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Journal of Neurology, Neurosurgery, and... Jul 2021Cognitive impairment is a common, far-reaching but imperceptible manifestation in patients with amyotrophic lateral sclerosis (ALS). We aimed to identify the risk... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Cognitive impairment is a common, far-reaching but imperceptible manifestation in patients with amyotrophic lateral sclerosis (ALS). We aimed to identify the risk factors for cognitive impairment in ALS.
METHODS
We searched PubMed and EMBASE for cross-sectional, case-control and cohort studies that reported predictors of cognitive impairment in ALS. The obtained data were meta-analysed to generate overall ORs and 95% CIs.
RESULTS
Twenty-seven eligible articles reporting on 6799 individuals were included out of 20 501 records. Nine predictors were identified: (OR 3.62, 95% CI 1.76 to 7.45), dysarthria (OR 2.25, 95% CI 1.20 to 4.22), family history of ALS (OR 1.76, 95% CI 1.18 to 2.61), predominant upper motor neuron (PUMN) phenotype (OR 1.73, 95% CI 1.09 to 2.73) and bulbar onset (OR 1.54, 95% CI 1.28 to 1.87) increased risk factors for cognitive impairment in ALS. ALS Functional Rating Scale-Revised scores, sex, age or education level were not significantly associated with cognitive impairment in ALS. In addition, (OR=5.94) and bulbar onset (OR=2.08) were strong predictors of ALS-frontotemporal dementia. Female sex conferred more susceptibility to executive cognitive impairment than male sex (OR=1.82).
CONCLUSIONS
Patients with repeat expansion, dysarthria, family history of ALS, PUMN phenotype and bulbar onset had a high risk for cognitive impairment in ALS. These associations may contribute to understanding the heterogeneity of ALS.
PROSPERO REGISTRATION NUMBER
CRD42020201085.
Topics: Amyotrophic Lateral Sclerosis; Cognitive Dysfunction; Female; Humans; Male; Risk Factors; Sex Factors
PubMed: 33563800
DOI: 10.1136/jnnp-2020-325701 -
Chinese Medicine Jan 2021Hereditary ataxia (HA) represents a group of genetically heterogeneous neurodegenerative diseases caused by dysfunction of the cerebellum or disruption of the connection... (Review)
Review
BACKGROUND
Hereditary ataxia (HA) represents a group of genetically heterogeneous neurodegenerative diseases caused by dysfunction of the cerebellum or disruption of the connection between the cerebellum and other areas of the central nervous system. Phenotypic manifestation of HA includes unsteadiness of stance and gait, dysarthria, nystagmus, dysmetria and complaints of clumsiness. There are no specific treatments for HA. Management strategies provide supportive treatment to reduce symptoms.
OBJECTIVES
This systematic review aimed to identify, evaluate and summarise the published literature on the therapeutic roles of natural remedies in the treatment of HA to provide evidence for clinical practice.
METHODS
A systematic literature search was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Web of Science, PubMed and Science Direct Scopus were thoroughly searched for relevant published articles from June 2007 to July 2020.
RESULTS
Ten pre-clinical and two clinical studies were eligible for inclusion in this systematic review. We identified the therapeutic roles of medicinal plants Brassica napus, Gardenia jasminoides, Gastrodia elata, Ginkgo biloba, Glycyrrhiza inflata, Paeonia lactiflora, Pueraria lobata and Rehmannia glutinosa; herbal formulations Shaoyao Gancao Tang and Zhengan Xifeng Tang; and medicinal mushroom Hericium erinaceus in the treatment of HA. In this review, we evaluated the mode of actions contributing to their therapeutic effects, including activation of the ubiquitin-proteasome system, activation of antioxidant pathways, maintenance of intracellular calcium homeostasis and regulation of chaperones. We also briefly highlighted the integral cellular signalling pathways responsible for orchestrating the mode of actions.
CONCLUSION
We reviewed the therapeutic roles of natural remedies in improving or halting the progression of HA, which warrant further study for applications into clinical practice.
PubMed: 33509239
DOI: 10.1186/s13020-020-00414-x -
Journal of Neurology Jan 2022Deep brain stimulation (DBS) is used for treating dystonia, commonly targeting the subthalamic nucleus (STN). Optimal stimulation parameters are required to achieve... (Review)
Review
BACKGROUND AND OBJECTIVE
Deep brain stimulation (DBS) is used for treating dystonia, commonly targeting the subthalamic nucleus (STN). Optimal stimulation parameters are required to achieve satisfying results. However, recommended parameters for STN-DBS remain to be identified. In this review, we aimed to assess the optimal stimulation parameters by analyzing previously published STN-DBS data of patients with dystonia.
METHODS
We examined the STN-DBS stimulation parameters used in studies on dystonia selected on the PubMed/Medline database.
RESULTS
Of the 86 publications retrieved from the PubMed/Medline database, we included 24, which consisted of data from 94 patients and 160 electrodes. Overall, the following average stimulation parameters were observed: amplitude, 2.59 ± 0.67 V; pulse width, 83.87 ± 34.70 μs; frequency, 142.08 ± 37.81 Hz. The average improvement rate was 64.72 ± 24.74%. The improvement rate and stimulation parameters were linearly dependent. The average improvement rate increased by 3.58% at each 10-Hz increase in frequency. In focal and segmental dystonia, the improvement rate and stimulation parameters were linearly dependent. The improvement rate increased by 6.06% and decreased by 2.14% at each 10-Hz increase in frequency and pulse width, respectively. Seventeen publications (83 patients) mentioned stimulation-related adverse effects, including dyskinesia (17), depression (8), transient dysarthria (5), weight gain (4), transient dysphasia (3), transient paresthesia (2), and sustained hyperkinesia (2).
CONCLUSIONS
The optimal stimulation parameter for STN-DBS varies across patients. Our findings may be useful for DBS programming based on the specific dystonia subtypes, especially for patients with focal and segmental dystonia.
Topics: Deep Brain Stimulation; Dystonia; Dystonic Disorders; Humans; Subthalamic Nucleus; Weight Gain
PubMed: 33385242
DOI: 10.1007/s00415-020-10372-x -
Disability and Rehabilitation Jun 2022This review aimed to evaluate the evidence for group therapy in improving speech production in adults with acquired dysarthria. Secondary outcomes included communication...
PURPOSE
This review aimed to evaluate the evidence for group therapy in improving speech production in adults with acquired dysarthria. Secondary outcomes included communication effectiveness and/or wellbeing.
MATERIALS AND METHODS
A review protocol was prospectively published on PROSPERO. Fourteen electronic databases were searched to identify experimental studies investigating adults with acquired dysarthria participating in group intervention with outcomes related to communication and/or wellbeing. The quality of included studies was assessed using the Mixed Methods Appraisal Tool (MMAT) or the McMaster University's Critical Review Form, and the TIDieR template for intervention description and replication.
RESULTS
21 studies were identified involving 330 individuals with dysarthria, from mostly Parkinson's disease (PD) (97%; 321). Treatment approaches included singing therapy ( = 10), loudness therapy ( = 5) and multi-components therapy (including a combination of impairment and/or compensatory approaches) ( = 4). Studies varied in intensity and outcome measures used. Statistically significant improvements to speech production and/or wellbeing were reported following most approaches.
CONCLUSION
There is some preliminary moderate-quality evidence to suggest that group therapy may improve speech production and in some cases communication effectiveness or wellbeing in people with dysarthria following PD, with more consistent improvements being found for loudness approaches. Singing approaches were frequently studied in PD with some improvements to intelligibility evident. Further well-designed controlled studies including individuals with non-progressive aetiologies is warranted to establish the effectiveness of group treatment.IMPLICATIONS FOR REHABILITATIONGroup therapy may be an effective means of improving speech production and/or wellbeing in individuals with dysarthria following Parkinson's disease.Studies' employing loudness-based group therapy for PD demonstrated more consistent improvements to intensity measures.Some controlled studies utilising singing group therapy resulted in improved intelligibility in PD. CRD42015029374.
Topics: Adult; Dysarthria; Humans; Language Therapy; Parkinson Disease; Singing; Speech Therapy
PubMed: 33356634
DOI: 10.1080/09638288.2020.1859629 -
Journal of Alzheimer's Disease : JAD 2021Anti-IgLON5 disease is a rare neurodegenerative tauopathy that displays heterogeneity in clinical spectrum, disease course, cerebrospinal fluid (CSF) findings, and...
BACKGROUND
Anti-IgLON5 disease is a rare neurodegenerative tauopathy that displays heterogeneity in clinical spectrum, disease course, cerebrospinal fluid (CSF) findings, and variable response to immunotherapy. Sleep disorders, bulbar dysfunction, and gait abnormalities are common presenting symptoms, and conventional brain MRI scanning is often unrevealing.
OBJECTIVE
To provide a comprehensive overview of the literature and to assess the frequency of symptoms, MRI findings, and treatment response in patients with IgLON5 autoimmunity in the serum and CSF or restricted to serum.
METHODS
We examined a 65-year-old woman with bulbar-onset IgLON5 disease with serum-restricted antibodies, and we also performed a systematic review of all confirmed cases reported in the English literature.
RESULTS
We identified 93 patients, included our case. Clinical data were obtained in 58 subjects, in whom the most frequent symptoms were sleep-disordered breathing, dysphagia, parasomnias, dysarthria, limb or gait ataxia, stridor or vocal cord paresis, movement disorders, and postural instability. Distinct MRI alterations were identified in 12.5% of cases, as opposed to unspecific or unremarkable changes in the remaining patients. T2-hyperintense non-enhancing signal alterations involving the hypothalamus and the brainstem tegmentum were observed only in the present case. Inflammatory CSF was found in half of the cases and serum-restricted antibodies in 4 patients. Treatment with immunosuppressant or immunomodulatory drugs led to sustained clinical response in 19/52 patients.
CONCLUSION
Anti-IgLON5 autoimmunity should be considered in patients with sleep disorders, bulbar syndrome, autonomic involvement, and movement disorders, and high-field brain MRI can be of diagnostic help.
Topics: Aged; Autoimmune Diseases; Bulbar Palsy, Progressive; Cell Adhesion Molecules, Neuronal; Female; Humans; Hypothalamic Diseases; Neurodegenerative Diseases; Tauopathies
PubMed: 33337376
DOI: 10.3233/JAD-201105 -
Annals of Clinical and Translational... Jan 2021X-linked Charcot-Marie-Tooth type 1 (CMTX1) is an inherited peripheral neuropathy caused by mutations in the gap junction beta 1 (GJB1) gene, which encodes the...
OBJECTIVE
X-linked Charcot-Marie-Tooth type 1 (CMTX1) is an inherited peripheral neuropathy caused by mutations in the gap junction beta 1 (GJB1) gene, which encodes the connexin32 protein. A small number of patients with GJB1 mutations present with episodic neurological dysfunction and reversible white matter lesions, which has not been adequately reported. Here, we aim to enable clinicians to further understand this particular situation through systematically reviewing all published relevant cases.
METHODS
We conducted a comprehensive search of the PubMed electronic database for medical literature relevant to CMTX1 patients with episodic neurological dysfunction and then fully analyzed the general information, clinical manifestations, and characteristics of magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and nerve conduction study (NCS).
RESULTS
We identified 47 cases of CMTX1 associated with episodic central nervous system (CNS) dysfunction from 38 publications. CMTX1 patients experienced episodic CNS deficits at a young age, ranging from infancy to 26 years, and 45 (95.7%) of them were male. The CNS symptoms manifested as facial, lingual, or limb weakness in 44 (93.6%), dysarthria or dysphagia in 39 (83.0%), facial or limb numbness in 15 (31.9%), and ataxia in 10 (21.3%) patients. The duration of episodic symptoms ranged from 3 minutes to 6 months. Thirty (63.8%) CMTX1 cases have reported obvious predisposing factors, among which the most common factors were infection or fever (27.7%), travel to high altitude (12.8%), and intensive exercise (8.5%). As for brain MRI, most abnormal signals were found in bilateral deep white matter (88.9%) and corpus callosum (80.0%). In addition, most of the NCS results were abnormal, including prolonged latency, reduced amplitude, and slowed conduction velocity. The motor nerve conduction velocity (MNCV) of median nerve was the most detectable and valuable, ranging from 25 to 45 m/s.
INTERPRETATION
We have reported the most comprehensive summary of the demographic and clinical profile from 47 CMTX1 patients with episodic CNS deficits and provided new insight into the phenotype spectrum of CMTX1. We hope that our study can help clinicians make early diagnosis and implement the best prevention and treatment strategies for CMTX1 patients with episodic CNS deficits.
Topics: Central Nervous System Diseases; Charcot-Marie-Tooth Disease; Female; Humans; Male
PubMed: 33314704
DOI: 10.1002/acn3.51271 -
Clinical Rehabilitation May 2021To assess the effect of speech and language therapy (SLT) on Hypokinetic dysarthria (HD) in Parkinson's disease. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To assess the effect of speech and language therapy (SLT) on Hypokinetic dysarthria (HD) in Parkinson's disease.
DESIGN
Systematic review and meta-analysis of randomized controlled trials.
METHODS
We performed a literature search of randomized controlled trials using PubMed, Web of Science, Science Direct and Cochrane database (last search October 2020). Quality assessment and risk of bias were assessed using the Downs and Black scale and the Cochrane tool. The data were pooled and a meta-analysis was completed for sound pressure levels, perceptual intelligibility and inflection of voice fundamental frequency.
RESULTS
We selected 15 high to moderate quality studies, which included 619 patients with Parkinson's disease. After pooling the data, 7 studies, which compared different speech language therapies to no treatment, control groups and 3 of their variables, (sound pressure level, semitone standard deviation and perceptual intelligibility) were included in the analysis.Results showed significant differences in favor of SLT for sound pressure level sustained phonation tasks (standard mean difference = 1.79; 95% confidence interval = 0.86, 2.72; ⩽ 0.0001). Significant results were also observed for sound pressure level and semitone standard deviation in reading tasks (standard mean difference = 1.32; 95% confidence interval = 1.03, 1.61; ⩽ 0.0001). Additionally, sound pressure levels in monologue tasks showed similar results when SLT was compared to other treatments (standard mean difference = 0.87; 95% confidence interval = 0.46, 1.28; ⩽ 0.0001).
CONCLUSION
This meta-analysis suggests a beneficial effect of SLT for reducing Hypokinetic Dysarthria in Parkinson's disease, improving perceptual intelligibility, sound pressure level and semitone standard deviation.
Topics: Dysarthria; Humans; Language Therapy; Parkinson Disease; Speech Therapy
PubMed: 33233932
DOI: 10.1177/0269215520976267 -
Journal of Infection and Chemotherapy :... Feb 2021Vagococcal infections are uncommon in humans; there are limited studies on the clinical manifestations, the optimal methods for identifications, and antimicrobial...
BACKGROUND
Vagococcal infections are uncommon in humans; there are limited studies on the clinical manifestations, the optimal methods for identifications, and antimicrobial susceptibility testing for vagococcal infections. Here, we have reported a case of Vagococcus fluvialis-induced bacteremia and decubitus ulcer and have systematically reviewed other reported Vagococcus infections.
CASE PRESENTATION
A 74-year-old man presented to our emergency department with muscle weakness on his left extremities, dysarthria, and altered mental status along with fever for the past 4 days. Physical examination revealed a decubitus ulcer with foul smelling and yellowish exudative pus on his left chest wall and abdomen, forearm, thigh, and lower leg. He was empirically treated with 2.25 mg of piperacillin/tazobactam every 8 hours and 0.5 g of vancomycin every 24 hours intravenously (IV) for his decubitus ulcer. Vagococcus fluvialis was detected in both aerobic and anaerobic blood cultures (upon admission) using the VITEC 2 GP ID card (bioMérieux) and matrix-assisted laser desorption/ionization-time-of-flight mass spectrometry (MALDI-TOF MS). We continued the mentioned IV antimicrobial therapies for 4 weeks following which the patient was transferred to a long-term care facility for further rehabilitation.
CONCLUSIONS
To our best knowledge, this is the first literature review of Vagococcus infections in humans. Since it is challenging to distinguish Vagococcus from Enterococcus by a conventional method due to the similarity of its biochemical properties to those of Enterococcus, based on our literature review, 16S rRNA sequencing or analysis of bacterial protein profile using MALDI-TOF MS may be useful for the precise identification.
Topics: Aged; Bacteremia; Enterococcaceae; Humans; Male; Pressure Ulcer; RNA, Ribosomal, 16S; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
PubMed: 33036895
DOI: 10.1016/j.jiac.2020.09.019 -
Evidence-based Complementary and... 2020This study aimed to evaluate the effect of acupuncture intervention and manipulation types on poststroke dysarthria. Electronic database, including PubMed, CENTRAL,... (Review)
Review
This study aimed to evaluate the effect of acupuncture intervention and manipulation types on poststroke dysarthria. Electronic database, including PubMed, CENTRAL, Scopus, RISS, and CNKI, were searched for randomized controlled trials (RCT), treating dysarthria using acupuncture, speech-language therapy (SLT), and general management (GM), published before April 2019. The number, distribution, intensity, depth, and repetition of acupuncture and bleeding therapy on the sublingual veins were considered as manipulation types. Risk of bias of the included trials was evaluated, and their efficacy was assessed using risk ratio (RR) and the standard mean differences in the Frenchay Dysarthria Assessment and Speech Function Grading, with 95% confidence intervals (CIs).Fifteen RCT trials involving 1453 patients were isolated. Electroacupuncture plus SLT and manual acupuncture plus SLT were more effective than SLT only, respectively (RR = 1.520, 95% CI [1.183-1.952], RR = 1.380, 95% CI [1.281-1.488]). The clinical efficacy of acupuncture plus GM was higher than that of GM alone (RR = 1.165, 95% CI [1.050-1.293]). Meta-ANOVA showed that none of the manipulation types increased the clinical efficacy of acupuncture on dysarthria. The methodological quality was low. In conclusion, our study suggests that the effect of acupuncture on poststroke dysarthria may be maximized when manual acupuncture or electroacupuncture is combined with SLT, irrespective of manipulation types.
PubMed: 33005199
DOI: 10.1155/2020/4981945 -
PM & R : the Journal of Injury,... Mar 2021To define methods to measure dysarthria due to stroke and guide physicians in delineating a diagnostic protocol using the best current strategies.
OBJECTIVE
To define methods to measure dysarthria due to stroke and guide physicians in delineating a diagnostic protocol using the best current strategies.
DESIGN
Systematic review.
LITERATURE SURVEY
A search was conducted on PubMed, EMBASE, the Cochrane Library, and Web of Science to identify measurement methods for dysarthria severity in adults after stroke.
METHODS
Two reviewers independently reviewed articles and came to a consensus about which ones to include. The authors excluded all duplicates, articles involving individuals with aphasia or other speech problems other than dysarthria, and articles unrelated to stroke. Articles were included if diagnostic measures were used to examine the effectiveness of speech rehabilitation in stroke patients.
SYNTHESIS
The search identified 1154 articles with the keywords "stroke" OR "ictus" OR "cerebral vascular accident" AND "dysarthria" OR "Speech and Language Disorders" AND "diagnosis" OR "assessment." The reviewers analyzed 86 full texts. There were 37 publications that met the criteria and were included in the systematic review. These articles were used to describe the main methods used for measuring the severity of stroke-related dysarthria before and after speech rehabilitation.
CONCLUSION
Despite the range of diagnostic tools available, robust trials are lacking, and the diagnostic approaches are always different. More research is needed to find the best diagnostic methodologies and delineate a definitive diagnostic protocol.
Topics: Adult; Aphasia; Dysarthria; Humans; Speech Disorders; Speech Therapy; Stroke
PubMed: 32818305
DOI: 10.1002/pmrj.12469