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Zhongguo Xiu Fu Chong Jian Wai Ke Za... Oct 2023To review the clinical research progress of spinal epidural lipomatosis (SEL). (Review)
Review
OBJECTIVE
To review the clinical research progress of spinal epidural lipomatosis (SEL).
METHODS
The clinical studies on SEL at home and abroad in recent years were extensively reviewed, and the pathogenesis, clinical and imaging manifestations, and treatment status of SEL were summarized and analyzed.
RESULTS
SEL is a disease characterized by compression of the spinal cord and nerve roots due to abnormal accumulation of epidural adipose tissue in the spinal canal. Its prevalence and diagnosis rate are low and the pathogenesis is not fully understood. MRI is the most sensitive and specific diagnostic test for SEL. Surgical decompression and removal of excess adipose tissue are the only options for patients with acute SEL or those who have failed conservative management, and conservative management should be considered for other patients.
CONCLUSION
SEL is a rare disease and related research still needs to be improved. In the future, high-quality, multi-center and large-sample studies will be of great significance for evaluating the choice of treatment methods and effectiveness of SEL patients.
Topics: Humans; Decompression, Surgical; Epidural Space; Lipomatosis; Magnetic Resonance Imaging; Spinal Cord Diseases
PubMed: 37848326
DOI: 10.7507/1002-1892.202305071 -
Continuum (Minneapolis, Minn.) Feb 2015Both acute and chronic spinal cord disorders present multisystem management problems to the clinician. This article highlights key issues associated with chronic spinal... (Review)
Review
PURPOSE OF REVIEW
Both acute and chronic spinal cord disorders present multisystem management problems to the clinician. This article highlights key issues associated with chronic spinal cord dysfunction.
RECENT FINDINGS
Advances in symptomatic management for chronic spinal cord dysfunction include use of botulinum toxin to manage detrusor hyperreflexia, pregabalin for management of neuropathic pain, and intensive locomotor training for improved walking ability in incomplete spinal cord injuries.
SUMMARY
The care of spinal cord dysfunction has advanced significantly over the past 2 decades. Management and treatment of neurologic and non-neurologic complications of chronic myelopathies ensure that each patient will be able to maximize their functional independence and quality of life.
Topics: Chronic Disease; Disease Management; Humans; Spinal Cord Diseases
PubMed: 25651225
DOI: 10.1212/01.CON.0000461092.86865.a4 -
Genitourinary Medicine Oct 1989Whether the clinical pattern of neurosyphilis has changed since the introduction of penicillin is controversial. This study describes the clinical, laboratory and...
Whether the clinical pattern of neurosyphilis has changed since the introduction of penicillin is controversial. This study describes the clinical, laboratory and radiological features of nine patients with syphilitic myelopathy, to assess whether the disease pattern has changed in this subgroup. Four patterns based on clinical course and radiological findings were identified: three patients presented with subacute paraparesis, four with prodromal backache and/or mild leg weakness followed by sudden paraplegia, one patient developed slowly progressive weakness over 4 years and one patient who progressed over one month was shown to have dural thickening on myelography. All patients showed CSF pleocytosis with positive CSF VDRL in seven patients. Despite therapy the prognosis for recovery was not good. Compared with pre-penicillin era studies, the clinical pattern has not significantly changed. Greater alertness to the diagnosis might result in earlier therapy and thus possibly lead to improved prognosis.
Topics: Adult; Female; Humans; Male; Middle Aged; Myelography; Neurosyphilis; Penicillin G; Spinal Cord Diseases
PubMed: 2583718
DOI: 10.1136/sti.65.5.338 -
Journal of Inherited Metabolic Disease Jul 2020All men and most women with X-linked adrenoleukodystrophy (ALD) develop myelopathy in adulthood. As clinical trials with new potential disease-modifying therapies are...
All men and most women with X-linked adrenoleukodystrophy (ALD) develop myelopathy in adulthood. As clinical trials with new potential disease-modifying therapies are emerging, sensitive outcome measures for quantifying myelopathy are needed. This prospective cohort study evaluated spinal cord size (cross-sectional area - CSA) and shape (eccentricity) as potential new quantitative outcome measures for myelopathy in ALD. Seventy-four baseline magnetic resonance imaging (MRI) scans, acquired in 42 male ALD patients and 32 age-matched healthy controls, and 26 follow-up scans of ALD patients were included in the study. We used routine T -weighted MRI sequences to measure mean CSA, eccentricity, right-left and anteroposterior diameters in the cervical spinal cord. We compared MRI measurements between groups and correlated CSA with clinical outcome measures of disease severity. Longitudinally, we compared MRI measurements between baseline and 1-year follow-up. CSA was significantly smaller in patients compared to controls on all measured spinal cord levels (P < .001). The difference was completely explained by the effect of the symptomatic subgroup. Furthermore, the spinal cord showed flattening (higher eccentricity and smaller anteroposterior diameters) in patients. CSA correlated strongly with all clinical measures of severity of myelopathy. There was no detectable change in CSA after 1-year follow-up. The cervical spinal cord in symptomatic ALD patients is smaller and flattened compared to controls, possibly due to atrophy of the dorsal columns. CSA is a reliable marker of disease severity and can be a valuable outcome measure in long-term follow-up studies in ALD. SYNOPSIS: A prospective cohort study in 42 adrenoleukodystrophy (ALD) patients and 32 controls demonstrated that the spinal cord cross-sectional area of patients is smaller compared to healthy controls and correlates with severity of myelopathy in patients, hence it could be valuable as a much needed surrogate outcome measure.
Topics: Adrenoleukodystrophy; Adult; Atrophy; Case-Control Studies; Disease Progression; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Prospective Studies; Spinal Cord; Spinal Cord Diseases
PubMed: 32077106
DOI: 10.1002/jimd.12226 -
Experimental & Molecular Medicine Jun 2023Spinal cord injury (SCI) is a clinical condition that leads to permanent and/or progressive disabilities of sensory, motor, and autonomic functions. Unfortunately, no...
Spinal cord injury (SCI) is a clinical condition that leads to permanent and/or progressive disabilities of sensory, motor, and autonomic functions. Unfortunately, no medical standard of care for SCI exists to reverse the damage. Here, we assessed the effects of induced neural stem cells (iNSCs) directly converted from human urine cells (UCs) in SCI rat models. We successfully generated iNSCs from human UCs, commercial fibroblasts, and patient-derived fibroblasts. These iNSCs expressed various neural stem cell markers and differentiated into diverse neuronal and glial cell types. When transplanted into injured spinal cords, UC-derived iNSCs survived, engrafted, and expressed neuronal and glial markers. Large numbers of axons extended from grafts over long distances, leading to connections between host and graft neurons at 8 weeks post-transplantation with significant improvement of locomotor function. This study suggests that iNSCs have biomedical applications for disease modeling and constitute an alternative transplantation strategy as a personalized cell source for neural regeneration in several spinal cord diseases.
Topics: Humans; Rats; Animals; Neural Stem Cells; Spinal Cord Injuries; Neurons; Axons; Spinal Cord; Cell Differentiation
PubMed: 37258581
DOI: 10.1038/s12276-023-01003-2 -
AJNR. American Journal of Neuroradiology Jan 2013MPS represents a group of rare hereditary disorders characterized by multisystem involvement due to intralysosomal GAG accumulation. Among various tissues, both the... (Review)
Review
MPS represents a group of rare hereditary disorders characterized by multisystem involvement due to intralysosomal GAG accumulation. Among various tissues, both the central and peripheral nervous system are affected in almost all types of the disease. Thus, brain and spinal MR imaging are valuable tools for the assessment of neurologic involvement, and there is evidence that they might be reliable markers demonstrating disease severity and efficacy of treatment options currently used in patients with MPS. We aimed to review the most prominent MR imaging features of patients with MPS, paying attention to the physiopathologic mechanisms responsible for these alterations. Along with the description of neuroimaging findings, existing data in relation to their correlation with the severity of neurologic involvement is discussed, while another topic of great importance is the effect of various therapeutic regimens in the progression of brain and spinal MR imaging alterations. Finally, recent data concerning MR spectroscopy studies in MPS are also critically discussed.
Topics: Brain; Brain Diseases; Female; Humans; Male; Mucopolysaccharidoses; Spinal Cord; Spinal Cord Diseases; Young Adult
PubMed: 22790241
DOI: 10.3174/ajnr.A2832 -
Spine Oct 2016Systematic literature review. (Review)
Review
STUDY DESIGN
Systematic literature review.
OBJECTIVES
To summarize the risks of 3 key complications of stereotactic body radiotherapy (SBRT) for spinal metastases, that is, radiation myelopathy (RM), vertebral compression fracture (VCF), and epidural disease progression, and to discuss strategies for minimizing them.
SUMMARY OF BACKGROUND DATA
RM, VCF and epidural disease progression are now recognized as important risks following SBRT for spine metastases. It is unclear at this stage exactly how large these risks are and what strategies can be employed to minimize these risks.
METHODS
A systematic review of the literature using MEDLINE and a review of the bibliographies of reviewed articles on SBRT for spinal metastases were conducted.
RESULTS
The initial literature search revealed a total of 376 articles, of which 38 were pertinent to the study objectives. The risk of RM following SBRT was found to be dependent on the maximum dose to the spinal cord and estimated to be ≤5% if the recommended published thecal sac dose constraints are adhered to. The crude risk of VCF was 13.7% (range: 0.7%-40.5%), and, on average, 45% were surgically salvaged. It has been shown that the risk of VCF is dependent on several anatomic and tumor-related factors including the SBRT dose per fraction. The crude risk of local failure at 1 year was 21.4% (range: 12%-27%) of which 67% (range: 38%-96%) occurred within the epidural space. The grade of epidural disease has been shown to be associated with the risk of local failure.
CONCLUSION
The risk of RM after spinal SBRT is low in particular if recommended dose metrics are adhered to. There is a significant risk of both VCF and epidural disease progression after spinal SBRT. These risks can potentially be minimized by identifying the risk factors for these complications, and performing careful radiotherapy and surgical planning.
LEVEL OF EVIDENCE
2.
Topics: Disease Progression; Fractures, Compression; Humans; Radiotherapy; Spinal Cord Diseases; Spinal Fractures; Spinal Neoplasms
PubMed: 27488294
DOI: 10.1097/BRS.0000000000001823 -
Stem Cell Research & Therapy Jul 2012Injury and disease of the spinal cord are generally met with a poor prognosis. This poor prognosis is due not only to the characteristics of the diseases but also to our... (Review)
Review
Injury and disease of the spinal cord are generally met with a poor prognosis. This poor prognosis is due not only to the characteristics of the diseases but also to our poor ability to deliver therapeutics to the spinal cord. The spinal cord is extremely sensitive to direct manipulation, and delivery of therapeutics has proven a challenge for both scientists and physicians. Recent advances in stem cell technologies have opened up a new avenue for the treatment of spinal cord disease and injury. Stem cells have proven beneficial in rodent models of spinal cord disease and injury. In these animal models, stem cells have been shown to produce their effect by the dual action of cell replacement and the trophic support of the factors secreted by these cells. In this review we look at the main clinical trials involving stem cell transplant into the spinal cord, focusing on motor neuron diseases and spinal cord injury. We will also discuss the major hurdles in optimizing stem cell delivery methods into the spinal cord. We shall examine current techniques such as functional magnetic resonance imaging guidance and cell labeling and will look at the current research striving to improve these techniques. With all caveats and future research taken into account, this is a very exciting time for stem cell transplant into the spinal cord. We are only beginning to realize the huge potential of stem cells in a central nervous system setting to provide cell replacement and trophic support. Many more trials will need to be undertaken before we can fully exploit the attributes of stem cells.
Topics: Animals; Cell- and Tissue-Based Therapy; Clinical Trials as Topic; Disease Models, Animal; Humans; Spinal Cord Injuries; Stem Cell Transplantation; Stem Cells
PubMed: 22776143
DOI: 10.1186/scrt115 -
Journal of the Neurological Sciences Jul 2017Development of effective treatments for amyotrophic lateral sclerosis (ALS) would be facilitated by identification of early events in the pathological cascade of disease...
Development of effective treatments for amyotrophic lateral sclerosis (ALS) would be facilitated by identification of early events in the pathological cascade of disease progression. Degenerative myelopathy (DM), a naturally occurring disease in dogs, is quite similar to forms of ALS associated with SOD1 mutations and is likely to be a good model for these forms of the human disease. The sequence of histopathological changes that occur in DM was characterized by analyzing tissue samples obtained from affected dogs euthanized at various stages of disease progression. Cervical spinal cord and the associated spinal nerve roots, ulnar nerve, and the extensor carpi radialis (ECR) muscle were obtained from Pembroke Welsh Corgi dogs (PWCs) with early and late stage DM and from age-matched unaffected PWCs. In early stage disease there was a substantial change in the ratio of the two main muscle fiber types and an increase in mean muscle fiber area in the ECR. DM, even in late stage disease, was not accompanied by changes in the number of motor neuron cell bodies, in the number of axons in the motor or sensory nerve roots, or in the ulnar nerve. In addition, no disease-related denervation of the acetylcholine receptors of the ECR was observed at any stage of the disease. On the other hand, axon densities in both motor and sensory nerve tracts in the cervical cord were reduced in affected dogs. SOD1-immunoreactive aggregates were observed in spinal cord motor neuron cell bodies only in late stage disease. These findings suggest that some of the earliest pathological changes in DM occur in the muscle fibers and upper motor and sensory neuron tracts in the spinal cord. Targeting therapeutic interventions to these early events in the disease are most likely to be effective in slowing disease progression for DM and may translate to therapy of SOD1-related forms of ALS.
Topics: Amyotrophic Lateral Sclerosis; Animals; Axons; Cell Count; Cell Size; Cervical Cord; Disease Models, Animal; Dog Diseases; Dogs; Female; Male; Motor Neurons; Muscle Fibers, Skeletal; Neurodegenerative Diseases; Neuromuscular Junction; Spinal Cord Diseases; Spinal Nerve Roots; Ulnar Nerve
PubMed: 28566164
DOI: 10.1016/j.jns.2017.05.009 -
Spinal Cord Series and Cases 2019This perspective advocates for the adoption of recently published clinical practice guidelines on identifying and managing cardiometabolic risk after spinal cord injury... (Review)
Review
This perspective advocates for the adoption of recently published clinical practice guidelines on identifying and managing cardiometabolic risk after spinal cord injury (SCI). It makes the case for acting now, with the knowledge that we currently have, while continuing to address knowledge gaps with high-quality research studies in this area. Cardiovascular disease is a leading cause of death in people with SCI. Cardiometabolic disease (CMD) and risks are more likely to be overlooked after SCI. Unique SCI-related considerations impact both assessment and management of cardiometabolic risk. Risk factors and components of CMD including obesity, impaired glucose tolerance/insulin resistance, dyslipidemia, and hypertension should be evaluated and managed to optimize the cardiometabolic health of this population. While it would be optimal to base all care on high-quality evidence-based research, its absence should not be an excuse for inaction. Applying what is currently known and filling the research gaps with empirical recommendations based on clinical rationale and expert consensus is both appropriate and necessary till more definitive SCI-specific evidence becomes available.
Topics: Adult; Cardiovascular Diseases; Health Knowledge, Attitudes, Practice; Humans; Metabolic Diseases; Practice Guidelines as Topic; Risk Factors; Spinal Cord Injuries
PubMed: 31798971
DOI: 10.1038/s41394-019-0241-5