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STUDY DESIGN: Literature Review. OBJECTIVE: Myelopathy affecting the thoracic spinal cord can arise secondary to several aetiologies which have similar presentation and management. Consequently, there are many uncertainties in this area, including optimal terminology and definitions. Recent collaborative cervical spinal research has led to the proposal and subsequent community adoption of the name degenerative cervical myelopathy(DCM), which has facilitated the establishment of internationally-agreed research priorities for DCM. We put forward the case for the introduction of the term degenerative thoracic myelopathy(DTM) and degenerative spinal myelopathy(DSM) as an umbrella term for both DCM and DTM. METHODS: Following PRISMA guidelines, a systematic literature search was performed to identify degenerative thoracic myelopathy literature in Embase and MEDLINE. RESULTS: Conditions encompassed within DTM include thoracic spondylotic myelopathy, ossification of the posterior longitudinal ligament, ossification of the ligamentum flavum, calcification of ligaments, hypertrophy of ligaments, degenerative disc disease, thoracic osteoarthritis, intervertebral disc herniation, and posterior osteophytosis. The classic presentation includes girdle pain, gait disturbance, leg weakness, sensory disturbance, and bladder or bowel dysfunction, often with associated back pain. Surgical management is typically favoured with post-surgical outcomes dependent on many factors, including the causative pathology, and presence of additional stenosis. CONCLUSION: The clinical entities encompassed by the term DTM are interrelated, can manifest concurrently, and present similarly. Building on the consensus adoption of DCM in the cervical spine and the recent proposal of degenerative cervical radiculopathy(DCR), extending this common nomenclature framework to the terms degenerative spinal myelopathy and degenerative thoracic myelopathy will help improve recognition and communication.

STUDY DESIGN: Delayed diagnosis of degenerative cervical myelopathy (DCM) is likely due to a combination of its subtle symptoms, incomplete neurological assessments by clinicians and a lack of public and professional awareness. Diagnostic criteria for DCM will likely facilitate earlier referral for definitive management. OBJECTIVES: This systematic review aims to determine (i) the diagnostic accuracy of various clinical signs and (ii) the association between clinical signs and disease severity in DCM? METHODS: A search was performed to identify studies on adult patients that evaluated the diagnostic accuracy of a clinical sign used for diagnosing DCM. Studies were also included if they assessed the association between the presence of a clinical sign and disease severity. The QUADAS-2 tool was used to evaluate the risk of bias of individual studies. RESULTS: This review identified eleven studies that used a control group to evaluate the diagnostic accuracy of various signs. An additional 61 articles reported on the frequency of clinical signs in a cohort of DCM patients. The most sensitive clinical tests for diagnosing DCM were the Tromner and hyperreflexia, whereas the most specific tests were the Babinski, Tromner, clonus and inverted supinator sign. Five studies evaluated the association between the presence of various clinical signs and disease severity. There was no definite association between Hoffmann sign, Babinski sign or hyperreflexia and disease severity. CONCLUSION: The presence of clinical signs suggesting spinal cord compression should encourage health care professionals to pursue further investigation, such as neuroimaging to either confirm or refute a diagnosis of DCM.

STUDY DESIGN: Delayed diagnosis of degenerative cervical myelopathy (DCM) is associated with reduced quality of life and greater disability. Developing diagnostic criteria for DCM has been identified as a top research priority. OBJECTIVES: This scoping review aims to address the following questions: What is the diagnostic accuracy and frequency of clinical symptoms in patients with DCM? METHODS: A scoping review was conducted using a database of all primary DCM studies published between 2005 and 2020. Studies were included if they (i) assessed the diagnostic accuracy of a symptom using an appropriate control group or (ii) reported the frequency of a symptom in a cohort of DCM patients. RESULTS: This review identified three studies that discussed the diagnostic accuracy of various symptoms and included a control group. An additional 58 reported on the frequency of symptoms in a cohort of patients with DCM. The most frequent and sensitive symptoms in DCM include unspecified paresthesias (86%), hand numbness (82%) and hand paresthesias (79%). Neck and/or shoulder pain was present in 51% of patients with DCM, whereas a minority had back (19%) or lower extremity pain (10%). Bladder dysfunction was uncommon (38%) although more frequent than bowel (23%) and sexual impairment (4%). Gait impairment is also commonly seen in patients with DCM (72%). CONCLUSION: Patients with DCM present with many different symptoms, most commonly sensorimotor impairment of the upper extremities, pain, bladder dysfunction and gait disturbance. If patients present with a combination of these symptoms, further neuroimaging is indicated to confirm the diagnosis of DCM.

Objective: To describe the demographic, clinical, laboratory, and radiological findings, and the clinical course of seven patients with severe N2O-induced subacute combined degeneration of the spinal cord (SACD). Methods: Retrospective study with prospective follow-up of patients with SACD associated with N2O abuse presenting at a single center between 2014 and 2024. Results: The median age (range) of the seven patients (one woman, six men) was 24 (18–33) years. Prior to disease onset, patients had consumed N2O daily over a median (range) of 12 (3-20) weeks, with a mean (SD; range) inhalation dosage of 2376.7 (2872.7; 160–9000) g of N2O per day. Clinical presentations included paresthesia and paresis in the legs and gait disturbances. All patients exhibited characteristic signal alterations in the posterior columns spanning from C1 to T10 on T2-weighted spinal MRIs. Electrophysiology demonstrated polyneuropathies in all but one patient. Vitamin B12 levels were decreased in four, but normal in three patients. Methylmalonic acid levels were elevated in all patients. Although the median (interquartile range [IQR]) modified Rankin Scale score improved from 3.0 (3.0–4.0) at baseline to 1.0 (1.0–2.0; p < 0.05, Wilcoxon matched-pairs signed-rank test) at follow-up after the start of vitamin B12 supplementation, all five patients who could be examined on follow-up exhibited persistent deficits on the last follow-up assessment at a median (range) of 5 (3-116) months after disease onset. Conclusions: N2O abuse over a few weeks can lead to severe SACD. The diagnosis is supported by characteristic findings on spinal MRI and elevated methylmalonic acid levels, while normal vitamin B12 levels do not rule out N2O-induced SACD. Although there was some clinical improvement upon cessation of N2O abuse and vitamin B12 supplementation, residual deficits persisted. [ABSTRACT FROM AUTHOR]

Holocord syringomyelia (HSM) is characterized by a continuous spinal cord cavitation along its entire length and is currently poorly documented in dogs. This retrospective multicentric case series investigates the clinical and MRI findings in 18 dogs with HSM. The median age at presentation was 82 months (range 9–108 months) and French Bulldogs were overrepresented (50%). Signs of myelopathy attributed to HSM were detected in 12 dogs (67%), spinal pain/paresthesia/allodynia in eight (44%), and four dogs (22%) had no HSM-associated signs. The most common neuroanatomical localization was C1-C5 spinal cord segments. On MRI, the cranial limit of HSM was at C1 vertebra in seven (39%) and at C2 in 11 dogs (61%). The caudal limit extended to L3 in four dogs (22%), L4 or L5 in six dogs (33%) each, and L6 in two dogs (11%). The conus medullaris terminated at L5 in one dog (6%), L6 in 11 (61%), and L7 in six dogs (33%). Seventeen dogs (94%) exhibited MRI features of Chiari-like and/or other intracranial/cranio-cervical junction malformations. One dog (6%) was treated surgically, 11 (61%) medically, and six (33%) received no treatment. Over a median follow-up of 12 months (range 2–65 months) in 16 dogs, one dog (6%) improved, nine (56%) deteriorated, four (25%) were static, and two (13%) remained free of HSM-associated signs. This is the first study to describe canine HSM, highlighting a possible breed predisposition in French Bulldogs. The frequent occurrence of myelopathy and disease progression underscores the need for further research into the underlying etiopathogenesis, natural disease evolution, and response to therapeutic interventions. [ABSTRACT FROM AUTHOR]

Background: Nitrous oxide (N₂O), commonly known as laughing gas, is widely recognized for its anesthetic and analgesic effects, and is frequently used in medical contexts. However, its misuse can lead to significant neurological complications, which are often under-recognized in clinical practice. Recent data on such cases in Germany are rare. We here report the spectrum of neurological complications associated with the recreational use of N₂O, as encountered in German neurology centers. Methods: We retrospectively analyzed of 23 cases presenting with neurological symptoms following N₂O abuse between July 2020 and August 2024 across five neurology departments in Germany. Data were collected on patient demographics, clinical manifestations, diagnostic findings, and treatment approaches. Results: Over the last four years the number of cases increased. Clinical presentations primarily included neuropathy, found in all patients, along with myelopathy. The most common symptoms were sensory loss, ataxia, and motor deficits. Conclusion: Our data suggest that N₂O abuse is on the rise in Germany. Further initiatives are warranted to raise awareness among users, healthcare and professionals. [ABSTRACT FROM AUTHOR]

Objective: Paralysis subsequent to spinal cord decompression in the cervical or thoracic region is infrequent, with White Cord Syndrome (WCS) being among its several causes. Due to WCS's infrequency, there exists a paucity of high-level evidence concerning its manifestations. Our primary objective is to systematically collate all documented WCS cases, discern prevalent risk and prognostic factors, appraise available treatment modalities, and evaluate patient outcomes. Methods: A systematic review was conducted following PRISMA guidelines. The search included PubMed, Scopus, Embase, and Web of Science databases. Inclusion criteria required studies to be written in English, be case reports, and contain data on clinical features, management, and treatment outcomes. Exclusion criteria excluded meta-analyses, reviews, editorials, letters, books, studies with insufficient clinical data, and studies not in English or with unavailable full texts. Grey literature was not actively pursued due to identification challenges, potentially introducing selection bias. Two authors independently evaluated papers based on criteria. Disagreements were resolved with a third author. Additionally, the included articles' references were screened for additional relevant articles. Results: We found a total of 580 articles through our electronic search. After removing duplicates, 399 articles were screened. Out of the remaining 51 studies, 27 were included in the final quantitative analysis. The average age was 54 (3–79 years) with a male-to-female ratio of 2:1, 33% had OPLL, and Common medical histories were hypertension (30%), diabetes mellitus (20%), and previous ACDF surgery (8%). Of all Surgeries, 70% were done with a posterior approach and 30% with the anterior approach. 48% of cases used Intraoperative NeuroMonitoring(IONM), and Loss of Motor Evoked Potentials (MEP) occurred in 37% of cases. Patients received high-dose intravenous steroids. In 26% of cases, additional posterior cervical decompression was performed, and efforts were made to maintain mean arterial pressure above 85 mmHg in 37% of cases. Other medications were administered in 30% of cases. Over an average 26-week follow-up, 37% of patients had good recovery, 40% had partial recovery, and 23% showed no recovery. The average final Nurick grade was 3.2. Conclusions: WCS is a rare cause of postoperative neurological deficit following spinal cord decompression surgery. Risk factors for WCS include advanced age, extensive surgery, posterior approach for decompression, and the presence of OPLL. Treatment includes high-dose steroids, posterior cervical decompression, maintaining MAP over 85mmHg, rehabilitation, and sometimes neurotrophic drugs. Most patients can walk with or without assistance during follow-up, but around a quarter never regain neurological function. The only preoperative factor impacting outcomes is the preoperative neurological status (Nurick Grade). [ABSTRACT FROM AUTHOR]

Purpose: To explore the impact of the snake-eye appearance (SEA) on the efficacy of anterior cervical discectomy and fusion (ACDF) in treating Degenerative Cervical Myelopathy (DCM). Methods: A total of 316 Patients were divided into three groups, those with SEA were in the SEA group, and those with the absence of SEA were in the non-SEA group. Meanwhile, in the non-SEA group, patients with grade 1 or 2 increased signal intensity (ISI) on T2-weighted MRI were in the ISI group, and the remaining patients without ISI were in the non-ISI group. The modified Japanese Orthopaedic Association scale (mJOA) and the neurologic functional improvement rate (IR) were measured. The number of patients who achieved upper-limb motor weakness improvement was recorded and compared. Propensity score matching (PSM) was used among the three groups. Results: The postoperative JOA and IR were similar in the SEA and non-SEA group; however, they were significantly lower than in the non-ISI group after PSM(P < 0.05). Besides, only 32.1% of patients in the SEA obtained an improvement of upper-limb motor weakness, which was the lowest in three groups after PSM (60.7% in the non-SEA group and 78.6% in the non-ISI group, P < 0.05). Conclusions: We suggested that patients with SEA tend to achieve an inadequate neurological recovery after ACDF. Besides, a SEA may be an unfavorable prognostic factor for upper extremity motor function recovery. [ABSTRACT FROM AUTHOR]

Nitrous oxide (N2O), used medically as an anaesthetic, has gained popularity as a recreational drug, with rising prevalence particularly among young adults. While its reinforcing and addictive potential remains debated, N2O is proven to be neurotoxic, especially with prolonged, heavy use, which is often unexpected for users. The neurotoxicological mechanism underlying N2O‐induced neurotoxicity involves inactivation of vitamin B12 (cobalamin), which disrupts methionine synthesis, essential for maintaining the myelin sheath. This can result in demyelinating diseases, including generalized demyelinating polyneuropathy (GDP). Clinical incidence of N2O‐induced peripheral neuropathy is largely unknown, although some research suggests it is not uncommon. Treatment includes immediate cessation of N2O use and vitamin B12 supplementation. Although this treatment often reverses damage, residual symptoms such as limb weakness may persist. Additionally, genetic and dietary factors, such as vitamin B12 deficiency, may heighten individual vulnerability for N2O's detrimental effects. [ABSTRACT FROM AUTHOR]

Objectives: To analyze the efficacy of K-line in surgical planning of approach selection for ossification of posterior longitudinal ligament (OPLL) and outcomes assessment by Nurick grading and Modified Japanese Orthopaedic Association (mJOA) scores. Methods: This is a retrospective case series study conducted at the Departments of Neurosurgery, Punjab Institute of Neurosciences, Lahore in the months of January and February 2024. Patients with complete records were considered. Google Form was used for data. Nurick grading and Modified Japanese Orthopaedic Association (mJOA) scores were calculated for each patient pre- and post-operatively. K-line assessment was done on computerized tomography (CT). Data analysis was performed using Microsoft excel in terms of frequency and percentages. Results: This study included ten patients with the mean age at presentation was 48.20 ± 9.37 years. Preoperative Nurick grading in our patients was Grade-V (five patients), Grade-IV (two patients) and Grade-III (three patients) with mean of 4.2 ± 0.91 SD whereas six months follow-up Nurick grading was Grade-V (three patients), Grade-IV (three patients) and Grade-III (four patients) with the mean of 3.90 ± 0.87 SD, which indicates neurological improvement. Types of OPLL present in our patients were segmental (4, 40%), continuous (3, 30%), mixed (2, 20%) and localized/ others (1, 10%). There were 2 (20%) K-line (+) and 8 (80%) K-line (-) cases. Anterior approach was used in 2 (20%) cases whereas posterior approach was used for the rest of 8 (80%) cases. Mean preoperative mJOA was 10.30 ± 2.45 and mean postoperative mJOA was12.40 ± 2.01 at 6-month follow up, which indicates improvement in our cases. Conclusion: K-line is a useful radiological indicator in selecting anterior versus posterior approach for patients with cervical OPLL in terms of Nurick grading and mJOA scores. [ABSTRACT FROM AUTHOR]

This article aims to demonstrate the uniportal full endoscopic surgery for treating complex anterior and posterior spinal pathology at the T1–2 level, offering a invasive, accessible, stable, and versatile approach to challenging anatomical situations. Uniportal full endoscopic surgery is one of the most minimally invasive spinal surgeries, utilizing slim, elongated, and compact instruments that provide access to lesions from any angle and distance. This characteristic makes the technique especially suitable for hard, such as the T1–2 level, where traditional approaches may be limited or difficult. We present the case of a 39-year-old male patient (height, 187 cm; weight, 130 kg) who developed myelopathy due to a hard disc herniation and ossification of the ligamentum flavum at the T1–2 leading to paraparesis, which was more severe on the left side. An anterior approach was challenging due to the anatomical constraints at the T1–2 level, as well as the patient's body size. A posterior access via the interlaminar approach facilitated the removal of the ossified ligamentum flavum. However, to the anterior lesion remained problematic without spinal cord retraction. Using the uniportal full endoscope, we were able to approach both anterior and posterior lesions through an incision 8 cm lateral to the midline, allowing for the treatment of the entire 270° arc of the pathology. The slim and elongated nature of the full endoscope enabled effective decompression without exerting pressure on the spinal cord, providing access from any angle and distance. This technique can be applied to a variety of cases involving difficult-to-access spinal. [ABSTRACT FROM AUTHOR]

Transarterial embolization (TAE) is generally the endovascular treatment of choice for tentorial dural arteriovenous fistula (dAVF). Although flow control of the feeder vessel has been reported to achieve complete shunt blockade, flow control in the absence of ischemia tolerance of internal carotid artery as a feeder has not been reported. We present a case in which treatment by Onyx TAE with intermittent flow control of the meningohypophyseal trunk as the feeder was successful for a tentorial dAVF presenting with myelopathy without tolerance of ischemia. The intermittent flow control is presented for a tentorial dAVF presenting with myelopathy without tolerance for ischemia. An inflation of the balloon in the internal carotid artery was set for 5 minutes, and the Onyx injection was repeated at intervals of at least 2 minutes. Injections and pauses were repeated to allow Onyx to reach the shunt pouch. The patient underwent successful TAE with intermittent flow control for a tentorial dAVF presenting with myelopathy. The disappearance of the shunt was confirmed with gait disturbance resolution postoperatively. Intermittent flow control of the meningohypophyseal trunk using a balloon may be safe and effective for cases showing no tolerance for ischemia. [ABSTRACT FROM AUTHOR]

Background: Recent studies assessing the importance of various preoperative factors on postoperative outcomes following spine surgery have uncovered several important variables that influence subjective and objective outcomes following cervical spine surgery, but it is still unclear which patients are most likely to benefit from operative management. Purpose: The objective of this study was to assess whether preoperative patient-reported outcome measures (PROMs) can be used to predict which patients achieve "normal" levels of pain and function after surgery. Study Design: This was a prospective cohort study. Patient Sample: This study included all adult patients undergoing cervical spine surgery by 1 of 7 senior spine surgeons at our institution between 2016 and 2018. Of the 164 patients who were eligible for 6-month follow-up at the time that study data were collected, 139 had available follow-up data and were included in our analysis. Outcomes Measures: Patients completed the Neck Disability Index (NDI) as well as the Patient-Reported Outcomes Measurement Information System (PROMIS) Physical Function (PF) and Pain Interference computer adaptive tests preoperatively and at 6 months postoperatively. Methods: Patients who achieved postoperative patient-acceptable symptom state (PASS) for NDI (=17) and the normative mean (50) for PROMIS were identified. The relationship between preoperative PROMs and the probability of achieving PASS and the normative mean was assessed. Results: One hundred thirty-nine patients met inclusion criteria with diagnoses of myelopathy (n = 36), radiculopathy (n = 48), and myeloradiculopathy (n = 49). For NDI, a 1-point worsening in the preoperative score resulted in an OR of achieving PASS of 0.96 (P < 0.001) in the overall population. This association held true for patients with radiculopathy (OR 0.96; P = 0.022) but not myelopathy (OR 0.98; P = 0.35). For PROMIS PF, a 1-point improvement in the preoperative score resulted in an OR of achieving the normative mean of 1.10 (P < 0.001). This association held true for patients with radiculopathy (OR 1.14; P = 0.033) but did not reach statistical significance for patients with myelopathy (OR 1.03; P = 0.515). Conclusions: Preoperative PROMs can predict postoperative benefit for patients undergoing cervical spine surgery, with worse baseline function associated with a lower likelihood of attaining PASS for NDI and the normative mean for PROMIS PF, especially for patients with radiculopathy. Clinical Relevance: Baseline symptoms and function, including myelopathy or radiculopathy-dominant symptoms and preoperative PROMs, may predict postoperative outcomes. [ABSTRACT FROM AUTHOR]

Background: Previous research suggests a relationship between complications associated with anterior cervical discectomy and fusion and level involvement; however, there is limited research comparing postoperative outcomes of upper cervical fusions (UCFs) with middle-to-lower cervical fusions (MLCFs). This study aims to compare the outcomes of UCF with MLCF. Methods: A retrospective medical record review was conducted on 835 anterior cervical discectomy and fusion patients from 2012 to 2022. Patients were classified as UCF, defined as inclusion of C3 to C4 disc space, or MLCF, defined as lacking C3 to C4 disc space. Demographics were compared using χ2 or Fisher exact tests. Clinical characteristics were compared in univariable analysis using χ2 tests, linear-mixed effects models, or generalized linear-mixed models depending on distribution. Significant pre- and intraoperative characteristics were included in multivariable models to minimize confounding. Results: Of the 835 patients included, 562 underwent MLCF and 281 underwent UCF. Median follow-up time was 211 days for UCF and 200 days for MLCF. UCF led to a 1.5-day longer length of stay in both univariable (1.5 vs 3.1, P < 0.0001) and multivariable analysis (2.3 days [95% CI: 1.8, 3.0] vs 3.3 days [2.6, 4.2], P < 0.0001). MLCF patients reported symptom improvement or resolution more often than UCF patients (0.43 [95% CI: 0.30, 0.62] and 0.46 [95% CI: 0.30, 0.70]). Additionally, a significantly higher rate of dysphagia was reported in the UCF group on both univariate and multivariable analysis, respectively (1.72 [95% CI: 1.18, 2.49] and 1.66 [95%CI: 1.08, 2.56]). Conclusions: To our knowledge, this is the first study to investigate the link between cervical fusion level and outcomes. UCF patients demonstrated greater rates of dysphagia, longer length of stay, and lower likelihood of improvement in neurological symptoms postoperatively both before and after controlling for differences in pre- and intraoperative characteristics. Clinical Relevance: This study highlights that UCFs may be associated with worse postoperative outcomes when compared to MLCFs, which can inform surgical decision-making and patient counseling. Level of Evidence: The study represents Level 3 evidence due to its retrospective design and potential biases, indicating a need for future prospective randomized controlled trials to validate these findings. [ABSTRACT FROM AUTHOR]

Abstract Background Nitrous oxide (N₂O), commonly known as laughing gas, is widely recognized for its anesthetic and analgesic effects, and is frequently used in medical contexts. However, its misuse can lead to significant neurological complications, which are often under-recognized in clinical practice. Recent data on such cases in Germany are rare. We here report the spectrum of neurological complications associated with the recreational use of N₂O, as encountered in German neurology centers. Methods We retrospectively analyzed of 23 cases presenting with neurological symptoms following N₂O abuse between July 2020 and August 2024 across five neurology departments in Germany. Data were collected on patient demographics, clinical manifestations, diagnostic findings, and treatment approaches. Results Over the last four years the number of cases increased. Clinical presentations primarily included neuropathy, found in all patients, along with myelopathy. The most common symptoms were sensory loss, ataxia, and motor deficits. Conclusion Our data suggest that N₂O abuse is on the rise in Germany. Further initiatives are warranted to raise awareness among users, healthcare and professionals.

Hailin Liu,1,&ast; Chao Huang,1,&ast; Yanjiao Du,1 Jiacheng Liu,1,2 Xiangyang Ren,1 Huilin Wang,1 Jingna Ye,1 Haitao Zhou,1 Zhihui Duan1 1Department of Neurology, Luoyang Central Hospital Affiliated to Zhengzhou University, Luoyang Cerebrovascular Disease (Stroke) Clinical Medical Research Center, Regional Medical Center for Neurological Diseases of Henan Province, Luoyang, People’s Republic of China; 2Xinxiang Medical College, Xinxiang, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Zhihui Duan, Department of Neurology, Luoyang Central Hospital Affiliated to Zhengzhou University, Luoyang Cerebrovascular Disease (Stroke) Clinical Medical Research Center, Regional Medical Center for Neurological Diseases of Henan Province, Xigong District, Luoyang, Henan, 471000, People’s Republic of China, Tel/Fax +0086-0379-63892044, Email 54487843@qq.comIntroduction: Transthyretin protein-related familial amyloidosis polyneuropathy (TTR-FAP) is an autosomal dominant genetic disease caused by mutations in the TTR gene. The disease is characterized primarily by peripheral and autonomic nerve damage. Disease progression is associated with frequent involvement of the heart, lungs, kidneys, eyes, and other organs. The most common TTR mutation is c.148G>A (p.Val50Met), although the FAP resulting from the mutation rarely involves the spinal cord.Patient Concerns: A 68-year-old man was diagnosed with the TTR c.148G>A (p.Val50Met) mutation by ultrasound, pathological, and genetic analyses. He presented with a late-onset, complicated spinal cord injury. The diagnostic process was tortuous, and despite the administration of regular treatment (conventional drugs, cardiac pacemaker, and the specific drug clofenadifen), the patient died.Interventions: To confirm TTR-FAP, ultrasound, MRI, pathological, and genetic tests were performed.Outcomes: The patient ultimately died of heart failure 7.5 years after the initial onset of symptoms.Conclusion: The patient presented with unusual symptoms of spinal cord injury, and despite a long and arduous diagnostic process and administration of standard treatment for over seven years, the outcome was poor. It is thus recommended that clinicians pay attention to the identification of rare diseases with timely imaging, pathological, and genetic testing, to avoid poor outcomes.Keywords: transthyretin familial amyloid polyneuropathy, myelopathy, case

Introduction: Minimally invasive surgical treatment of myelopathy caused by central thoracic disc herniation (TDH) is challenging to carry out because reaching the herniation site is difficult and the thoracic spinal cord is fragile. In this study, using the posterior-lateral approach for central TDH with myelopathy, we present a novel procedure of transcostal microendoscopic discectomy (TCMED). Technical Note: The patient was operated in a prone position under general anesthesia. At a preoperatively determined distance from the midline, an 18-mm-long longitudinal incision was conducted, and using a 25-degree microendoscope, the operation was carried out. The endoscope was placed at an inward angle of approximately 50 degrees in the vertical direction. The ribs adjacent to the disc were identified, and the disc was exposed by resecting the ribs using a surgical high-speed drill while preserving the cortical bone of the ribs on the pleural side. The herniation was identified by drilling the ventral side of the disc and was then successfully removed. After discectomy, the dura mater expanded ventrally. Three male patients (mean age, 47.3 years) were treated, with 20 weeks of follow-up on average, 237.7-min mean operative time, and 26.7-mL mean blood loss. The average preoperative modified Japanese Orthopedic Association score was 5.2/11, which improved to 9.5/11 postoperatively, with a 75.6% average recovery rate. The 10-s step test score improved from an average of eight times preoperatively to 20 times postoperatively. No serious perioperative or postoperative complications or residual rib pain were observed. Conclusions: The proposed TCMED approach for treating central TDH that causes myelopathy allows for safe access to the level of the posterior vertebral wall using the rib as a landmark for resecting the rib head without opening the chest. Using the angled microendoscope and curved surgical instruments, the central TDH, located ventral to the spinal canal, can then be safely and effectively resected without spinal cord retraction.

Abstract Objectives Nitrous oxide has long been used as an anesthetic agent. The recreational use and abuse are rapidly increased in Western countries and lead to many neurological complications. Methods Retrospectively review of seven patients. Results Seven patients aged 19–32 years, mean 22.6 years. They inhaled nitrous oxide between 1 month to 1 year prior to the symptom onset. They all presented with acute or subacute ataxia or motor, and sensory dysfunction. The two had coexisting encephalopathy. Electrodiagnosis showed sensorimotor axonal polyneuropathy. All patients had borderline or low serum vitamin B12 level. Two had high serum homocysteine or methylmalonic acid levels. Cervical spine MRI in two patients showed posterior column lesion. At average 2 month-follow up, all patients had minimal improvement. While at more than 6 month-follow up, most patients had moderate to complete recovery. Conclusion Seven patients with nitrous oxide induced neurological disease are reported. All patients present with acute myelopathy and sensorimotor polyneuropathy. Short term outcome is generally not favorable while long term outcome shows remarkable improvement.

Peri-medullary arteriovenous fistula (PMAVF) is a rare spinal vascular malformation that manifests as progressive neurologic deficits or hemorrhage in the spinal canal. We report a case of high-flow PMAVF in a child, with a single feeder artery and a large venous pouch, which was successfully treated with transarterial endovascular intervention. A 2-year-old boy was referred with a progressive 2-year history of myelopathy. The MRI revealed a large venous pouch at the midthoracic spinal cord with segmental surrounding edema. A spinal angiogram confirmed high-flow PMAVF with a single feeder artery from the anterior radiculomedullary artery, draining into the peri-medullary vein. The patient underwent transarterial embolization at the distal feeder artery, resulting in gradual motor strength improvement. PMAVF is classified as type IV spinal vascular malformation, usually presenting as a large, high-flow fistula with multiple feeders, although there was only one in this case. PMAVFs are intradural and may cause severe neurologic deficits due to mass effect, venous congestion, or hemorrhage, hence requiring prompt treatment. Treatment options for PMAVF include microsurgery, endovascular intervention, or a combination of the 2. Endovascular intervention with coil or liquid embolic material is considered first-line treatment for IVc PMAVF, and effective in type IVb with good clinical outcome. PMAVF is a rare spinal vascular malformation commonly manifesting as severe neurologic deficits but has the potential of favorable outcomes with endovascular therapy. This case demonstrates a unique angioarchitecture of high-flow PMAVF with a single feeder artery and large venous pouch, treated successfully with endovascular therapy.

Objectives: Nitrous oxide has long been used as an anesthetic agent. The recreational use and abuse are rapidly increased in Western countries and lead to many neurological complications. Methods: Retrospectively review of seven patients. Results: Seven patients aged 19–32 years, mean 22.6 years. They inhaled nitrous oxide between 1 month to 1 year prior to the symptom onset. They all presented with acute or subacute ataxia or motor, and sensory dysfunction. The two had coexisting encephalopathy. Electrodiagnosis showed sensorimotor axonal polyneuropathy. All patients had borderline or low serum vitamin B12 level. Two had high serum homocysteine or methylmalonic acid levels. Cervical spine MRI in two patients showed posterior column lesion. At average 2 month-follow up, all patients had minimal improvement. While at more than 6 month-follow up, most patients had moderate to complete recovery. Conclusion: Seven patients with nitrous oxide induced neurological disease are reported. All patients present with acute myelopathy and sensorimotor polyneuropathy. Short term outcome is generally not favorable while long term outcome shows remarkable improvement. [ABSTRACT FROM AUTHOR]

To conduct a systematic review and meta-analysis comparing the functional and radiological outcomes in cervical spondylotic myelopathy (CSM) when treated by Laminectomy (LC) vs. laminectomy with instrumented fusion (LCF). The systematic review was conducted in accordance with PRISMA guidelines. A systematic electronic search was performed in PubMed, EMBASE, and CENTRAL databases. All Studies comparing clinical and radiological outcomes in patients with CSM when treated with LCF and LC were included in the systematic review. Neuromuscular function measured by modified Japanese orthopaedic association (mJOA), Cervical lordosis measured by C2-C7 Cobb's angle, and data on complications were retrieved from the studies. Random effect meta-analysis was used to account for heterogeneity. Quality assessment was performed using ROBINS-I and ROB-2 tools. Certainty of evidence was assessed using the GRADE working group recommendations. A total of 3985 articles were retrieved from the databases, of which 9 articles (1 RCT, 8 controlled comparative trials; n = 1146) met the eligibility criteria. The improvement in mJOA showed results favouring laminectomy with fusion with an SMD of 0.45 (95% CI, 0.07–0.84; p = 0.02). The loss of cervical lordosis was significantly lesser in the laminectomy with fusion group with an SMD of − 0.454 (95% CI, (− 0.12) – (− 0.79), p = 0.01). The complication rate in the LCF group was lower (LogOR = − 0.92; 95% CI: (− 1.30) – (− 0.55); p = < 0.05). In CSM, laminectomy with fusion is superior to standalone laminectomy in terms of improvement in m-JOA, maintenance of cervical lordosis and reduction in complications. [ABSTRACT FROM AUTHOR]

Background/Objectives: This study investigated the relationship between spinal canal expansion and clinical outcomes in patients with myelopathy due to ossification of the posterior longitudinal ligament (OPLL) who underwent cervical open-door laminoplasty. Methods: A retrospective study was conducted on 36 OPLL patients who underwent open-door laminoplasty between 2009 and 2021. Preoperative and two-year postoperative radiologic parameters, including bony canal area (BCA) and spinal canal area (SCA), were measured. Clinical outcomes were assessed using the Numerical Rating Scale (NRS) for neck pain and radicular pain, the Neck Disability Index (NDI), and Japanese Orthopaedic Association (JOA) scores. Results: The mean expansion of BCA was 112.1 mm2 (47%) and SCA was 100.5 mm2 (64%). All clinical outcomes improved after surgery, although not statistically significant. JOA scores improved significantly in the severe group, while NDI and NRS-neck scores improved in the mild to moderate group. Significant correlations were found between improvements in NRS-neck and expansions of BCA (r = 0.533, p = 0.001) and SCA (r = 0.537, p = 0.001). NDI improvement was also associated with BCA expansion. No significant correlations were found between canal expansion and NRS-R, NRS-L, or JOA scores. Conclusions: Cervical open-door laminoplasty effectively increased the bony and spinal canal areas in patients with OPLL and myelopathy. In addition to improving myelopathy symptoms, this procedure may also improve neck pain and disability. Further research is needed to assess the long-term outcomes and to better understand these clinical improvements. [ABSTRACT FROM AUTHOR]

• Copper deficiency myelopathy (CDM) is a rare disease of progressive dorsal column dysfunction resulting in sensory ataxia, weakness, and spasticity. • Patients presenting to a spine surgeon with myelopathy that progress in spite of adequate surgical decompression, or myelopathy concomitant with cytopenia, require further investigation for copper deficiency myelopathy. • Clues to copper deficiency include a distinct MRI T2 hyperintense inverted "V" signal in the dorsal columns in a patient with risk factors for copper deficiency such as gastric bypass, bariatric surgery, or excessive zinc exposure. • Hematological abnormalities resolve rapidly with replenishment of copper, while neurological deficits only stabilize or partially improve Appropriate copper supplementation therapy may lead to a reversal of T2 signal-intensity changes in the dorsal columns. Copper deficiency myelopathy (CDM) is a rare disease that can present with spastic quadriparesis and sensory ataxia. As a result, it can precisely mimic cervical spondylitic myelopathy (CSM). Copper deficiency may be seen following gastric bypass surgery, malabsorption syndromes such as celiac disease, and with excessive exogenous zinc intake. We present a systematic review of the literature for CDM and an illustrative case. Provide a systematic review of CDM to highlight the importance of recognizing the consideration of CDM in patients presenting to a spine surgeon with myelopathy that progress despite adequate surgical decompression, or myelopathy concomitant with cytopenia, thus requiring further workup. Retrospective medical record review and systematic review of the literature. PubMed and Ovid-Embase database search was conducted in July 2022. Self-reported measures include PRISMA flow diagram for retrospective review; Physiological measures include retrospective review of MRI imaging of cervical spine; alternate demographic and laboratory value data extracted via literature review. A PubMed and Ovid-Embase database search was conducted in July 2022 searching for "copper deficiency myelopathy (MeSH)" from 2000 to 2022 via PRISMA guidelines. Following title and abstract review, the following data was extracted from full text: age, sex, etiology, hematological values upon presentation (mean corpuscular volume, white blood count, platelet count, and hemoglobin level), metal serum studies (serum copper, ceruloplasmin, and zinc), 24-hour collection of copper and zinc, and distinct radiographic findings on MRI. A total of 116 studies were included in this review which contained 198 cases of copper deficiency myelopathy. The mean age was 53.57±14.14 years, with the majority being females (63.8%). The most common etiology was prior gastric surgery (n=55, 36.2 %) followed by excessive zinc consumption from the use of zinc denture cream (n=39, 19.9%). The mean serum copper was 15.67±17.84 (normal=80.0–155.0) mcg/dL and mean ceruloplasmin was 6.43±5.25 (normal=16–45) mg/dL. In spite of appropriate treatment with copper supplementation, only 47 cases (24%) reported improvement in neurological status, and only 10 (5.1%) recovered to baseline. A hyperintense T2 signal abnormality resembling an inverted "v" in the dorsal columns was the most common radiographic abnormality. Pertinent risk factors for copper deficiency myelopathy include prior upper gastrointestinal surgery, zinc excess, and malabsorption. Characteristic laboratory and imaging findings include cytopenia, low serum copper and ceruloplasmin, and distinct inverted "v" T2 signal hyperintensity in the dorsal columns. The neurologic deterioration with copper deficiency will progress in spite of decompressive surgery, and can be devastating and irreversible even with copper supplementation, reinforcing the importance of early detection. We thus recommend patients with myelopathy presenting with a history of gastric bypass, malabsorption syndromes, excessive zinc exposure, cytopenia, or imaging resembling an inverted "v" shaped hyperintense T2 MRI signal in the dorsal columns, should first undergo blood tests for copper, ceruloplasmin, and B12 levels prior to surgical consideration. [ABSTRACT FROM AUTHOR]

Objective: We describe two cases of myelopathy onset due to intracranial dural arteriovenous fistulas (DAVFs) and present a literature review. Case Presentation: (Case 1) A 44-year-old man with subacute onset myelopathy underwent an MRI and DSA. MRI showed T2-hyperintensity from the medulla oblongata to the cervical spinal cord with vascular flow voids, suggestive of a spinal DAVF. Unexpectedly, cerebral angiography revealed a tentorial DAVF. (Case 2) A 47-year-old man with progressive myelopathy underwent a head and spinal MRI. Head MRI and MRA were considered to be normal. Spinal MRI revealed T2-hyperintensity in the cervical spinal cord without obvious vascular flow voids around the spinal cord. Contrast-enhanced MRI showed a patchy gadolinium enhancement in the same spinal cord region with the enhancement of perimedullary vessels. Although myelitis was initially suspected, subsequently spinal DAVF was suspected because cervical CTA revealed abnormal spinal venous drainage. Unexpectedly, cerebral angiography identified a foramen magnum DAVF. Conclusion: Regarding unexplained cervical myelopathy, even the absence of spinal cord surface vascular flow voids cannot necessarily exclude venous congestive myelopathy due to the DAVFs. In such cases, the contrast-enhanced MRI and cervical CTA are useful for visualizing abnormal vessels around the brain stem and the cervical spine. Especially, the co-presence of the abnormal vessels around the brain stem can suggest the intracranial DAVFs. Not only spinal DAVFs but also intracranial DAVFs should be considered as the differential diagnoses for venous congestive cervical myelopathy, in which cases cerebral angiography including carotid angiography is essential. [ABSTRACT FROM AUTHOR]

Thoracic back pain is a common site for inflammatory, neoplastic, metabolic, infectious, and degenerative conditions, and may be associated with significant disability and morbidity. Uncomplicated acute thoracic back pain and/or radiculopathy does not typically warrant imaging. Imaging may be considered in those patients who have persistent pain despite 6 weeks of conservative treatment. Early imaging may also be warranted in patients presenting with "red flag" history or symptoms, including those with a known or suspected history of cancer, infection, immunosuppression, or trauma; in myelopathic patients; or in those with a history of prior thoracic spine fusion. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation. [ABSTRACT FROM AUTHOR]

Objective. The objective of this study was to identify a unique serum profile of circulating miRNAs and inflammatory markers in patients with degenerative cervical myelopathy (DCM) compared with healthy controls (HC). Summary of Background Data. Currently, DCM is diagnosed with a combination of history, physical examination, and close correlation to advanced imaging. To date, no serum marker has been identified to be diagnostic of this condition. Methods. Whole venous blood was collected from patients with DCM as well as healthy age-matched and gender-matched controls. miRNA was extracted from venous blood, and a screening analysis was initially conducted to identify miRNA dysregulation in DCM patients. RT-qPCR was used to analyze the expression of 2 specific miRNAs based on screening analysis and literature review. Bioinformatics analysis was used to identify gene networks and potential targets of the miRNA. In addition, the serum inflammatory profile of DCM and HC groups was differentiated using a pro-inflammatory panel. Results. Thirty-six patients were enrolled in the DCM group (36.1% male, 61.5 ±9.5 y), while 35 patients were enrolled in the HC group (31.4% male, 57.5± 8.9 y). Of the 15 total miRNAs differentially expressed between DCM and HC groups, two were selected for further analysis: miR-223-3p (upregulated) and miR-451a (downregulated). Functional gene network analysis revealed the highest-ranking gene network was involved in neurological disease, while the most overexpressed miRNA in this network (miR-233-3p) was noted to have over 100 targets, including CDKN1B and the insulin receptor. Serum cytokine analysis showed significant upregulation of several pro-in- flammatory cytokines in the DCM cohort compared with the HC group. Conclusion. DCM patients demonstrated a set of unique circulating miRNAs in addition to a different serum inflammatory profile compared with HC. These miRNAs may potentially serve as targets for future therapeutic intervention or diagnostic/ prognostic testing. [ABSTRACT FROM AUTHOR]

Objectives: Thoracic hypertrophied ligamentum flavum (HLF) is a rare cause of thoracic myelopathy which requires surgical treatment. The study aims to analyze the pre-operative factors and surgical outcomes in patients with thoracic myelopathy secondary to HLF. Materials and Methods: This is a retrospective study of patients who underwent surgery for thoracic myelopathy due to HLF at a single center from December 2015 to November 2023. We included patient's demographic data, clinical symptoms, radiological details, operative details, and outcome. We used Nurick's grading system for outcome evaluation in pre-operative, post-operative, and follow-up. Relationship of various pre-operative parameters was evaluated with surgical outcomes on binary scale (favorable group [Nurick's grade 1 and 2] and unfavorable outcome [Nurick's grade 3–5]) using univariate and multivariate analysis. Results: A total of 57 patients were included in the study. On analyzing various prognostic factors with respect to favorable and unfavorable outcomes using univariate analysis, four factors came out to be statistically significant which were segments involved (multi vs. single segment involvement) (P < 0.05), walking difficulty (P < 0.05), intramedullary signal changes on T2-weighted magnetic resonance imaging (MRI) (P < 0.001), and pre-operative Nurick's grade (P < 0.001). On multivariate analysis, only one factor, pre-operative Nurick's grade came out statistically significant. Conclusion: Various factors are important in predicting the outcome of a patient with thoracic myelopathy secondary to HLF. The most important of which is pre-operative Nurick's grade. Other factors that also affect the outcome are the presence of multisegmented disease and intramedullary T2 signal changes on MRI. [ABSTRACT FROM AUTHOR]

Background: Cervical Spinal cord is considered an important Organ at risk while Radiotherapy of head and neck cancer (HNC). Thus when the tolerance dose is exceeded for spinal cord it is important to look for symptoms of demyelination. Objective: To assess post radiotherapy demyelination of Spinal cord in HNC Patients in whom tolerance dose for spinal Cord were exceeded. Material & Methods: This prospective observational study was conducted on 485 patients receiving radiotherapy for treatment of HNC. In patients whom the doses for spinal cord exceeds tolerance dose of 50 Gy were then assessed for demyelination. Patients showing symptoms of demyelination were examined and Magnetic Resonance Imaging (MRI) to assess extent of damage was obtained and conservatively managed. Results: Only 80 patients received dose greater than 50 Gray (Gy) to more than 10 cm length of spinal cord. Of these 12 patients developed demyelination which was confirmed on MRI and managed conservatively with Vit.B12 supplementation and pregabaline. Among these10 patients recovered while 2 patients had irreversibly damaged cord. Main reasons for exceeding tolerance dose include locally advanced disease extending to posterior triangle in 80 % patients, progression of disease in 10 % which required additional doses to the gross tumor and Positive margins in 10 % patients for which additional boost doses needed to be delivered. Conclusions: Demyelination is a debilitating late sequel of Radiotherapy and adversely affect quality of life of the patient and care should be taken while planning radiotherapy so that tolerance doses are not exceeded. [ABSTRACT FROM AUTHOR]

The use of biomodels in the laboratory for studying and training cervical laminoplasty has not yet been reported. We propose the use of a cervical spine biomodel for surgical laminoplasty training. This is an experimental study. Ten 3D identical cervical spine biomodels were printed based on computed tomography (CT) and magnetic resonance imaging scans of a patient diagnosed with spondylotic cervical myelopathy. The additive manufacturing method used fused deposition modeling and polylactic acid (PLA) was selected as the raw material. The sample was divided into 2 groups: control (n = 5; the biomodels were submitted to CT scanning) and open-door (n = 5; the biomodels were submitted to open-door laminoplasty and postoperative CT). The area and anteroposterior diameter of the vertebral canal were measured on CT scans. Printing each piece took 12 hours. During the surgical procedure, there was sufficient support from the biomodels to keep them immobilized. Using the drill was feasible; however continuous irrigation was mandatory to prevent plastic material overheating. The raw material made the biomodel CT study possible. The vertebral canal dimensions increased 24.80% (0.62 cm2) in area and 24.88% (3.12 mm) in anteroposterior diameter The cervical spine biomodels can be used for laminoplasty training, even by using thermosensitive material such as PLA. The use of continuous irrigation is essential while drilling. [ABSTRACT FROM AUTHOR]

The enhanced recovery after surgery (ERAS) protocol is a proven method to improve postsurgical outcomes. While recent studies have shown the benefit of ERAS even in frail patient populations, myelopathy is another factor affecting outcomes in patients undergoing posterior cervical fusion (PCF). This study evaluated the benefit of an ERAS protocol in frail patients undergoing PCF. A retrospective chart review identified consecutive patients undergoing PCF by a single surgeon from August 2015–July 2021, with implementation of ERAS in December 2018. Outcome measures included length of stay (LOS), nonhome discharge disposition, complications, return of physiologic function, and severe pain score. A mFI-5 score of ≥ 2 and a Nurick score of ≥ 3 defined frail and myelopathic patients, respectively. Univariate analysis (P < 0.05) and multivariate analyses using mixed-effect models (P < 0.0125) were performed. There were a total of 174 patients, 71 frail (41%). Of the frail patients, 61% were also myelopathic, and 56% underwent ERAS. Of the nonfrail patients, 43% were myelopathic, and 57% underwent ERAS. On univariate analyses, frail patients with ERAS had less drains placed (P < 0.0001), decreased urinary retention (P = 0.0002), decreased LOS (P = 0.013), and were less likely to have a nonhome discharge (P = 0.001). On multivariate analysis, LOS (P = 0.0003), time to return of physiologic function (P = 0.004), complications (P = 0.001), and nonhome discharges (P < 0.0001) were decreased with ERAS, irrespective of groups. ERAS is an effective protocol in PCF patients that may expedite return of physiologic function, lessen LOS, decrease the number of nonhome discharges, and reduce complications, irrespective of frailty or myelopathy status. [ABSTRACT FROM AUTHOR]

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Study Design: This study is a scoping review. Objective: There is a broad variability in the definition of degenerative cervical myelopathy (DCM) and no standardized set of diagnostic criteria to date. Methods: We interrogated the Myelopathy.org database, a hand-indexed database of primary clinical studies conducted exclusively on DCM in humans between 2005-2021. The DCM inclusion criteria used in these studies were inputted into 3 topic modeling algorithms: Hierarchical Dirichlet Process (HDP), Latent Dirichlet Allocation (LDA), and BERtopic. The emerging topics were subjected to manual labeling and interpretation. Results: Of 1676 reports, 120 papers (7.16%) had well-defined inclusion criteria and were subjected to topic modeling. Four topics emerged from the HDP model: disturbance from extremity weakness and motor signs; fine-motor and sensory disturbance of upper extremity; a combination of imaging and clinical findings is required for the diagnosis; and "reinforcing" (or modifying) factors that can aid in the diagnosis in borderline cases. The LDA model showed the following topics: disturbance to the patient is required for the diagnosis; reinforcing factors can aid in the diagnosis in borderline cases; clinical findings from the extremities; and a combination of imaging and clinical findings is required for the diagnosis. BERTopic identified the following topics: imaging abnormality, typical clinical features, range of objective criteria, and presence of clinical findings. Conclusions: This review provides quantifiable data that only a minority of past studies in DCM provided meaningful inclusion criteria. The items and patterns found here are very useful for the development of diagnostic criteria for DCM. [ABSTRACT FROM AUTHOR]

Objectives: The main objective of this study was to establish a mouse model of spinal ligament ossification to simulate the chronic spinal cord compression observed in patients with ossification of the posterior longitudinal ligament (OPLL). The study also aimed to examine the mice's neurobiological, radiological, and pathological changes. Methods: In the previous study, a genetically modified mouse strain was created using Crispr‐Cas9 technology, namely, Enpp1flox/flox/EIIa‐Cre (C57/B6 background), to establish the OPLL model. Wild‐type (WT) mice without compression were used as controls. Functional deficits were evaluated through motor score assessment, inclined plate testing, and gait analysis. The extent of compression was determined using CT imaging. Hematoxylin and eosin staining, luxol fast blue staining, TUNEL assay, immunofluorescence staining, qPCR, and Western blotting were performed to evaluate levels of apoptosis, inflammation, vascularization, and demyelination in the study. Results: The results demonstrated a gradual deterioration of compression in the Enpp1flox/flox/EIIa‐Cre mice group as they aged. The progression rate was more rapid between 12 and 20 weeks, followed by a gradual stabilization between 20 and 28 weeks. The scores for spinal cord function and strength, assessed using the Basso Mouse Scale and inclined plate test, showed a significant decline. Gait analysis revealed a noticeable reduction in fore and hind stride lengths, stride width, and toe spread. Chronic spinal cord compression resulted in neuronal damage and activated astrocytes and microglia in the gray matter and anterior horn. Progressive posterior cervical compression impeded blood supply, leading to inflammation and Fas‐mediated neuronal apoptosis. The activation of Bcl2 and Caspase 3 was associated with the development of progressive neurological deficits (p < 0.05). Conclusions: The study presents a validated model of chronic spinal cord compression, enabling researchers to explore clinically relevant therapeutic approaches for OPLL. [ABSTRACT FROM AUTHOR]

ABSTRACT This review aims to update the cervical laminoplasty techniques described in the literature and perform a systematic review of the open-door (ODL) and French-door (FDL) laminoplasties. A literature review was conducted on the Pubmed and Scielo platforms. This study was separated into two phases. The first section reviewed the laminoplasty techniques described in the literature. In this phase, a literature review was carried out using the descriptor “cervical laminoplasty”, employing “clinical trial”, “meta-analysis”, “review,” and “systematic review” as filters. The second section was a systematic review of the two most-studied techniques, the open-door and French-door laminoplasties. In this phase, a review was carried out using the descriptors “cervical laminoplasty”, “open-door” and “French-door” or “double-door”, employing “meta-analysis” and “review” as filters. In both phases, no limits were defined on the period. The first laminoplasty was described in 1973 and, since then, there have been, at least, six more techniques described that involve opening the spinal canal. Other studies report slight modifications in the type of spine access or details related to the osteotomy technique. Five systematic reviews were performed comparing the ODL and FDL. Only two of them analyzed common variables, and their results were divergent. Both ODL and FDL achieve favorable clinical and radiological results for the patient, and it is not yet possible to say whether one is better than the other. Level of Evidence II; Therapeutic Studies.

Holocord syringomyelia (HSM) is characterized by a continuous spinal cord cavitation along its entire length and is currently poorly documented in dogs. This retrospective multicentric case series investigates the clinical and MRI findings in 18 dogs with HSM. The median age at presentation was 82 months (range 9–108 months) and French Bulldogs were overrepresented (50%). Signs of myelopathy attributed to HSM were detected in 12 dogs (67%), spinal pain/paresthesia/allodynia in eight (44%), and four dogs (22%) had no HSM-associated signs. The most common neuroanatomical localization was C1-C5 spinal cord segments. On MRI, the cranial limit of HSM was at C1 vertebra in seven (39%) and at C2 in 11 dogs (61%). The caudal limit extended to L3 in four dogs (22%), L4 or L5 in six dogs (33%) each, and L6 in two dogs (11%). The conus medullaris terminated at L5 in one dog (6%), L6 in 11 (61%), and L7 in six dogs (33%). Seventeen dogs (94%) exhibited MRI features of Chiari-like and/or other intracranial/cranio-cervical junction malformations. One dog (6%) was treated surgically, 11 (61%) medically, and six (33%) received no treatment. Over a median follow-up of 12 months (range 2–65 months) in 16 dogs, one dog (6%) improved, nine (56%) deteriorated, four (25%) were static, and two (13%) remained free of HSM-associated signs. This is the first study to describe canine HSM, highlighting a possible breed predisposition in French Bulldogs. The frequent occurrence of myelopathy and disease progression underscores the need for further research into the underlying etiopathogenesis, natural disease evolution, and response to therapeutic interventions.

Spinal dural arteriovenous fistulas (SDAVFs) are rare vascular malformations with unspecific clinical manifestations, which often lead to misdiagnosis and delay in the establishment of effective therapies. Since the neurological prognosis depends on symptom duration and pretreatment neurological status, rapid identification and obliteration of the fistula are key to provide patients with a better quality of life. We herein describe an illustrative case of an elderly patient presenting with progressive myelopathy, with a definitive diagnosis of SDAVF after 18 months of the initial symptoms. We also review the core concepts regarding this condition and discuss strategies to prevent misdiagnosis and worsening of the neurological outcome.

Abstract Background Cerebrotendinous xanthomatosis (CTX, OMIM #213700) is a rare inherited metabolic disease caused by the mutation in the CYP27A1 gene. Spinal CTX is a rare clinical subgroup of CTX which lacks typical symptoms seen in classical CTX. Here we report a spinal CTX case revealed double mutation of CYP27A1 gene. Case presentation A 42-year-old Asian man visited our hospital with spastic gait started at 35. Physical examination showed bilateral masses on his Achilles tendons and were identified as xanthoma on ankle magnetic resonance imaging (MRI). Brain and spinal cord MRI revealed high signal lesions in bilateral cerebellar dentate nuclei and long tract lesions involving lateral corticospinal and gracile tracts. Gene analysis revealed double heterozygous mutation, c.223C > T (p. Gln75Ter) and c.1214G > A (p. Arg405Gln). Conclusions We believe that novel mutation detected in our case might have a role in the pathomechanism in CTX. Moreover, spinal CTX should be considered in the patients only presenting with pyramidal symptoms, as CTX shows good prognosis in early treatment with chenodeoxycholic acid.

Among rather rare but extremely interesting and important conditions, myelitis stands apart due to its complexity and diversity of clinical manifestations. In this article, in addition to the well-known data on this group of diseases, we present the results of the analysis of eight cases of myelitis, which may serve as a starting point for the development of more effective and streamlined strategies in diagnosis and treatment. It may also contribute to deeper understanding of difficult cases in neurological practice.

Background: Cervical posterior instrumentation and fusion is often performed to avoid post-laminectomy kyphosis. However, larger comparative analyses of cervical laminectomy with or without fusion are sparse. Methods: A retrospective, two-center, comparative cohort study included patients after stand-alone dorsal laminectomy with (n = 91) or without (n = 46) additional fusion for degenerative cervical myelopathy with a median follow-up of 59 (interquartile range (IQR) 52) months. The primary outcome was the C2-7 Cobb angle and secondary outcomes were Neck Disability Index (NDI), modified Japanese Orthopaedic Association (mJOA) scale, revision rates, T1 slope and C2-7 sagittal vertical axis (C2-7 SVA) at final follow-up. Logistic regression analysis adjusted for potential confounders (i.e. age, operated levels, and follow-up). Results: Preoperative C2-7 Cobb angle and T1 slope were higher in the laminectomy group, while the C2-7 SVA was similar. The decrease in C2-7 Cobb angle from pre- to postoperatively was more pronounced in the laminectomy group (− 6° (IQR 20) versus −1° (IQR 7), p = 0.002). When adjusting for confounders, the decrease in C2-7 Cobb angle remained higher in the laminectomy group (coefficient − 12 (95% confidence interval (CI) −18 to −5), p = 0.001). However, there were no adjusted differences for postoperative NDI (− 11 (− 23 to 2), p = 0.10), mJOA, revision rates, T1 slope and C2-7 SVA. Conclusion: Posterior cervical laminectomy without fusion is associated with mild loss of cervical lordosis of around 6° in the mid-term after approximately five years, however without any clinical relevance regarding NDI or mJOA in well-selected patients (particularly in shorter segment laminectomies of < 3 levels). [ABSTRACT FROM AUTHOR]

According to the modular hypothesis for the control of movement, muscles are recruited in synergies, which capture muscle coordination in space, time, or both. In the last two decades, muscle synergy analysis has become a well-established framework in the motor control field and for the characterization of motor impairments in neurological patients. Altered modular control during a locomotion task has been often proposed as a potential quantitative metric for characterizing pathological conditions. Therefore, the purpose of this systematic review is to analyze the recent literature that used a muscle synergy analysis of neurological patients' locomotion as an indicator of motor rehabilitation therapy effectiveness, encompassing the key methodological elements to date. Searches for the relevant literature were made in Web of Science, PubMed, and Scopus. Most of the 15 full-text articles which were retrieved and included in this review identified an effect of the rehabilitation intervention on muscle synergies. However, the used experimental and methodological approaches varied across studies. Despite the scarcity of studies that investigated the effect of rehabilitation on muscle synergies, this review supports the utility of muscle synergies as a marker of the effectiveness of rehabilitative therapy and highlights the challenges and open issues that future works need to address to introduce the muscle synergies in the clinical practice and decisional process. [ABSTRACT FROM AUTHOR]

Copyright of Die Orthopädie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Cerebrotendinous xanthomatosis (CTX, OMIM #213700) is a rare inherited metabolic disease caused by the mutation in the CYP27A1 gene. Spinal CTX is a rare clinical subgroup of CTX which lacks typical symptoms seen in classical CTX. Here we report a spinal CTX case revealed double mutation of CYP27A1 gene. Case presentation: A 42-year-old Asian man visited our hospital with spastic gait started at 35. Physical examination showed bilateral masses on his Achilles tendons and were identified as xanthoma on ankle magnetic resonance imaging (MRI). Brain and spinal cord MRI revealed high signal lesions in bilateral cerebellar dentate nuclei and long tract lesions involving lateral corticospinal and gracile tracts. Gene analysis revealed double heterozygous mutation, c.223C > T (p. Gln75Ter) and c.1214G > A (p. Arg405Gln). Conclusions: We believe that novel mutation detected in our case might have a role in the pathomechanism in CTX. Moreover, spinal CTX should be considered in the patients only presenting with pyramidal symptoms, as CTX shows good prognosis in early treatment with chenodeoxycholic acid. [ABSTRACT FROM AUTHOR]

Ossification of the posterior longitudinal ligament (OPLL) is a rare but potentially devastating clinical entity of degenerative myelopathy. Highest reported prevalence of OPLL is in East Asian countries. The cervical spine is more commonly affected followed by thoracic and lumbar spine. The clinical manifestations range from asymptomatic to myelopathy or myeloradiculopathy. Although rare, thoracic OPLL is more severe than cervical OPLL and usually requires operative intervention. CT scan is the investigation of choice to determine the extent and thickness of the ossification. Treatment ranges from observation in patients with minimal symptoms to Surgical decompression followed by stabilization for patients with myelopathy. Here we discuss a middle-aged lady who presenting with a slowly progressive spastic paraplegia with bladder dysfunction, without sensory involvement. She was being treated conservatively as a case of spondylotic degenerative disease, but showed no improvement. Finally proper evaluation of her radiologic finding and clinical correlation revealed the diagnosis of OPLL. She underwent laminoplasty with decompressive surgery. The outcome was proved to be curative. [ABSTRACT FROM AUTHOR]

Background: The factors underlying the topography of nitrous oxide (N2O)‐induced neurological complications are unknown. Methods: We included all consecutive patients admitted to the university hospital of Marseille for N2O‐induced neurological complications in a prospective observational study. Patients underwent neurological examination, spinal cord magnetic resonance imaging, and nerve conduction studies within the first 4 weeks after admission. Results: In total, 61 patients were included: 45% with myeloneuropathy, 34% with isolated myelopathy, and 21% with isolated neuropathy. On multivariable analysis, the odds of myelopathy were associated with the amount of weekly N2O consumption (~600 g cylinder per week, odds ratio [OR] = 1.11, 95% confidence interval [CI] = 1.001–1.24). The extent of the myelopathy (number of vertebral segments) was correlated with the number of ~600‐g cylinders consumed weekly (ρ = 0.40, p < 0.005). The odds of neuropathy were associated with the duration of consumption (per month; OR = 1.29, 95% CI = 1.05–1.58). Mean lower‐limb motor nerve amplitude was correlated with the duration of consumption (in months; ρ = −0.34, p < 0.05). Conclusions: The odds of myelopathy increased with the amount of N2O consumption, and the odds of neuropathy increased with the duration of N2O exposure, which suggests distinct pathophysiological mechanisms underlying these two neurological complications. [ABSTRACT FROM AUTHOR]

Cervical spondylosis is the most common progressive disease of the cervical spine. As a disease that directly affects the function and morphology of the spinal cord and nerve roots, it is of particular interest to neurologists and neurosurgeons. However, due to its prevalence, physicians of all specialties encounter this disease. This article summarizes the current knowledge of the pathomechanism, diagnostics, and treatment of cervical spondylosis. [ABSTRACT FROM AUTHOR]