic vision. CONCLUSION: Biportal endoscopic transforaminal thoracic interbody fusion can be a feasible technique for treating thoracic spondylotic myelopathy at the thoracolumbar junction levels.

Paraneoplastic/autoimmune myelopathies.

Paraneoplastic myelopathies are a rare but important category of myelopathy. They usually present with an insidious or subacute progressive neurologic syndrome. Risk factors include tobacco use and family history of cancer. Cerebrospinal fluid analysis usually shows lymphocytic pleocytosis with elevated protein. MRI findings suggest that paraneoplastic myelopathies include longitudinally extensive T2 hyperintensities that are tract-specific and accompanied by enhancement, but spinal MRIs can also be normal. The most commonly associated neural antibodies include amphiphysin and collapsin-response-mediator-protein-5 (CRMP5/anti-CV2) antibodies with lung and breast cancers being the most frequent oncologic accompaniments. The differential diagnosis of paraneoplastic myelopathies includes nutritional deficiency myelopathy (B12, copper) as well as autoimmune/inflammatory conditions such as primary progressive multiple sclerosis or spinal cord sarcoidosis. Patients treated with immune checkpoint inhibitors for cancer may develop myelitis, that can be considered along the spectrum of paraneoplastic myelopathies. Management of paraneoplastic myelopathy includes oncologic treatment and immunotherapy. Despite these treatments, the prognosis is poor and the majority of patients eventually become wheelchair-dependent.

Vertebral and spinal malformations in small brachycephalic dog breeds: Current knowledge and remaining questions.

Small brachycephalic dog breeds, such as the French bulldog, English bulldog and pug have become increasingly popular. These breeds are predisposed to a variety of vertebral and spinal malformations, including hemivertebra, caudal articular process dysplasia, transitional vertebra, cranial thoracic vertebral canal stenosis, spinal arachnoid diverticulum and meningeal fibrosis. Recent studies have provided new insights into the prevalence, anatomical characteristics, pathophysiology and treatment of these conditions. Thoracic hemivertebra, caudal articular process dysplasia, transitional vertebra, and cranial thoracic vertebral canal stenosis occur commonly in neurologically normal dogs. Although the clinical relevance of these vertebral anomalies has therefore been questioned, severe kyphosis and hemivertebra in pugs have been associated with an increased likelihood of neurological signs. Meningeal fibrosis is characterised by the formation of dense intradural fibrotic adhesions, constricting the spinal cord. This condition has been heavily associated with the pug breed. It is in pugs further common to observe multiple concurrent spinal disorder in association with chronic progressive pelvic limb gait abnormalities. This clinical presentation has been referred to as 'pug dog thoracolumbar myelopathy' and potential genetic risk factors have recently been identified. Despite our increased knowledge, many questions remain currently unanswered. This review discusses our current understanding and controversies surrounding vertebral and spinal malformations in small brachycephalic dog breeds.

An infratentorial dural arteriovenous fistula mimicking cervical myelopathy: A case report.

Dural arteriovenous fistulas may have insidious clinical presentations and are often challenging to diagnose. A small number of cases have been associated with perimedullary venous congestion and cord oedema, mimicking common pathologies, such as cervical myelopathy. We describe a case report of a patient presenting with a constellation of symptoms and radiological signs mimicking C5/6 cervical myelopathy secondary to disc herniation. The patient was managed with anterior cervical discectomy and fusion, with postoperative neurological deterioration unresponsive to steroid therapy. This prompted further investigation of other pathologies. An infratentorial Cognard 5 and Borden type 3 dural arteriovenous fistula was diagnosed on 6-vessel DSA and managed with onyx embolization. Marked improvement of neurological symptoms, notably bilateral lower limb weakness, was achieved postoperatively. In summary, this case demonstrates the importance of considering alternative, less common pathologies that involve the cervical spinal cord when neurological improvement is not achieved following decompressive surgery for cervical myelopathy.

People with degenerative cervical myelopathy have impaired reactive balance during walking.

BACKGROUND: People with degenerative cervical myelopathy are known to have impaired standing balance and walking abilities, but less is known about balance responses during walking. RESEARCH QUESTION: The aim of this project was to assess reactive balance impairments during walking in people with degenerative cervical myelopathy (PwDCM). We hypothesized that center of mass motion following perturbations would be larger in PwDCM and gluteus medius electromyographic amplitude responses would be decreased in PwDCM. METHODS: Reactive balance responses were quantified during unanticipated lateral pulls to the waist while treadmill walking. Walking biomechanics data were collected from 10 PwDCM (F=6) and 10 non-myelopathic controls (F=7) using an 8 camera Vicon System (Vicon MX T-Series). Electromyography was collected from lower limb muscles. Participants walked on an instrumented treadmill and received lateral pulls at random intervals and in randomized direction at 5% and 2.5% body mass. Participants walked at 3 prescribed foot placements to control for effects of the size of base of support. RESULTS: As compared with controls, the perturbation-related positional change of the center of mass motion (ΔCOM) was increased in PwDCM (p=0.001) with similar changes in foot placement (p>0.05). Change in gluteus medius electromyography, however, was less in PwDCM than in controls (p<0.001). SIGNIFICANCE: After experimentally controlling step width, people with mild-to-moderate degenerative cervical myelopathy at least 3 months following cervical spine surgery have impaired reactive balance during walking likely coupled with reduced gluteus medius electromyographic responses. Rehabilitation programs focusing on reactive balance and power are likely necessary for this population.

Biomechanical Comparison of Anterior Cervical Corpectomy Decompression and Fusion, Anterior Cervical Discectomy and Fusion, and Anterior Controllable Antedisplacement and Fusion in the Surgical Treatment of Multilevel Cervical Spondylotic Myelopathy: A Finite Element Analysis.

PURPOSE: Multilevel cervical spondylotic myelopathy poses significant challenges in selecting optimal surgical approaches, warranting a comprehensive understanding of their biomechanical impacts. Given the lack of consensus regarding the most effective technique, this study aims to fill this critical knowledge gap by rigorously assessing and comparing the biomechanical properties of three distinct surgical interventions, including anterior controllable antedisplacement and fusion (ACAF), anterior cervical corpectomy decompression and fusion (ACCF), and anterior cervical discectomy and fusion (ACDF). The study offers pivotal insights to enhance treatment precision and patient outcomes. METHODS: The construction of the cervical spine model involved a detailed process using CT data, specialized software (Mimics, Geomagic Studio, and Hypermesh) and material properties obtained from prior studies. Surgical instruments were modeled (titanium mesh, anterior cervical plate, interbody cage, and self-tapping screws) to simulate three surgical approaches: ACAF, ACCF, and ACDF, each with specific procedures replicating clinical protocols. A 75-N follower load with 2 Nm was applied to simulate biomechanical effects. RESULTS: The range of motion decreased more after surgery for ACAF and ACDF than for ACCF, especially in flexion and lateral bending. ACCF have higher stress peaks in the fixation system than those of ACAF and ACDF, especially in flexion. The maximum von Mises stresses of the bone-screw interfaces at C3 of ACCF were higher than those of ACAF and ACDF. The maximum von Mises stresses of the bone-screw interfaces at C6 of ACDF were much higher than those of ACAF and ACCF. The maximum von Mises stresses of the grafts of ACCF and ACAF were much higher than those of ACDF. The maximum von Mises stresses of the endplate of ACCF were much higher than those of ACAF and ACDF. CONCLUSION: The ACAF and ACDF models demonstrated superior cervical reconstruction stability over the ACCF model. ACAF exhibited lower risks of internal fixation failure and cage subsidence compared to ACCF, making it a promising approach. However, while ACAF revealed improved stability over ACCF, higher rates of subsidence and internal fixation failure persisted compared to ACDF, suggesting the need for further exploration of ACAF's long-term efficacy and potential improvements in clinical outcomes.

Prospective Investigation of the Posterior Longitudinal Ligament and Disc Complex after Posterior Stabilization and Decompression Surgery in Patients With Non-OPLL (Ossification of the Posterior Longitudinal Ligament) Cervical Spondylotic Myelopathy.

BACKGROUND: Cervical spondylotic myelopathy (CSM) is a prevalent cause of spinal cord dysfunction in adults, primarily from degenerative changes. The efficacy of treatment strategies, especially surgical approaches, remains debated. OBJECTIVE: This study aimed to assess the long-term impact of posterior fusion laminectomy on the posterior longitudinal ligament (PLL) thickness, disc complex, and myelomalacia signal changes in CSM patients. METHODS: A single-centre, prospective study from January 2020 to December 2021 included CSM patients without ossified posterior longitudinal ligament (OPLL). Magnetic resonance imaging (MRI) data from baseline, 6, and 12 months postoperatively were collected. Measurements on the MRI were performed using the Osirix MD software, focusing on the PLL width, myelopathic foci dimensions, and canal diameter. RESULTS: Out of the 82 initially enrolled patients, 64 were considered for analysis. Postoperatively, a significant reduction in PLL width and myelopathic foci dimensions was observed, alongside a considerable increase in the canal diameter. Clinical outcomes based on the Modified Japanese Orthopaedic Association (mJOA) scale also showcased marked improvements post-surgery. CONCLUSIONS: Posterior fusion laminectomy effectively reduces anterior pressure in CSM patients. This treatment may represent an optimal surgical approach for selected CSM cases. Furthermore, more extensive studies with extended follow-up are advocated.

Disseminated Tuberculosis Revealed by Conus Medullaris Tuberculoma.

A 29-year-old man from Comoros presented with rapidly progressive paraplegia and sexual dysfunction. Magnetic resonance imaging (MRI) showed a contrast-enhanced conus medullaris lesion. Differential diagnoses included tumors, abscesses, and inflammatory diseases. Neurosurgery was delayed to complete examinations. Cerebral MRI showed three abscesses. Body computed tomography scan showed supracentimetric polyadenopathies, pulmonary nodules, prostatic lesion, and enhanced seminal vesicle, with hypermetabolism on positron emission tomography with 2-deoxy-2-[fluorine-18]fluoro-D-glucose scan. Histology of lymph node biopsy showed granulomatous infiltration without acid-fast bacilli, and positive polymerase chain reaction for Mycobacterium tuberculosis. Lymph node culture was positive after 2 months, urine culture after 3 weeks, but cerebrospinal fluid and sputum cultures were negative. A 1-year antituberculosis therapy was initiated, associated with corticosteroids because the patient developed tuberculosis-immune reconstitution syndrome, revealed by the recurrence of neurological symptoms. After 2 months the patient completely recovered and could run. MRI showed stability of the voluminous tuberculoma with decrease of medullary edema. Avoiding surgery in those cases may prevent iatrogenic neurological deterioration.

How I do it: angiography-assisted full endoscopic treatment of spinal dural arteriovenous fistula.

BACKGROUND: Spinal dural arteriovenous fistula (sDAVF) is a rare vascular malformation that leads to serious neurological symptoms. We treat a 52-year-old man with sDAVF in the thoracic segment exhibiting uncoordinated gait. METHOD: Thoracic MRI of the lesion indicated myelomalacia and dilated blood vessels, while DSA revealed the right T6 radicular artery as the feeding arteriole. A full endoscopic obliteration of the lesion was performed under angiography guidance in a hybrid operation room. CONCLUSION: The case underscores the importance of a multidisciplinary and individualized approach to successfully manage sDAVF using a fully endoscopic approach.

Free-hand technique of C7 pedicle screw insertion using a simply defined entry point without fluoroscopic guidance for cervical spondylotic myelopathy patients with C3 to C6 instrumented by lateral mass screws: a retrospective cohort study.

BACKGROUND: Nowadays, both lateral mass screw (LMS) and pedicle screw were effective instrumentation for posterior stabilization of cervical spine. This study aims to evaluate the feasibility of a new free-hand technique of C7 pedicle screw insertion without fluoroscopic guidance for cervical spondylotic myelopathy (CSM) patients with C3 to C6 instrumented by lateral mass screws. METHODS: A total of 53 CSM patients underwent lateral mass screws instrumentation at C3 to C6 levels and pedicle screw instrumentation at C7 level were included. The preoperative 3-dimenional computed tomography (CT) reconstruction images of cervical spine were used to determine 2 different C7 pedicle screw trajectories. Trajectory A passed through the axis of the C7 pedicle while trajectory B selected the midpoint of the base of C7 superior facet as the entry point. All these 53 patients had the C7 pedicle screw inserted through trajectory B by free-hand without fluoroscopic guidance and the postoperative CT images were obtained to evaluate the accuracy of C7 pedicle screw insertion. RESULTS: Trajectory B had smaller transverse angle, smaller screw length, and smaller screw width but both similar sagittal angle and similar pedicle height when compared with trajectory A. A total of 106 pedicle screws were inserted at C7 through trajectory B and only 8 screws were displaced with the accuracy of screw placement as high as 92.5%. CONCLUSION: In CSM patients with C3 to C6 instrumented by LMS, using trajectory B for C7 pedicle screw insertion is easy to both identify the entry point and facilitate the rod insertion.

Structural Myelopathies.

OBJECTIVE: This article illustrates the clinical importance, diagnosis, and management of degenerative and nondegenerative structural myelopathies. It also aims to create a diagnostic approach for the evaluation of patients with suspected degenerative myelopathies. LATEST DEVELOPMENTS: There is considerable interest in developing diagnostic methods that can assist in deciding if surgery is indicated in patients with structural myelopathy and the optimal timing for surgery. Diffusion tensor imaging has emerged as a promising imaging modality although it is not used routinely in clinical practice. Neuroprotective medications and interventions are being studied in patients with degenerative myelopathies. ESSENTIAL POINTS: Structural myelopathies and particularly degenerative myelopathies are common disorders that are routinely encountered in clinical practice, with symptoms that frequently overlap with other neurologic disorders. The prompt diagnosis and treatment of patients are essential in achieving good functional outcomes.

Metabolic and Toxic Myelopathies.

OBJECTIVE: This article reviews the clinical presentation, diagnostic evaluation, and treatment of metabolic and toxic myelopathies resulting from nutritional deficiencies, environmental and dietary toxins, drugs of abuse, systemic medical illnesses, and oncologic treatments. LATEST DEVELOPMENTS: Increased use of bariatric surgery for obesity has led to higher incidences of deficiencies in nutrients such as vitamin B12 and copper, which can cause subacute combined degeneration. Myelopathies secondary to dietary toxins including konzo and lathyrism are likely to become more prevalent in the setting of climate change leading to drought and flooding. Although modern advances in radiation therapy techniques have reduced the incidence of radiation myelopathy, patients with cancer are living longer due to improved treatments and may require reirradiation that can increase the risk of this condition. Immune checkpoint inhibitors are increasingly used for the treatment of cancer and are associated with a wide variety of immune-mediated neurologic syndromes including myelitis. ESSENTIAL POINTS: Metabolic and toxic causes should be considered in the diagnosis of myelopathy in patients with particular clinical syndromes, risk factors, and neuroimaging findings. Some of these conditions may be reversible if identified and treated early, requiring careful history, examination, and laboratory and radiologic evaluation for prompt diagnosis.

The efficacy of virtual reality technology for the postoperative rehabilitation of patients with cervical spondylotic myelopathy (CSM): a study protocol for a randomized controlled trial.

BACKGROUND: Patients with cervical spondylosis myelopathy (CSM) may experience severe neurological dysfunction due to untimely spinal cord compression after surgery. These disorders may lead to sensory and motion disorders, causing considerable psychological distress. Recent studies found that virtual reality (VR) technology can be an effective tool for treating spinal cord injuries. Owing to this discovery, we developed an exploratory research project to investigate the impact of this intervention on the postoperative recovery of patients with CSM. METHODS: The purpose of this randomized controlled trial was to evaluate the efficacy of combining VR technology with conventional rehabilitation strategies for the postoperative rehabilitation of patients with CSM. A total of 78 patients will be recruited and randomized to either the conventional rehabilitation group or the group subjected to VR technology combined with conventional rehabilitation strategies. The Japanese Orthopaedic Association (JOA) scale will be the main tool used, and secondary outcomes will be measured via the visual analogue scale (VAS), neck disability index (NDI), and functional MRI (fMRI). The data analysis will identify differences between the intervention and control groups as well as any relationship between the intragroup changes in the functional area of the brain and the subjective scale scores after the intervention. DISCUSSION: The aim of this trial is to investigate the effect of VR training on the postoperative rehabilitation of patients with CSM after 12 intervention treatments. Positive and negative outcomes will help us better understand the effectiveness of the intervention and its neural impact. If effective, this study could provide new options for the postoperative rehabilitation of patients with CSM. TRIAL REGISTRATION: Chinese Clinical Trial Registry (ChiCTR2300071544). Registered 17 May 2023, https://www.chictr.org.cn/ .

Surgical treatment and outcome of haematomyelia with a traumatic cause in a dog and a cat.

Two surgically treated haematomyelia cases were documented. One dog and one cat were presented for acute progressive paraplegia following a fall from height incident. Neurological examinations suggested a L4-S3 myelopathy in both cases. Radiography and magnetic resonance imaging in both cases revealed no fracture or subluxation of the spine, but well-defined intramedullary mass lesions in lower lumbar regions compatible with haemorrhage and haematoma formation. Exploratory surgeries were performed over the lesions. Dark-red friable masses were removed via myelotomy. Histopathological examinations revealed organizing haematomas at the acute to subacute stage. Postoperatively, both cases improved and regained ambulation. The dog walked normally but remained urinary and faecal incontinent 9 months after the surgery. The cat was continent and ambulatory with a paraparetic gait 5 months after the surgery. In both cases, the outcomes and the patients' quality of life were considered satisfactory by the owners.

Anterior Full-endoscopic Single-port Double Transcorporeal Spinal Cord Decompression for Noncontinuous Two-segment Cervical Spondylotic Myelopathy: A Technical Note.

OBJECTIVE: In clinical practice, noncontinuous two-segment spinal cord cervical spondylosis is a particular form of cervical degenerative disease. Traditional anterior open surgery frequently comes with severe trauma, risks, and debatable treatment options. This study aimed to describe for the first time a novel minimally invasive technique, namely, anterior full-endoscopic single-port double transcorporeal spinal cord decompression for the treatment of patients with noncontinuous two-segment cervical spondylotic myelopathy. METHOD: From February 2020 to May 2021, five patients with noncontinuous two-segment cervical spondylotic myelopathy were treated with anterior full-endoscopic single-port double transcorporeal spinal cord decompression. Two bone channels were established by the trephine through the vertebral body oblique upward and downward to the herniated disc osteophyte complex, and the full-endoscopic system could decompress the spinal cord through the channels. All cases were followed up for over 2 years. The modified Japanese Orthopaedic Association (mJOA) score and visual analogue scale (VAS) score before and after operation and during follow-up were used to evaluate the clinical effectiveness. Radiological examinations, including CT and MRI, were utilized to evaluate the efficacy of spinal cord decompression and bone channel repair. RESULTS: All operations were successfully completed and the average operation time was 185 min, with no operation-related complications. Compared with the preoperative evaluation, the mJOA score and VAS score were improved at each time point after operation and follow-up. Postoperative CT and MRI scans showed that the intervertebral disc-osteophyte complex was removed through the vertebral bone passage, and the spinal cord was fully decompressed. After 24 months of follow-up, CT and MRI scans showed that the bone channel was almost repaired and healed. CONCLUSION: Anterior full-endoscopic single-port double transcorporeal spinal cord decompression is an effective minimally invasive technique for noncontinuous two-segment cervical spondylosis. It provides precise and satisfactory spinal cord decompression under endoscopic visualization with minimum trauma.

What predicts the best 24-month outcomes following surgery for cervical spondylotic myelopathy? A QOD prospective registry study.

OBJECTIVE: The aim of this study was to identify predictors of the best 24-month improvements in patients undergoing surgery for cervical spondylotic myelopathy (CSM). For this purpose, the authors leveraged a large prospective cohort of surgically treated patients with CSM to identify factors predicting the best outcomes for disability, quality of life, and functional status following surgery. METHODS: This was a retrospective analysis of prospectively collected data. The Quality Outcomes Database (QOD) CSM dataset (1141 patients) at 14 top enrolling sites was used. Baseline and surgical characteristics were compared for those reporting the top and bottom 20th percentile 24-month Neck Disability Index (NDI), EuroQol-5D (EQ-5D), and modified Japanese Orthopaedic Association (mJOA) change scores. A multivariable logistic model was constructed and included candidate variables reaching p ≤ 0.20 on univariate analyses. Least important variables were removed in a stepwise manner to determine the significant predictors of the best outcomes (top 20th percentile) for 24-month NDI, EQ-5D, and mJOA change. RESULTS: A total of 948 (83.1%) patients with 24-month follow-up were included in this study. For NDI, 204 (17.9%) had the best NDI outcome and 200 (17.5%) had the worst NDI outcome. Factors predicting the best NDI outcomes included symptom duration less than 12 months (OR 1.5, 95% CI 1.1-1.9; p = 0.01); procedure other than posterior fusion (OR 1.5, 95% CI 1.03-2.1; p = 0.03); higher preoperative visual analog scale neck pain score (OR 1.2, 95% CI 1.1-1.3; p < 0.001); and higher baseline NDI (OR 1.06, 95% CI 1.05-1.07; p < 0.001). For EQ-5D, 163 (14.3%) had the best EQ-5D outcome and 169 (14.8%) had the worst EQ-5D outcome. Factors predicting the best EQ-5D outcomes included arm pain-only complaints (compared to neck pain) (OR 1.9, 95% CI 1.3-2.9; p = 0.002) and lower baseline EQ-5D (OR 167.7 per unit lower, 95% CI 85.0-339.4; p < 0.001). For mJOA, 222 (19.5%) had the best mJOA outcome and 238 (20.9%) had the worst mJOA outcome. Factors predicting the best mJOA outcomes included lower BMI (OR 1.03 per unit lower, 95% CI 1.004-1.05; p = 0.02; cutoff value of ≤ 29.5 kg/m2); arm pain-only complaints (compared to neck pain) (OR 1.7, 95% CI 1.1-2.5; p = 0.02); and lower baseline mJOA (OR 1.6 per unit lower, 95% CI 1.5-1.7; p < 0.001). CONCLUSIONS: Compared to the worst outcomes for EQ-5D, the best outcomes were associated with patients with arm pain-only complaints. For mJOA, lower BMI and arm pain-only complaints portended the best outcomes. For NDI, those with the best outcomes had shorter symptom durations, higher preoperative neck pain scores, and less often underwent posterior spinal fusions. Given the positive impact of shorter symptom duration on outcomes, these data suggest that early surgery may be beneficial for patients with CSM.

Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease.

PURPOSE: Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature. METHODS: A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English. RESULTS: Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old. CONCLUSION: Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms.