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European Journal of Paediatric... Mar 2022Clinical manifestations of malformations of cortical development (MCD) are variable and can range from mild to severe intellectual disability, cerebral palsy and... (Review)
Review
Clinical manifestations of malformations of cortical development (MCD) are variable and can range from mild to severe intellectual disability, cerebral palsy and drug-resistant epilepsy. Besides common clinical features, non-specific or more subtle clinical symptoms may be present in association with different types of MCD. Especially in severely affected individuals, subtle but specific underlying clinical symptoms can be overlooked or overshadowed by the global clinical presentation. To facilitate the interpretation of genetic variants detailed clinical information is indispensable. Detailed (neurological) examination can be helpful in assisting with the diagnostic trajectory, both when referring for genetic work-up as well as when interpreting data from molecular genetic testing. This systematic literature review focusses on different clues derived from the neurological examination and potential further work-up triggered by these signs and symptoms in genetically defined MCDs. A concise overview of specific neurological findings and their associations with MCD subtype and genotype are presented, easily applicable in daily clinical practice. The following pathologies will be discussed: neuropathy, myopathy, muscular dystrophies and spastic paraplegia. In the discussion section, tips and pitfalls are illustrated to improve clinical outcome in the future.
Topics: Cerebral Cortex; Drug Resistant Epilepsy; Epilepsy; Humans; Malformations of Cortical Development; Nervous System Malformations; Peripheral Nervous System
PubMed: 34535379
DOI: 10.1016/j.ejpn.2021.08.006 -
Journal of Vascular Surgery Oct 2017A systematic review and meta-analysis was conducted to evaluate the effectiveness of thoracic endovascular aortic repair (TEVAR) and open repair in patients with... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
A systematic review and meta-analysis was conducted to evaluate the effectiveness of thoracic endovascular aortic repair (TEVAR) and open repair in patients with descending thoracic aortic aneurysms (TAAs).
METHODS
PubMed, Ovid MEDLINE, Ovid Embase, EBSCO Cumulative Index to Nursing and Allied Health Literature, and Scopus were searched from each database's inception to January 29, 2016. We selected studies that compared the two approaches in adults with TAAs and reported 30-day mortality or procedure complications. Two reviewers independently extracted data, and conflicts were resolved by consensus. Random-effects meta-analysis was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs). The main outcomes and measures were all-cause 30-day mortality, 30-day paraplegia or spinal cord ischemia, stroke, pulmonary complications, and length of hospital and intensive care unit (ICU) stay.
RESULTS
Twenty-seven studies of moderate methodologic quality were included. TEVAR was associated with lower 30-day mortality in ruptured (OR, 0.58; 95% CI, 0.38-0.88) and intact (OR, 0.6; 95% CI, 0.36-0.99) aneurysms. Paraplegia or spinal cord ischemia (OR, 0.35; 95% CI, 0.2-0.61) and pulmonary complications (OR, 0.41; 95% CI, 0.37-0.46) were reduced in patients undergoing TEVAR, whereas a reduction in stroke risk was not statistically significant (OR, 0.89; 95% CI, 0.76-1.03). Pooled mean difference in length of hospital and ICU stay was lower for TEVAR by -5.17 days (95% CI, -7.77 to -2.57) and -5.89 days (95% CI, -9.65 to -2.12), respectively. Three studies showed that compared with open repair, a hybrid approach reduced hospital stay (pooled mean difference, -8.83 days; 95% CI, -14.37 to -3.29) and ICU stay (pooled mean difference, -3.17 days (95% CI, -5.54 to -0.97), with minimal evidence on other outcomes studied.
CONCLUSIONS
Observational evidence at high risk of confounding suggests that compared with open repair for TAA, TEVAR reduced risk of mortality, paraplegia, spinal cord ischemia, and pulmonary complications within 30 days of intervention. Patients undergoing TEVAR also had shorter length of hospital and ICU stay compared with patients undergoing open repair.
Topics: Aortic Aneurysm, Thoracic; Blood Vessel Prosthesis Implantation; Endovascular Procedures; Humans; Length of Stay; Odds Ratio; Postoperative Complications; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome
PubMed: 28756047
DOI: 10.1016/j.jvs.2017.05.082 -
Clinical Obesity Aug 2020Obesity contributes to a plethora of significant chronic diseases. Bariatric surgery has been demonstrated to be the most cost-effective treatment for severe obesity and...
INTRODUCTION
Obesity contributes to a plethora of significant chronic diseases. Bariatric surgery has been demonstrated to be the most cost-effective treatment for severe obesity and significantly reduces morbidity and mortality from metabolic syndrome. Patients with paraplegia have significantly impaired mobility and are therefore at a much higher risk of developing severe obesity and its sequelae. Bariatric surgery may bring significant improvements to mobility, morbidity and quality of life for patients with paraplegia, but evidence in the literature is poor for this group of patients.
METHODS
A systematic review was conducted conforming to PRISMA guidelines. The MEDLINE and Cochrane databases were searched for all articles published prior to April 2019 matching all of the keywords 'bariatric', 'paraplegia' and 'spinal cord'. Articles were assessed for relevance and full texts reviewed. In addition, clinical records were reviewed for three patients who underwent bariatric surgery at a single UK private institution. Non-identifiable demographic, clinical, operative and outcome data were obtained from electronic records.
RESULTS
Twenty seven articles were retrieved from the initial database search, of which nine eligible full texts were reviewed. Eight articles were case reports or case series and the final article was a systematic review. All cases reported had positive outcomes with significant weight loss, improvement in mobility and increased quality of life. Outcomes from the three diversely aged patients in our case series were similarly positive, with no significant post-operative complications.
DISCUSSION
Patients with obesity and paraplegia may significantly benefit from bariatric surgery. There is a need for multi-centre cohort studies to evaluate outcomes and the choice of bariatric intervention. UK guidelines do not include criteria based on mobility or neurological deficit, resulting in a potential missed opportunity to offer a cost-effective treatment that can significantly improve quality of life for patients with severe obesity and paraplegia.
Topics: Adult; Bariatric Surgery; Female; Humans; Middle Aged; Obesity; Paraplegia; Postoperative Complications; Quality of Life; Treatment Outcome; Weight Loss
PubMed: 32506828
DOI: 10.1111/cob.12382 -
Frontiers in Neurology 2019Hereditary Spastic Paraplegia (HSP) is a slowly progressive neurodegenerative disorder with no disease modifying treatment. Potential therapeutic approaches are...
Hereditary Spastic Paraplegia (HSP) is a slowly progressive neurodegenerative disorder with no disease modifying treatment. Potential therapeutic approaches are emerging and large-scale clinical drug trials for patients with HSP are imminent. A sensitive biomarker to measure the drug efficacy in these trials is required. Motor evoked potentials (MEPs) are a potential biomarker for HSP as they assess the central motor pathways and can be standardized with set protocols and guidelines. We performed a systematic review to investigate the utility of MEPs as a diagnostic and disease severity biomarker for HSP. Systematic searches of PubMed, Embase, Medline, and Scopus were performed. Studies reporting on central motor conduction time measured with MEPs in adult and pediatric patients with HSP were included. We excluded studies in non-HSP patient cohorts, not in English, not original research, and unpublished journal articles. Search results were de-duplicated and screened according to the inclusion and exclusion criteria. The included papers were reviewed independently by two reviewers and data was collected on patient cohorts, test methods, results, and study quality. Results were analyzed using descriptive methods. Of the 882 search results, 32 studies were included in the review. The most common finding was absent or prolonged lower limb (LL) central motor conduction time (CMCT) in patients with HSP (78% of patients studied). Quality assessment revealed variability in study methodology and reporting of results. Variations included patient cohorts of various genotypes as well as variations in equipment and techniques used. Aside from CMCT, none of the MEP parameter measures correlated with disease severity and many did not show significant difference between HSP patients and controls. Systematic review of MEP studies in HSP patient cohorts demonstrated mixed findings. Lower limb CMCT was the most promising parameter in terms of differentiating HSP patients from controls, with one study demonstrating a weak correlation with clinical disease severity. It is possible that the lack of consistency in study methodologies and small patient cohorts have contributed to the variable findings. A longitudinal study of MEPs in a large cohort of HSP patients with the same genotype will help clarify the utility of MEPs as a biomarker for disease severity and use in clinical trials.
PubMed: 31620065
DOI: 10.3389/fneur.2019.00967 -
Archives of Physical Medicine and... Jul 2022To analyze and summarize the effect of regulating exercise training interventions with subjective measures of intensity on cardiorespiratory fitness, measured by peak... (Meta-Analysis)
Meta-Analysis Review
Effects of Perceptually Regulated Exercise Training on Cardiorespiratory Fitness and Peak Power Output in Adults With Spinal Cord Injury: A Systematic Review and Meta-analysis.
OBJECTIVE
To analyze and summarize the effect of regulating exercise training interventions with subjective measures of intensity on cardiorespiratory fitness, measured by peak oxygen uptake (V̇O) and peak power output (PO) in adults with spinal cord injury (SCI).
DATA SOURCES
Four databases (MEDLINE, Embase, PsycINFO, SPORTDiscus) were searched from inception up until September 1, 2020, and updated November 18, 2021.
STUDY SELECTION
Searches combined keywords relating to the topics: SCI, subjective measures of exercise intensity, and exercise.
DATA EXTRACTION
Two reviewers independently conducted eligibility screening, data extraction, and assessed the risk of bias. Nine studies were included in the systematic review and meta-analysis, resulting in the inclusion of data from 95 adults with SCI representing both sexes and a diverse range of age, time since injury, lesion level, and lesion completeness classifications.
DATA SYNTHESIS
Data were extracted and added to summary tables with 3 outcomes: V̇O, PO, and Other. Mean and SD values for V̇O and PO were extracted from pre- and post-perceptually regulated exercise training.
CONCLUSIONS
All studies used ratings of perceived exertion scale to prescribe exercise intensity. Seven of 8 studies concluded an improvement in V̇O, and 5 studies of 7 concluded an improvement in PO. In the outcome Other, 5 studies concluded an improvement, and 3 studies concluded no change. There was evidence for an improvement in cardiorespiratory fitness, measured by V̇O and PO after perceptually regulated exercise training in adults with SCI (Grading of Recommendations, Assessment, Development, and Evaluation ratings: Low) (mean difference [MD], 2.92mL/kg/min; 95% confidence interval [CI], 1.30-4.54; P=.0004 and MD, 9.8W; 95% CI, 5.5-14.3; P<.0001, respectively). This review provides critically appraised, cumulative evidence on the use of perceptually regulated exercise training in individuals with SCI.
Topics: Adult; Cardiorespiratory Fitness; Exercise; Exercise Therapy; Female; Humans; Male; Spinal Cord Injuries
PubMed: 35398049
DOI: 10.1016/j.apmr.2022.03.008 -
Plastic and Reconstructive Surgery.... Aug 2016The purpose of this article is to describe the indications, operative technique, outcomes, and systematic review of the literature on the reconstruction of patients with...
BACKGROUND
The purpose of this article is to describe the indications, operative technique, outcomes, and systematic review of the literature on the reconstruction of patients with end-stage pressure ulcers using a fillet flap technique. In this technique, the femur, tibia, and fibula are removed from the thigh and leg, and the soft tissue is used as a pedicled, or free, myocutaneous flap for reconstruction. Long-term outcomes, salient surgical technique of flap elevation, and design are detailed for patients who had a fillet of leg flap for reconstruction of extensive pressure ulcers.
METHODS
The indications, surgical technique, and postoperative outcomes of 5 patients who had pedicled fillet flaps are reviewed including patient age, sex, underlying comorbidities, duration of paraplegia, operative technique, and complications. A systematic review of the literature was performed searching PubMed, Cochrane Database, and Medline with the following MeSH terms: pressure ulcer, pressure sore, decubitus ulcer, fillet flap, and fillet flap. Inclusion criteria were use of a fillet technique, article data on the number of reconstructions before fillet flap, complications, and English language.
RESULTS
Most of our patients were male 75% (n = 3) with an average age of 47.5 years, had been paralyzed for an average of 16 years, and had few medical comorbidities. Two patients (3 flaps) required hip disarticulation, 1 patient had a bilateral fillet flaps, and 3 patients had resection of tibia/fibula. After following patients for an average of 1.4 years (4 mo to 2 yr), complications were limited to 1 patient who had partial-thickness flap loss at the distal skin flap that healed by secondary intention and 1 patient who had ulcer recurrence because of noncompliance. Four articles met inclusion criteria for systematic review and 3 were excluded.
CONCLUSIONS
The fillet of leg flap remains a useful and reliable method of reconstructing end-stage pressure ulcers.
PubMed: 27622082
DOI: 10.1097/GOX.0000000000001001 -
American Journal of Physical Medicine &... Feb 2024The aim of the study is to examine the effectiveness of extracorporeal shockwave therapy in reducing pain, improving functionality, joint range of motion, quality of... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The aim of the study is to examine the effectiveness of extracorporeal shockwave therapy in reducing pain, improving functionality, joint range of motion, quality of life, fatigue, and health status self-perception in people with myofascial pain syndrome.
METHODS
PubMed, the Cochrane Library, CINAHL, the Physiotherapy Evidence Database, and SPORTDiscus were systematically searched for only randomized clinical trials published up to June 2, 2022. The main outcome variables were pain, as reported on the visual analog scale and pressure pain threshold, and functionality. A quantitative analysis was conducted using the inverse variance method and the random effects model.
RESULTS
Twenty-seven studies were included ( N = 595 participants in the extracorporeal shockwave therapy group). The effectiveness of extracorporeal shockwave therapy for relieving pain was superior for the extracorporeal shockwave therapy group compared with the control group on the visual analog scale (MD = -1.7 cm; 95% confidence interval = -2.2 to -1.1) and pressure pain threshold (mean difference = 1.1 kg/cm 2 ; 95% confidence interval = 0.4 to 1.7) and functionality (standardized mean difference = -0.8; 95% confidence interval = -1.6 to -0.04) with high heterogeneity. However, no differences were found between extracorporeal shockwave therapy and other interventions as dry needling, exercises, infiltrations, and lasers interventions.
CONCLUSIONS
Extracorporeal shockwave therapy is effective in relieving pain and improving functionality in patients with myofascial pain syndrome compared with control and ultrasound therapy.
TO CLAIM CME CREDITS
Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCME.
TO CLAIM CME CREDITS
Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCME.
CME OBJECTIVES
Upon completion of this article, the reader should be able to: (1) Determine the effectiveness of radial and focal extracorporeal shockwaves on pain perception, the pressure pain threshold, and functionality in people with myofascial pain syndrome; (2) Describe the intervention protocol of extracorporeal shockwave therapy to improve pain perception in people with myofascial pain syndrome; and (3) Describe the advantages and disadvantages of extracorporeal shockwave therapy versus other intervention such as dry needling.
LEVEL
Advanced.
ACCREDITATION
The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s) ™. Physicians should only claim credit commensurate with the extent of their participation in the activity.
LEVEL
Advanced.
ACCREDITATION
The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The Association of Academic Physiatrists designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s) ™. Physicians should only claim credit commensurate with the extent of their participation in the activity.
Topics: Humans; Extracorporeal Shockwave Therapy; Fibromyalgia; Myofascial Pain Syndromes; Pain; Quality of Life; Randomized Controlled Trials as Topic
PubMed: 37205742
DOI: 10.1097/PHM.0000000000002286 -
The Neurologist Jan 2017Spinal subdural hematomas (SSDHs) are rare. Causes are (1) posttraumatic, (2) iatrogenic (following surgery or lumbar puncture), (3) spontaneous including underlying... (Review)
Review
OBJECTIVE
Spinal subdural hematomas (SSDHs) are rare. Causes are (1) posttraumatic, (2) iatrogenic (following surgery or lumbar puncture), (3) spontaneous including underlying malformations or coagulation deficits. With a systematic review of literature we want to shed light on the last group: symptomatology, etiology, treatment and outcome will we discussed.
METHODS
Systematic review of literature on PubMed for cases of acute nontraumatic noniatrogenic SSDHs in adults (≥18 y of age). A total of 122 cases were reviewed including 2 cases from our hospital.
RESULTS
There was a slight preponderance of female patients with spontaneous SSDHs and the mean age was 60 years. Spontaneous SSDHs were mostly located in the thoracic region (40%). Motor symptoms were most frequent (89%), followed by pain. Sensory deficits were present in 64%, of which 81% had a sensory level. In 6% radiculating pain, without any focal neurological deficits, was the presenting symptom. SSDHs were mainly caused by a coagulopathy (48%), predominantly due to the use of coumarins (34%). Other causes were underlying (vascular) malformations and vasculitis. Forty-three percent the SSDHs was idiopathic. 72% of patients underwent a decompressive laminectomy. 59% had a favorable outcome and 34% had a poor outcome.
CONCLUSIONS
Spontaneous SSDHs were predominantly located in the thoracic spine, presenting with paraparesis/paraplegia, sensory level and pain. Over 40% was caused by a coagulation defect, most frequently due to coumarins. Six percent of patients presented with radiculating pain without any focal neurological deficits.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Hematoma, Subdural, Spinal; Humans; Male; Middle Aged; Young Adult
PubMed: 28009771
DOI: 10.1097/NRL.0000000000000100 -
Journal of Vascular Surgery Jan 2020The purpose of this study was to compare clinical outcomes between open repair and thoracic endovascular aortic repair (TEVAR) in traumatic ruptured thoracic aorta. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The purpose of this study was to compare clinical outcomes between open repair and thoracic endovascular aortic repair (TEVAR) in traumatic ruptured thoracic aorta.
METHODS
A comprehensive search was undertaken of the four major databases (PubMed, Embase, Scopus, and Ovid) to identify all published data comparing open vs endovascular repair. Databases were evaluated to July 2018. Odds ratios (ORs), weighted mean differences, or standardized mean differences and their 95% confidence intervals (CIs) were analyzed. The primary outcomes were stroke, paraplegia, and 30-day mortality rates; secondary outcomes were requirement for reintervention and 1-year and five-year mortality rates.
RESULTS
A total of 1968 patients were analyzed in 21 articles. TEVAR was performed in 29% (n = 578) and open repair in 71% (n = 1390). TEVAR and open repair did not differ in the mean age of patients (42.1 ± 14 years vs 44.1 ± 14 years; P = .48). There was no difference in duration of intensive care and total hospital stay between TEVAR and open repair groups (12.7 ± 11.1 days vs 12.6 ± 8 days [P = .35] and 27.5 ± 14.6 days vs 25.9 ± 11 days [P = .80], respectively). Similarly, no statistically significant difference in postoperative paraplegia or stroke rate was noted between TEVAR and open repair (1.4% vs 2.3% [OR, 1.27; 95% CI, 0.59-2.70; P = .54] and 1% vs 0.5% [OR, 0.63; 95% CI, 0.18-2.18; P = .46]). Lower 30-day and 1-year mortality was noted in TEVAR (7.9% vs 20% [OR, 2.94; 95% CI, 1.92-4.49; P < .00001] and 8.7% vs 17% [OR, 2.11; 95% CI, 0.99-4.52; P = .05]). There was no difference in 5-year mortality (23% vs 17%; OR, 0.07; 95% CI, -0.07 to 0.20; P = .33). However, there was a higher rate of reintervention at 1 year in the endovascular group (0% vs 6%; OR, 0.17; 95% CI, 0.03-0.96; P = .04).
CONCLUSIONS
TEVAR carries lower in-hospital mortality and provides satisfactory perioperative outcomes compared with open repair in traumatic ruptured thoracic aorta. It also provides a favorable 1-year survival at the expense of higher reintervention rates.
Topics: Adult; Aorta, Thoracic; Aortic Rupture; Blood Vessel Prosthesis Implantation; Endovascular Procedures; Female; Hospital Mortality; Humans; Male; Middle Aged; Postoperative Complications; Reoperation; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome; Vascular System Injuries
PubMed: 31327611
DOI: 10.1016/j.jvs.2019.05.011 -
The Journal of Cardiovascular Surgery Oct 2010Acute complicated type B aortic dissection is a life-threatening condition. We summarized all published studies for TEVAR among patients with acute complicated typ B... (Meta-Analysis)
Meta-Analysis Review
Outcome of patients with open and endovascular repair in acute complicated type B aortic dissection: a systematic review and meta-analysis of case series and comparative studies.
AIM
Acute complicated type B aortic dissection is a life-threatening condition. We summarized all published studies for TEVAR among patients with acute complicated typ B aortic dissection (TBAD) with respect to clinical success, complications, and outcomes. Furthermore, we determined whether TEVAR reduces death and morbidity compared with open repair for TBAD.
METHODS
Studies were identified from a literature search using various databases, and included studies when three or more patients were reported and at least in-hospital mortality was reported. Data from comparative studies of TEVAR versus open repair of the descending aorta in TBAD were combined through meta-analysis.
RESULTS
Seventy-six observational studies involving 1951 patients were included in the present meta-analysis. In-hospital mortality was 11.5% and other major complications (i.e., stroke (6.3%), paraplegia (4.9%), retrograde type A aortic dissection (7%), renal impairment including dialysis (6.9%), bowel infarction (4.1%), vascular problems including major amputation (2.5%)) occurred less frequently. Long-term follow-up was limited to a mean of 24 months. During this time, endovascular reintervention was required in 11.3%, surgical reintervention in 7.7%, and late aortic rupture was calculated for 3.2% of cases. A complete false lumen thrombosis was estimated to occur in 76.1% of cases. In comparative studies, 30-day/in-hospital mortality (OR=0.256, P=0.001) and paraplegia/paraparesis (OR=0.256, P=0.001) were significantly reduced for TEVAR versus open repair. In addition to that, the rate of vascular complications was reduced for TEVAR (OR=0.373, P=0.036). There was no significant difference between TEVAR and open repair in patients with acute complicated TBAD for the following outcomes: late mortality, reintervention rate, renal dysfunction (including dialysis), and stroke rate.
CONCLUSION
This summary analysis suggests that endovascular treatment of complicated acute type B aortic dissection produces favourable initial outcomes and would seem to be a great addition to the treatment options for this condition. Further study of long-term outcomes is required.
Topics: Aortic Dissection; Aortic Aneurysm; Aortic Rupture; Blood Vessel Prosthesis Implantation; Endovascular Procedures; Hospital Mortality; Humans; Kidney Diseases; Odds Ratio; Paraplegia; Reoperation; Risk Assessment; Risk Factors; Stroke; Time Factors; Treatment Outcome
PubMed: 20924323
DOI: No ID Found