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Transverse myelitis in systemic lupus erythematosus: A case report and systematic literature review.Autoimmunity Reviews Jun 2022Acute transverse myelitis (TM) is a rare complication secondary to systemic lupus erythematosus (SLE) that can cause patients' extensive and severe neuropsychiatric... (Review)
Review
OBJECTIVE
Acute transverse myelitis (TM) is a rare complication secondary to systemic lupus erythematosus (SLE) that can cause patients' extensive and severe neuropsychiatric disorders. Due to the rarity of the onset of acute TM, there is still no standard treatment protocol. This study was to summarize the clinical features of SLE-TM through a case report and systematic review.
METHODS
We report a case of acute TM with the initial symptoms of headache and fever on admission to hospital, with lesions in medulla oblongata, cervical medulla, and thoracic medulla. Furthermore, all cases of SLE combined with acute TM from January 1975 to February 2022 were concluded and reviewed to compare the disease's current treatment strategies and prognosis.
RESULTS
Patients with SLE-TM are mainly female (97.65%), with an average age of 36.89, a TM incidence of 24.51% and a longitudinal myelitis (LM) incidence of 67.76%. In addition, 68.63% of patients present an increased albumin, and only 16.50% of patients could recover. 32.35% of patients showed positive anti-cardiolipin antibody. Moreover, the patients who could recover are generally younger than those in the improved and paraparesis groups. After classifying the statistical results twice according to magnetic resonance imaging results and prognosis respectively, the erythrocyte sedimentation rate (ESR) in LM group was significantly higher than that in the other two groups. The positive rate of anti-DNA and anti-cardiolipin antibody (ANCL) in TM group was significantly higher than that of the other groups. According to the prognostic grouping, ESR in the recovery group was significantly higher than those in the other two groups. The positive ANCL in the poor prognosis group was slightly higher than that in the other two groups.
CONCLUSION
We offer a novel insight for this rare disease and hope to bring some inspiration the basic research for SLE-TM.
Topics: Adult; Antibodies, Anticardiolipin; Female; Humans; Lupus Erythematosus, Systemic; Magnetic Resonance Imaging; Myelitis, Transverse; Prognosis
PubMed: 35452852
DOI: 10.1016/j.autrev.2022.103103 -
International Journal of Surgery... Nov 2020Current treatment approaches for acute type B aortic dissection (TBAD) are diversified. Thoracic endovascular aortic repair (TEVAR) as an effective and convenient... (Comparative Study)
Comparative Study Meta-Analysis
Comparison of the efficacy and safety of thoracic endovascular aortic repair with open surgical repair and optimal medical therapy for acute type B aortic dissection: A systematic review and meta-analysis.
BACKGROUND
Current treatment approaches for acute type B aortic dissection (TBAD) are diversified. Thoracic endovascular aortic repair (TEVAR) as an effective and convenient intervention has been adopted extensively. However, the superior efficacy and safety of TEVAR have not yet been well evaluated. This meta-analysis was designed to comprehensively compare the efficacy and safety of TEVAR with open surgical repair and optimal medical therapy for acute type B aortic dissection.
METHODS
A systematic search of PubMed, Embase, Cochrane Library and Web of Science up to April 1, 2020 was conducted for relevant studies that compared the efficacy of TEVAR and other conventional interventions in the treatment of TBAD. The primary outcomes were early mortality and midterm or long term survival. The secondary outcomes included early complications and other late outcomes. Two reviewers assessed trial quality and extracted the data independently. All statistical analyses were performed using the standard statistical procedures provided in Review Manager 5.2.
RESULTS
A total of 18 studies including 12,789 patients were identified. 30-day/in-hospital mortality was significantly lower in TBAD patients with TEVAR than open surgical repair (OSR), with a pooled OR of 0.54 (95% CI 0.43-0.68; P < 0.00001). Compared with optimal medical therapy (OMT), TEVAR experienced lower incidence of long-term death (≥5-yr mortality), with a pooled OR of 0.46 (95% CI 0.24-0.86; P = 0.02). However, no significant difference between TEVAR and OSR or OMT in long-term survival was found. Compared with OSR, lower incidence of cardiac and pulmonary complications as well as shorter length of stay were observed in TEVAR. Compared with OMT, TEVAR showed higher rate of paraplegia or paraparesis, higher complete thrombosis of the false lumen, as well as longer length of ICU stay.
CONCLUSIONS
Our analysis shows that TEVAR may be favorable in reducing 30-day/in-hospital mortality (than OSR) and long-term mortality (than OMT). TEVAR experienced equal efficacy with OSR and OMT in long-term survival. TEVAR showed higher rate of paraplegia or paraparesis, higher complete thrombosis of the false lumen, as well as longer length of ICU stay than OMT; and lower incidence of cardiac and pulmonary complications as well as shorter length of stay than OSR. However, TEVAR indicated similar incidence of other complications and outcomes with OSR and OMT. Further studies especially randomized clinical trials are needed to comprehensively compare the efficacy TEVAR.
Topics: Adult; Aged; Aortic Dissection; Aorta, Thoracic; Aortic Aneurysm, Thoracic; Endovascular Procedures; Female; Hospital Mortality; Humans; Male; Middle Aged
PubMed: 32927144
DOI: 10.1016/j.ijsu.2020.08.051 -
Neurology(R) Neuroimmunology &... May 2021To define the characteristics and the outcome of myelitis associated with immune checkpoint inhibitors (ICIs).
OBJECTIVE
To define the characteristics and the outcome of myelitis associated with immune checkpoint inhibitors (ICIs).
METHODS
We performed a retrospective research in the databases of the French Pharmacovigilance Agency and the OncoNeuroTox network for patients who developed myelitis following treatment with ICIs (2011-2020). A systematic review of the literature was performed to identify similar cases.
RESULTS
We identified 7 patients who developed myelitis after treatment with ICIs (anti-PD1 [n = 6], anti-PD1 + anti-CTLA4 [n = 1]). Neurologic symptoms included paraparesis (100%), sphincter dysfunction (86%), tactile/thermic sensory disturbances (71%), and proprioceptive ataxia (43%). At the peak of symptom severity, all patients were nonambulatory. MRI typically showed longitudinally extensive lesions, with patchy contrast enhancement. CSF invariably showed inflammatory findings. Five patients (71%) had clinical and/or paraclinical evidence of concomitant cerebral, meningeal, caudal roots, and/or peripheral nerve involvement. Despite the prompt discontinuation of ICIs and administration of high-dose glucocorticoids (n = 7), most patients needed second-line immune therapies (n = 5) because of poor recovery or early relapses. At last follow-up, only 3 patients had regained an ambulatory status (43%). Literature review identified 13 previously reported cases, showing similar clinical and paraclinical features. All patients discontinued ICIs and received high-dose glucocorticoids, with the addition of other immune therapies in 8. Clinical improvement was reported for 10 patients.
CONCLUSION
Myelitis is a rare but severe complication of ICIs that shows limited response to glucocorticoids. Considering the poor functional outcome associated with longitudinally extensive myelitis, strong and protracted immune therapy combinations are probably needed upfront to improve patient outcome and prevent early relapses.
Topics: Adolescent; Adult; Aged; Female; Glucocorticoids; Humans; Immune Checkpoint Inhibitors; Immunotherapy; Magnetic Resonance Imaging; Male; Middle Aged; Myelitis; Neoplasms; Retrospective Studies
PubMed: 33637598
DOI: 10.1212/NXI.0000000000000967 -
Journal of Endovascular Therapy : An... Apr 2022The comparisons between thoracic endovascular aortic repair for ruptured thoracic aorta (TEVAR) and open surgery (OS) have not been well documented, although both... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The comparisons between thoracic endovascular aortic repair for ruptured thoracic aorta (TEVAR) and open surgery (OS) have not been well documented, although both procedures have been widely utilized. We performed a systematic review and meta-analysis to investigate the effectiveness and safety between TEVAR and OS in the repair of ruptured descending thoracic aorta.
METHODS
PubMed, Embase, and Cochrane Library databases were searched to find relevant studies to assess TEVAR and OS outcomes. The comparative parameters were perioperative mortality (30 day/in-hospital), 1 year mortality, paraplegia or paraparesis, renal insufficiency, stroke, pulmonary embolism, re-intervention rate, pulmonary complications, and cardiac complications. A fixed-effects model was applied to calculate the odds ratio (OR) with a 95% confidence interval (CI) on pooled outcomes from different studies.
RESULTS
Eighteen observational trials involving 2088 patients were evaluated (TEVAR=560; OS=1528). Meta-analysis showed that TEVAR in repairing the ruptured descending thoracic aorta was associated with lower perioperative mortality (OR=0.47; 95% CI: 0.34-0.66; p<0.01), 1 year mortality (OR=0.46; 95% CI: 0.29-0.75; p<0.01), renal insufficiency incidence (OR=0.56; 95% CI: 0.33-0.93; p=0.03), and pulmonary complications (OR=0.69; 95% CI: 0.52-0.92; p=0.01) when compared with OS. There was no significant difference between TEVAR and OS in terms of paraplegia, stroke, pulmonary embolism, cardiac complications, and early re-intervention rates. However, the late re-intervention rate was higher in the TEVAR group than that in the OS group.
CONCLUSIONS
When repairing the ruptured descending thoracic aorta, TEVAR may be performed rapidly and safely. TEVAR is associated with lower rates of perioperative morbidity and early postoperative complications than OS.
Topics: Aorta, Thoracic; Aortic Aneurysm, Thoracic; Blood Vessel Prosthesis Implantation; Endovascular Procedures; Humans; Odds Ratio; Postoperative Complications; Retrospective Studies; Risk Factors; Treatment Outcome
PubMed: 34779300
DOI: 10.1177/15266028211057087 -
Journal of Neurology Jan 2022Monoallelic variants in the KIF1A gene are associated with a large set of clinical phenotypes including neurodevelopmental and neurodegenerative disorders, underpinned...
BACKGROUND
Monoallelic variants in the KIF1A gene are associated with a large set of clinical phenotypes including neurodevelopmental and neurodegenerative disorders, underpinned by a broad spectrum of central and peripheral nervous system involvement.
METHODS
In a multicenter study conducted in patients presenting spastic gait or complex neurodevelopmental disorders, we analyzed the clinical, genetic and neuroradiological features of 28 index cases harboring heterozygous variants in KIF1A. We conducted a literature systematic review with the aim to comparing our findings with previously reported KIF1A-related phenotypes.
RESULTS
Among 28 patients, we identified nine novel monoallelic variants, and one a copy number variation encompassing KIF1A. Mutations arose de novo in most patients and were prevalently located in the motor domain. Most patients presented features of a continuum ataxia-spasticity spectrum with only five cases showing a prevalently pure spastic phenotype and six presenting congenital ataxias. Seventeen mutations occurred in the motor domain of the Kinesin-1A protein, but location of mutation did not correlate with neurological and imaging presentations. When tested in 15 patients, muscle biopsy showed oxidative metabolism alterations (6 cases), impaired respiratory chain complexes II + III activity (3/6) and low CoQ10 levels (6/9). Ubiquinol supplementation (1gr/die) was used in 6 patients with subjective benefit.
CONCLUSIONS
This study broadened our clinical, genetic, and neuroimaging knowledge of KIF1A-related disorders. Although highly heterogeneous, it seems that manifestations of ataxia-spasticity spectrum disorders seem to occur in most patients. Some patients also present secondary impairment of oxidative metabolism; in this subset, ubiquinol supplementation therapy might be appropriate.
Topics: Cross-Sectional Studies; DNA Copy Number Variations; Heterozygote; Humans; Kinesins; Mutation; Phenotype; Spastic Paraplegia, Hereditary
PubMed: 34487232
DOI: 10.1007/s00415-021-10792-3 -
Burns : Journal of the International... Mar 2020Direct current (DC) powered equipment and devices, including photovoltaic systems, high-voltage direct current power lines and novel concepts in electromobility have...
BACKGROUND
Direct current (DC) powered equipment and devices, including photovoltaic systems, high-voltage direct current power lines and novel concepts in electromobility have become increasingly popular in recent years. However, under adverse circumstances by malfunction or mishandling of these applications electrical injuries may occur when electric current passes through the human body. This review aimed at systematically summarizing the medical consequences of DC electrical injuries described in case reports and case series.
METHODS
The Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guided the methodological conduct and reporting.
RESULTS
Sixteen case reports and 3 case series were eligible for this review and included 70 patients. The reviewed articles were very heterogeneous regarding sources of DC electrical injuries and the reported medical consequences, including burns and skin lesions (n=67), neurological consequences (n=11), unconsciousness (n=10), cardiac consequences (n=8) and bone fractures (n=6). Seventeen individuals did not survive the electrical injuries.
CONCLUSION
From the few available data and partly incomplete documentations of cases we could gather hints of DC medical consequences, however, it was not possible to identify well-defined medical consequences for various circumstances of DC electrical injuries in occupational and non-occupational settings. To achieve this goal, additional studies are required, each providing a comprehensive description of the medical consequences and the circumstances of the electrical injuries.
Topics: Adolescent; Adult; Age Distribution; Arrhythmias, Cardiac; Brain Concussion; Burns, Electric; Child; Electric Injuries; Female; Fractures, Bone; Headache; Heart Arrest; Humans; Infant, Newborn; Lethargy; Male; Middle Aged; Nervous System Diseases; Occupational Injuries; Paraparesis; Paraplegia; Pregnancy; Psychotic Disorders; Sex Distribution; Unconsciousness; Young Adult
PubMed: 31208768
DOI: 10.1016/j.burns.2018.11.020 -
Spontaneous Spinal Hematoma in Patients Using Antiplatelets and Anticoagulants: A Systematic Review.World Neurosurgery Apr 2024Spontaneous spinal hematoma (SSH) is a debilitating complication in patients taking either antiplatelet (AP) or anticoagulation (AC) medications. SSH is rare and,...
BACKGROUND
Spontaneous spinal hematoma (SSH) is a debilitating complication in patients taking either antiplatelet (AP) or anticoagulation (AC) medications. SSH is rare and, therefore, a systematic review is warranted to re-examine and outline trends, clinical characteristics, and outcomes associated with SSH formation.
METHODS
PubMed, EMBASE, Scopus, and Web-of-Science were searched. Studies reporting clinical data of patients with SSH using AC medications were included. In addition, clinical studies meeting our a priori inclusion criteria limited to SSH were further defined in quality through risk bias assessment.
RESULTS
We included 10 studies with 259 patients' pooled data post-screening 3083 abstracts. Within the cohort (n = 259), the prevalence of idiopathic, nontraumatic SSH with concomitant treatment with AC medications was greater 191 (73.75%) compared with AP treatment (27%). The lumbar spine was the most common site of hematoma (41.70%), followed by the cervical (22.01%) and thoracic (8.49%) spine. Most patients had surgical intervention (70.27%), and 29.73% had conservative management. The pooled data suggest that immediate diagnosis and intervention are the best prognostic factors in clinical outcomes. American Spinal Injury Association grading at initial symptom onset and post-treatment showed the greatest efficacy in symptomatic relief (87.64%) and return of motor and sensory symptoms (39.19%).
CONCLUSIONS
Our review suggested that AC medications were related to SSH in most patients (74%), followed by APs (27%) and combined ACs + APs (1.9%). We recommend prompt intervention, a high suspicion for patients with neurologic deficits and diagnostic imaging before intervention to determine a case-specific treatment plan.
Topics: Humans; Anticoagulants; Hematoma, Epidural, Spinal; Spinal Cord Diseases; Lumbar Vertebrae; Risk Assessment; Magnetic Resonance Imaging
PubMed: 38278210
DOI: 10.1016/j.wneu.2024.01.082 -
Movement Disorders Clinical Practice Jun 2024As the diagnosis of Parkinson's disease (PD) is fundamentally clinical, the usefulness of ioflupane (I) single-photon emission computed tomography (SPECT) or DaTSCAN as... (Review)
Review
BACKGROUND
As the diagnosis of Parkinson's disease (PD) is fundamentally clinical, the usefulness of ioflupane (I) single-photon emission computed tomography (SPECT) or DaTSCAN as a diagnostic tool has been a matter of debate for years. The performance of DaTSCAN is generally recommended in the follow-up of patients with a clinically uncertain diagnosis, especially in those with a suspected essential tremor, drug-induced parkinsonism, or vascular parkinsonism. However, there is a dearth of DaTSCAN findings regarding neurodegenerative parkinsonisms besides PD and atypical parkinsonisms. To date, a specific nigrostriatal dopamine uptake pattern that would help differentiate PD from the most frequent atypical parkinsonisms is yet to be described. This fact is further complicated by the possible visualization of abnormalities in the uptake pattern in patients with rarer neurodegenerative parkinsonisms.
OBJECTIVES
We aimed to summarize the current literature regarding DaTSCAN findings in patients with rare neurodegenerative parkinsonisms.
METHODS
The PubMed database was systematically screened for studies in English or Spanish up to October 15, 2023, using search terms "DaTSCAN", "ioflupane", "DaT-SPECT", "123I-FP-CIT SPECT", "dopamine transporter imaging", and "[123I] FP-CIT SPECT". Duplicated publications and studies regarding PD, atypical parkinsonisms, dystonia-parkinsonism, essential tremor, and parkinsonism due to non-degenerative causes were excluded.
RESULTS
The obtained results were reviewed and summarized, including DaTSCAN findings in fragile X-associated tremor/ataxia syndrome, prion diseases, Huntington's disease, spinocerebellar ataxia, hereditary spastic paraparesis, metabolic disorders, and other diseases (anti-IgLON5 disease, ring chromosome 20 syndrome, chorea-acanthocytosis, and neuronal ceroid lipofuscinosis).
CONCLUSIONS
This review highlights the need to determine in the future the utility and cost-effectiveness of DaTSCAN, both as a diagnostic and a prognostic tool, in patients with parkinsonian symptoms in rare neurodegenerative diseases.
Topics: Humans; Tomography, Emission-Computed, Single-Photon; Parkinsonian Disorders; Tropanes; Parkinson Disease
PubMed: 38693679
DOI: 10.1002/mdc3.14055 -
Neurosurgical Review May 2024Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which... (Comparative Study)
Comparative Study
Surgery and endovascular therapy are the primary treatment options for spinal dural arteriovenous fistula (SDAVF). Due to the absence of a consensus regarding which therapy yields a superior outcome, we conducted a comparative analysis of the surgical and endovascular treatment of SDAVF through a multicenter case series and a systematic literature review. Patients with SDAVF, surgically or endovascularly treated at four neurosurgical centers from January 2001 to December 2021, were included in this study. Level of SDAVF, primary treatment modality, baseline and post-procedural neurological status were collected. The primary outcomes were failure, complication rates, and a newly introduced parameter named as therapeutic delay. A systematic review of the literature was performed according to PRISMA-P guidelines. The systematic review identified 511 papers, of which 18 were eligible for analysis, for a total of 814 patients, predominantly male (72%) with a median age of 61 and mainly thoracic SDAVFs (65%). The failure rate was significantly higher for endovascular therapy (20%) compared to surgery (4%) (p < 0.01). Neurological complications were generally rare, with similar rates among the two groups (endovascular 2.9%; surgery 2.6%). Endovascular treatment showed a statistically significantly higher rate of persistent neurological complications than surgical treatment (2.9% versus 0.2%; p < 0.01). Both treatments showed similar rates of clinical improvement based on Aminoff Logue scale score. The multicenter, retrospective study involved 131 patients. The thoracic region was the most frequent location (58%), followed by lumbar (37%). Paraparesis (45%) and back pain (41%) were the most common presenting symptoms, followed by bladder dysfunction (34%) and sensory disturbances (21%). The mean clinical follow-up was 21 months, with all patients followed for at least 12 months. No statistically significant differences were found in demographic and clinical data, lesion characteristics, or outcomes between the two treatment groups. Median pre-treatment Aminoff-Logue score was 2.6, decreasing to 1.4 post-treatment with both treatments. The mean therapeutic delay for surgery and endovascular treatment showed no statistically significant difference. Surgical treatment demonstrated significantly lower failure rates (5% vs. 46%, p < 0.01). In the surgical group, 2 transient neurological (1 epidural hematoma, 1 CSF leak) and 3 non-neurological (3 wound infections) complications were recorded; while 2 permanent neurological (spinal infarcts), and 5 non-neurological (inguinal hematomas) were reported in the endovascular group. According to the literature review and this multicenter clinical series, surgical treatment has a significantly lower failure rate than endovascular treatment. Although the two treatments have similar complication rates, endovascular treatment seems to have a higher rate of persistent neurological complications.
Topics: Humans; Central Nervous System Vascular Malformations; Endovascular Procedures; Neurosurgical Procedures; Male; Female; Middle Aged; Treatment Outcome; Aged; Postoperative Complications; Embolization, Therapeutic
PubMed: 38713376
DOI: 10.1007/s10143-024-02443-8 -
Clinical and Experimental Pediatrics Jul 2023The peripartum period is both a highly vulnerable stage and a significant indicator of a population's health status. Interest is increasing in human T-cell lymphotropic...
BACKGROUND
The peripartum period is both a highly vulnerable stage and a significant indicator of a population's health status. Interest is increasing in human T-cell lymphotropic virus type-1 (HTLV-1) transmission due to its adverse health impacts. However, nationally representative data on HTLV-1 that are important for health planning are unavailable for this subpopulation.
PURPOSE
This study aimed to conduct a pooled estimate of HTLV-1 prevalence among pregnant women in Nigeria to quantify its clinical burden and public health implications.
METHODS
This systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis 2020 statement.
RESULTS
After a systematic review of the Nigerian literature, 12 studies (2,821 pregnant or postnatal women) were included in the final evidence synthesis. The estimated HTLV-1 prevalence in Nigerian peripartum women following a positive screening test by enzyme-linked immunosorbent assay was 5.44% (95% confidence interval [CI], 3.16%-9.20%). A subgroup analysis of the 2 major regions showed a slightly higher prevalence in the Western versus Southern region (5.55% [95% CI, 2.49%-11.87%]; and 4.91% [95% CI, 2.11%-11.02%]; P=0.84). However, a subgroup analysis by geopolitical zone revealed that Southwestern and Northwestern Nigeria had the highest prevalence (9.23% [95% CI, 4.35%-18.55%; I2=93%] and 7.15% [95% CI, 1.54%-27.54%]; I2=92%). Our decade-old subgroup analysis found inconsistencies in the HTLV-1 prevalence. Furthermore, our literature review revealed a prevalence of HTLV infection among patients with various clinical types of lymphomas/leukemias and myelopathy of 2%-22%.
CONCLUSION
These findings have important implications in defining the epidemiological patterns of HTLV-1 infection in Nigeria. They also suggest the presence of HTLV-endemic clusters near low-endemic areas, even within the same geopolitical zones.
PubMed: 36550773
DOI: 10.3345/cep.2022.00710