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Journal of Neurology Sep 2023Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are progressive neurodegenerative syndromes characterised by Parkinsonism with additional features... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are progressive neurodegenerative syndromes characterised by Parkinsonism with additional features including cognitive dysfunction, falls, and oculomotor abnormalities. Understanding the epidemiology of these conditions is critical to planning for future service provision.
METHODS
We conducted a systematic review of studies reporting incidence and prevalence of CBS and PSP. A search of the PubMed and EMBASE data bases was conducted from their date of inception to 13th July 2021. Meta-analysis of studies sharing similar methodologies was carried out to generate estimated pooled prevalence and incidence.
RESULTS
We found 32 studies meeting our criteria for inclusion. There were 20 studies with data on prevalence and 12 with incidence data of PSP. Prevalence of CBS was reported in eight studies while seven studies reported incidence. Reported estimates of prevalence for PSP ranged from 1.00 (0.9-1.1) to 18 (8-28) per 100,000 while prevalence rates for CBS ranged from 0.83 (0.1-3.0) to 25 (0-59). Incidence rates for PSP and CBS respectively ranged from 0.16 (0.07-0.39) to 2.6 per 100,000 person-years and 0.03 (0-0.18) to 0.8 (0.4-1.3) per 100,000 person-years. A random effects model meta-analysis of studies with similar methodologies yielded a pooled prevalence estimate for PSP of 6.92 (4.33-11.06, I = 89%, τ = 0.3907) and 3.91 (2.03-7.51, I = 72%, τ = 0.2573) per 100,000 for CBS.
CONCLUSION
Studies of the epidemiology of PSP and CBS report highly heterogeneous findings. There is a need for further studies using rigorous phenotyping and the most recent diagnostic criteria to understand the true burden of these conditions.
Topics: Humans; Supranuclear Palsy, Progressive; Incidence; Corticobasal Degeneration; Prevalence; Syndrome
PubMed: 37289323
DOI: 10.1007/s00415-023-11791-2 -
Complementary Therapies in Medicine Dec 2023This study aimed to employ bibliometric approaches to assess the worldwide scientific achievements in acupuncture for facial paralysis research from 2013 to 2023, and... (Review)
Review
OBJECTIVE
This study aimed to employ bibliometric approaches to assess the worldwide scientific achievements in acupuncture for facial paralysis research from 2013 to 2023, and explore the hotspots and frontiers.
METHODS
Articles related to acupuncture facial paralysis were retrieved from the Web of Science Core Collection (WoSCC). CiteSpace was utilized to examine journals, publication year, country, institution, cited authors, as well as authors.Research hotspots and trends were analyzed by mapping co-citation networks and keywords.
RESULTS
The period from January 1, 2013, to July 31, 2023, yielded nearly 145 research records on acupuncture treatment for facial paralysis, with a steady increase in the number of annual publications.In terms of the number of publications, OTOLARYNGOLOGY HEAD NECK had the highest publication count, while AM J CHINESE MED exhibited the highest centrality and citation frequency among the cited journals. Further, 54.4 % of publications originated from China, followed by USA (8.2 %) and Germany (8.2 %). Guangzhou University of Chinese Medicine stood out with the highest publication volume among institutions. Guntinas-lichius, Orlando was the most prolific author, and PEITERSEN E was the most cited author. The keywords "Randomized controlled trials" and "multicenter" displayed high frequency and centrality, indicating that clinical trials with a randomized controlled design and multicenter studies were prevalent research methods, likely to remain a future trend.
CONCLUSION
Acupuncture's potential in the treatment of facial paralysis merits further research. Authors from different countries/regions and organizations need to eliminate language and academic barriers and strengthen collaboration and communication. Current research hotspots focus on "brain", "nerve", "electrical stimulation", "RCT" and "guidelines". The study of acupuncture mechanisms, especially based on the central nervous system mechanism, may be the future research hotspot.
Topics: Humans; Facial Paralysis; Acupuncture Therapy; Asian People; Bibliometrics; Brain
PubMed: 37972694
DOI: 10.1016/j.ctim.2023.103006 -
BMC Neurology Sep 2023Paralysis of the facial nerve (CN VII) is one of the most debilitating issues that any patient can encounter. Bell's palsy is the most commonly seen mononeuropathy....
BACKGROUND
Paralysis of the facial nerve (CN VII) is one of the most debilitating issues that any patient can encounter. Bell's palsy is the most commonly seen mononeuropathy. Although usually self-limited, symptomatology can persist for decades in persistent cases. The non-surgical alternative therapies discussed in this study are successful without reconstruction and are regenerative.
OBJECTIVE AND DESIGN
We sought to determine a safe new treatment could be developed to restore facial nerve function using extracellular vesicles (EVs) in patients who have been unable to return to normal under a variety of conditions. We performed a pilot safety study of 7 patients with idiopathic and secondary facial paralysis to determine if any functional restoration was possible. Each patient had symptomology for varying periods of time, with diverse House-Brackmann scores. They were all treated with the same protocol of extracellular vesicles (EVs) over a 4-week period of time and were evaluated both before and after treatment.
CASE PRESENTATIONS
All patients in this study received treatment by their private physicians prior to entering the study. A record review was completed, with independent physical examinations. House-Brackmann scores and Facial Disability Indices were obtained prior to, and after completing the study. EVs were injected into the area of the main trunk of the facial nerve on the affected side, and an intravenous drip of EVs on visits during weeks 1, 2, and 4.
CONCLUSIONS
All seven patients enrolled in the study improved with this treatment protocol. After the second week of treatment, we saw a progression of independent motion of the affected eyelid, brow motion, and commissure. Although all patients began at different House-Brackman starting points, almost all ended at the same endpoint on the scale over the same period of time - four weeks. No adverse effects were encountered. Clearly, the duration of the treatment protocol needs to be longer than one month. The pathomechanism is still unknown. But it appears that the mechanism is reversible. At last, these patients can have hope.
TRIAL REGISTRATION
The Institute of Regenerative and Cellular Medicine IRB approval number: IRCM-2021-304.
Topics: Humans; Bell Palsy; Facial Paralysis; Facial Nerve; Acupuncture Therapy; Extracellular Vesicles
PubMed: 37770834
DOI: 10.1186/s12883-023-03400-6 -
Nature Communications Nov 2023Progressive supranuclear palsy (PSP) is a neurodegenerative parkinsonian disorder characterized by cell-type-specific tau lesions in neurons and glia. Prior work...
Progressive supranuclear palsy (PSP) is a neurodegenerative parkinsonian disorder characterized by cell-type-specific tau lesions in neurons and glia. Prior work uncovered transcriptome changes in human PSP brains, although their cell-specificity is unknown. Further, systematic data integration and experimental validation platforms to prioritize brain transcriptional perturbations as therapeutic targets in PSP are currently lacking. In this study, we combine bulk tissue (n = 408) and single nucleus RNAseq (n = 34) data from PSP and control brains with transcriptome data from a mouse tauopathy and experimental validations in Drosophila tau models for systematic discovery of high-confidence expression changes in PSP with therapeutic potential. We discover, replicate, and annotate thousands of differentially expressed genes in PSP, many of which reside in glia-enriched co-expression modules and cells. We prioritize DDR2, STOM, and KANK2 as promising therapeutic targets in PSP with striking cross-species validations. We share our findings and data via our interactive application tool PSP RNAseq Atlas ( https://rtools.mayo.edu/PSP_RNAseq_Atlas/ ). Our findings reveal robust glial transcriptome changes in PSP, provide a cross-species systems biology approach, and a tool for therapeutic target discoveries in PSP with potential application in other neurodegenerative diseases.
Topics: Humans; Animals; Mice; Supranuclear Palsy, Progressive; tau Proteins; Systems Biology; Tauopathies; Neuroglia; Discoidin Domain Receptor 2
PubMed: 37919278
DOI: 10.1038/s41467-023-42626-3 -
Journal of Virology Jul 2023Powassan virus (POWV) is an emerging tick-borne flavivirus that causes neuroinvasive diseases, including encephalitis, meningitis, and paralysis. Similar to other...
Powassan virus (POWV) is an emerging tick-borne flavivirus that causes neuroinvasive diseases, including encephalitis, meningitis, and paralysis. Similar to other neuroinvasive flaviviruses, such as West Nile virus (WNV) and Japanese encephalitis virus (JEV), POWV disease presentation is heterogeneous, and the factors influencing disease outcome are not fully understood. We used Collaborative Cross (CC) mice to assess the impact of host genetic factors on POWV pathogenesis. We infected a panel of -null CC lines with POWV and observed a range of susceptibility, indicating that host factors other than the well-characterized flavivirus restriction factor modulate POWV pathogenesis in CC mice. Among the -null CC lines, we identified multiple highly susceptible lines (0% survival), including CC071 and CC015, and two resistant lines, CC045 and CC057 (>75% survival). The susceptibility phenotypes generally were concordant among neuroinvasive flaviviruses, although we did identify one line, CC006, that was specifically resistant to JEV, suggesting that both pan-flavivirus and virus-specific mechanisms contribute to susceptibility phenotypes in CC mice. We found that POWV replication was restricted in bone marrow-derived macrophages from CC045 and CC057 mice, suggesting that resistance could result from cell-intrinsic restriction of viral replication. Although serum viral loads at 2 days postinfection were equivalent between resistant and susceptible CC lines, clearance of POWV from the serum was significantly enhanced in CC045 mice. Furthermore, CC045 mice had significantly lower viral loads in the brain at 7 days postinfection than did CC071 mice, suggesting that reduced central nervous system (CNS) infection contributes to the resistant phenotype of CC045 mice. Neuroinvasive flaviviruses, such as WNV, JEV, and POWV, are transmitted to humans by mosquitoes or ticks and can cause neurologic diseases, such as encephalitis, meningitis, and paralysis, and they can result in death or long-term sequelae. Although potentially severe, neuroinvasive disease is a rare outcome of flavivirus infection. The factors that determine whether someone develops severe disease after a flavivirus infection are not fully understood, but host genetic differences in polymorphic antiviral response genes likely contribute to the outcome of infection. We evaluated a panel of genetically diverse mice and identified lines with distinct outcomes following infection with POWV. We found that resistance to POWV pathogenesis corresponded to reduced viral replication in macrophages, more rapid clearance of virus in peripheral tissues, and reduced viral infection in the brain. These susceptible and resistant mouse lines will provide a system for investigating the pathogenic mechanisms of POWV and identifying polymorphic host genes that contribute to resistance.
Topics: Humans; Mice; Animals; Flavivirus; Collaborative Cross Mice; Flavivirus Infections; West Nile virus; Encephalitis Viruses, Tick-Borne; Encephalitis Virus, Japanese; Encephalitis; Disease Susceptibility; Paralysis; 2',5'-Oligoadenylate Synthetase
PubMed: 37310228
DOI: 10.1128/jvi.00715-23 -
PloS One 2023This systematic review and meta-analysis aimed to assessment effects of electroacupuncture (EA) therapy on intractable facial paralysis. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
This systematic review and meta-analysis aimed to assessment effects of electroacupuncture (EA) therapy on intractable facial paralysis.
METHODS
The articles of EA treatment for intractable facial paralysis were retrieved from seven databases, the publication period was from its inception to November 30, 2022. Primary measure was the total effective rate, and other measures included the cure rate, Portmann scores, House-Brackmann scores, Sunnybrook scores and adverse events. The effect size of meta-analysis was expressed using relative risk (RR) or standardized mean difference (SMD) with 95% confidence interval (CI).
RESULTS
A total of 18 studies with 1,119 participants were included, all of them had various aspects of bias risk. Meta-analysis results revealed that EA ways improved total effective rate more effectively compared with non-EA counterparts (RR 1.23, 95% CI 1.17-1.31, I2 = 0%, 18 studies, 1119 participants), and improved cure rate more significantly than non-EA groups (RR 2.04, 95% CI 1.70-2.44, I2 = 0%, 18 studies, 1119 participants). None of studies reported adverse events.
CONCLUSION
EA therapy is more beneficial for patients with intractable facial paralysis than non-EA, but we lack sufficient evidence to evaluate its safety and follow-up effect. Therefore, more clinical trials with high quality methodologies are needed to further verify long-term effects of EA for IFP and improve the level of evidence.
TRIAL REGISTRATION
Registration number: CRD42021278541.
Topics: Humans; Electroacupuncture; Facial Paralysis
PubMed: 37440529
DOI: 10.1371/journal.pone.0288606 -
Journal of Neuroengineering and... Nov 2023Individuals with a locked-in state live with severe whole-body paralysis that limits their ability to communicate with family and loved ones. Recent advances in... (Review)
Review
Individuals with a locked-in state live with severe whole-body paralysis that limits their ability to communicate with family and loved ones. Recent advances in brain-computer interface (BCI) technology have presented a potential alternative for these people to communicate by detecting neural activity associated with attempted hand or speech movements and translating the decoded intended movements to a control signal for a computer. A technique that could potentially enrich the communication capacity of BCIs is functional electrical stimulation (FES) of paralyzed limbs and face to restore body and facial movements of paralyzed individuals, allowing to add body language and facial expression to communication BCI utterances. Here, we review the current state of the art of existing BCI and FES work in people with paralysis of body and face and propose that a combined BCI-FES approach, which has already proved successful in several applications in stroke and spinal cord injury, can provide a novel promising mode of communication for locked-in individuals.
Topics: Humans; Brain-Computer Interfaces; User-Computer Interface; Locked-In Syndrome; Paralysis; Electric Stimulation; Brain
PubMed: 37980536
DOI: 10.1186/s12984-023-01272-y -
Parkinsonism & Related Disorders Nov 2023Many studies of the Richardson's syndrome phenotype of progressive supranuclear palsy (PSP) have elucidated regions of progressive atrophy and neural correlates of...
INTRODUCTION
Many studies of the Richardson's syndrome phenotype of progressive supranuclear palsy (PSP) have elucidated regions of progressive atrophy and neural correlates of clinical severity. However, the neural correlates of survival and how these differ according to variant phenotypes are poorly understood. We set out to identify structural changes that predict severity and survival from scanning date to death.
METHODS
Structural magnetic resonance imaging data from 112 deceased people with clinically defined 'probable' or 'possible' PSP were analysed. Neuroanatomical regions of interest volumes, thickness and area were correlated with 'temporal stage', defined as the ratio of time from symptom onset to death, time from scan to death ('survival from scan'), and in a subset of patients, clinical severity, adjusting for age and total intracranial volume. Forty-nine participants had post mortem confirmation of the diagnosis.
RESULTS
Using T1-weighted magnetic resonance imaging, we confirmed the midbrain, and bilateral cortical structural correlates of contemporary disease severity. Atrophy of the striatum, cerebellum and frontotemporal cortex correlate with temporal stage and survival from scan, even after adjusting for severity. Subcortical structure-survival relationships were stronger in Richardson's syndrome than variant phenotypes.
CONCLUSIONS
Although the duration of PSP varies widely between people, an individual's progress from disease onset to death (their temporal stage) reflects atrophy in striatal, cerebellar and frontotemporal cortical regions. Our findings suggest magnetic resonance imaging may contribute to prognostication and stratification of patients with heterogenous clinical trajectories and clarify the processes that confer mortality risk in PSP.
Topics: Humans; Supranuclear Palsy, Progressive; Magnetic Resonance Imaging; Mesencephalon; Cerebellum; Atrophy
PubMed: 37804622
DOI: 10.1016/j.parkreldis.2023.105866 -
Human Brain Mapping Aug 2023There is a pressing need to understand the factors that predict prognosis in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), with high...
There is a pressing need to understand the factors that predict prognosis in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), with high heterogeneity over the poor average survival. We test the hypothesis that the magnitude and distribution of connectivity changes in PSP and CBS predict the rate of progression and survival time, using datasets from the Cambridge Centre for Parkinson-plus and the UK National PSP Research Network (PROSPECT-MR). Resting-state functional MRI images were available from 146 participants with PSP, 82 participants with CBS, and 90 healthy controls. Large-scale networks were identified through independent component analyses, with correlations taken between component time series. Independent component analysis was also used to select between-network connectivity components to compare with baseline clinical severity, longitudinal rate of change in severity, and survival. Transdiagnostic survival predictors were identified using partial least squares regression for Cox models, with connectivity compared to patients' demographics, structural imaging, and clinical scores using five-fold cross-validation. In PSP and CBS, between-network connectivity components were identified that differed from controls, were associated with disease severity, and were related to survival and rate of change in clinical severity. A transdiagnostic component predicted survival beyond demographic and motion metrics but with lower accuracy than an optimal model that included the clinical and structural imaging measures. Cortical atrophy enhanced the connectivity changes that were most predictive of survival. Between-network connectivity is associated with variability in prognosis in PSP and CBS but does not improve predictive accuracy beyond clinical and structural imaging metrics.
Topics: Humans; Supranuclear Palsy, Progressive; Corticobasal Degeneration; Prognosis; Neurodegenerative Diseases
PubMed: 37269181
DOI: 10.1002/hbm.26342 -
Journal of Neurology Feb 2024Progressive supranuclear palsy (PSP) is usually diagnosed in elderly. Currently, little is known about comorbidities and the co-medication in these patients.
BACKGROUND
Progressive supranuclear palsy (PSP) is usually diagnosed in elderly. Currently, little is known about comorbidities and the co-medication in these patients.
OBJECTIVES
To explore the pattern of comorbidities and co-medication in PSP patients according to the known different phenotypes and in comparison with patients without neurodegenerative disease.
METHODS
Cross-sectional data of PSP and patients without neurodegenerative diseases (non-ND) were collected from three German multicenter observational studies (DescribePSP, ProPSP and DANCER). The prevalence of comorbidities according to WHO ICD-10 classification and the prevalence of drugs administered according to WHO ATC system were analyzed. Potential drug-drug interactions were evaluated using AiDKlinik®.
RESULTS
In total, 335 PSP and 275 non-ND patients were included in this analysis. The prevalence of diseases of the circulatory and the nervous system was higher in PSP at first level of ICD-10. Dorsopathies, diabetes mellitus, other nutritional deficiencies and polyneuropathies were more frequent in PSP at second level of ICD-10. In particular, the summed prevalence of cardiovascular and cerebrovascular diseases was higher in PSP patients. More drugs were administered in the PSP group leading to a greater percentage of patients with polypharmacy. Accordingly, the prevalence of potential drug-drug interactions was higher in PSP patients, especially severe and moderate interactions.
CONCLUSIONS
PSP patients possess a characteristic profile of comorbidities, particularly diabetes and cardiovascular diseases. The eminent burden of comorbidities and resulting polypharmacy should be carefully considered when treating PSP patients.
Topics: Humans; Aged; Supranuclear Palsy, Progressive; Neurodegenerative Diseases; Cross-Sectional Studies; Comorbidity
PubMed: 37803149
DOI: 10.1007/s00415-023-12006-4