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Movement Disorders Clinical Practice Dec 2023Individuals with multiple system atrophy (MSA) often complain about pain, nonetheless this remains a poorly investigated non-motor feature of MSA. (Review)
Review
BACKGROUND
Individuals with multiple system atrophy (MSA) often complain about pain, nonetheless this remains a poorly investigated non-motor feature of MSA.
OBJECTIVES
Here, we aimed at assessing the prevalence, characteristics, and risk factors for pain in individuals with MSA.
METHODS
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes (PRISMA) guidelines, we systematically screened the PubMED, Cochrane, and Web of Science databases for papers published in English until September 30, 2022, combining the following keywords: "pain," "multiple system atrophy," "MSA," "olivopontocerebellar atrophy," "OPCA," "striatonigral degeneration," "SND," "Shy Drager," and "atypical parkinsonism."
RESULTS
The search identified 700 records. Sixteen studies provided information on pain prevalence in cohorts of MSA individuals and were included in a qualitative assessment based on the Quality Assessment of Diagnostic Accuracy Studies (QUADAS) tool. Thirteen studies (11 cross-sectional, two longitudinal) scored ≥14 points on QUADAS assessment and were included in a quantitative analysis, pooling data from 1236 MSA individuals. The resulting pooled prevalence of pain in MSA was 67% (95% confidence intervals [CI] = 57%-75%), and significantly higher in individuals with MSA of parkinsonian rather than cerebellar type (76% [95% CI = 63%-87%] vs. 45% [95% CI = 33%-57%], = 0.001). Pain assessment tools and collected information were highly heterogeneous across studies. Two studies reported pain treatment strategies and found that only every second person with MSA complaining about pain had received targeted treatment.
CONCLUSIONS
We found that pain is a frequent, but still under-recognized and undertreated feature of MSA. Further research is needed to improve pain detection and treatment in MSA.
PubMed: 38094640
DOI: 10.1002/mdc3.13897 -
Movement Disorders : Official Journal... Jun 2009The aim of this study was to determine the effectiveness and safety of available treatment alternatives for degenerative ataxias (DA). We systematically reviewed studies... (Review)
Review
The aim of this study was to determine the effectiveness and safety of available treatment alternatives for degenerative ataxias (DA). We systematically reviewed studies that assess pharmacological, rehabilitative, or psychological treatments in patients with DA. Studies were included if they fulfilled prespecified criteria. All included clinical trials were scored for methodological quality. Main outcome measures were clinical status of neurological disorder, adverse events, and patient-based factors. Twenty-five studies were included. Most studies were of small sample sizes, wide age variations, and low scientific validity. Only one study gave information on physical rehabilitation and none on psychological therapy. The remaining 24 studies reported on the effects of different pharmacological treatments. Outcomes such as functional capacity and psychological functioning of patients were evaluated by few studies. Some evidence supports that 5-hydroxytryptophan is more effective than placebo improving neurological symptoms in patients with Friedreich ataxia (FA), olivopontocerebellar atrophy, or cerebellar atrophy. Idebenone is more effective than placebo for halting and reversing the hypertrophic cardiomyopathy associated with FA, but it seems unable to improve neurological semiology. Limited evidence for other therapies was found. No relevant side effects for drugs that shown some degree of effectiveness were reported. Availability of quality studies to evaluate the safety and efficacy of treatments for most DA is scarce. No valid information on the actual value of physical rehabilitation and psychological support as treatments for DA is available. Further investigations with improved trial designs are necessary.
Topics: Ataxia; Databases, Bibliographic; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Outcome Assessment, Health Care; Physical Therapy Modalities; Spinocerebellar Degenerations
PubMed: 19412936
DOI: 10.1002/mds.22564 -
Gait & Posture Mar 2019Analysis of sensorimotor synergies has been greatly advanced by the Uncontrolled Manifold (UCM) approach. The UCM method is based on partitioning inter-trial variance...
BACKGROUND
Analysis of sensorimotor synergies has been greatly advanced by the Uncontrolled Manifold (UCM) approach. The UCM method is based on partitioning inter-trial variance displayed by elemental variables into 'good' (V) and 'bad' (V) variability that, respectively, indicate maintenance or loss of task stability. In clinical populations, these indices can be used to investigate the strength, flexibility, stereotypy and agility of synergistic control.
RESEARCH QUESTION
How are synergies affected by neurological impairment in adults? Specifically, this study aimed to determine i) the impact of pathology on V, V, and their ratio (synergy index); ii) the relationship between synergy indices and functional performance; iii) changes in anticipatory synergy adjustments (ASAs); and iv) the effects of interventions on synergies.
METHODS
Systematic review of UCM studies on adults with neurological impairment.
RESULTS
Most of the 17 studies had moderate to high quality scores in the adapted Critical Review Form and the UCM reporting quality checklist developed for this review. i) Most of the studies found reduced synergy indices for patients with Parkinson's disease (PD), olivo-ponto-cerebellar atrophy, multiple sclerosis and spinocerebellar degeneration, with variable levels of change in V and V. Reduction in synergy indices was not as consistent for stroke, in three out of six studies it was unchanged. ii) Five of seven studies found no significant correlations between scores on motor function scales and UCM indices. iii) Seven studies consistently reported ASAs that are smaller in magnitude, delayed, or both, for patients compared to healthy controls. iv) Two studies reported increased synergy indices, either via increase in V or decrease in V, after dopaminergic drugs for patients with PD. There were similar synergy indices but improved ASAs after deep brain stimulation for patients with PD.
SIGNIFICANCE
UCM can provide reliable and sensitive indicators of altered synergistic control in adults with neurological impairment.
Topics: Deep Brain Stimulation; Dopamine Agents; Humans; Parkinson Disease; Psychomotor Performance
PubMed: 30677709
DOI: 10.1016/j.gaitpost.2019.01.003 -
Neurology Feb 1997A systematic review of the neurologic literature identified 433 cases of pathologically proven multiple system atrophy over a 100-year period. Earlier case reports... (Meta-Analysis)
Meta-Analysis
A systematic review of the neurologic literature identified 433 cases of pathologically proven multiple system atrophy over a 100-year period. Earlier case reports included patients younger in age with more frequent cerebellar involvement. Mean age of onset was 54.2 years (range 31 to 78) and survival was 6.2 years (range 0.5 to 24). Survival analysis showed a secular trend from a median duration of 4.9 years for publications between 1887 and 1970 to 6.8 years between 1991 and 1994. Older age of onset was associated with shorter survival; the hazard ratio for patients with onset after 60 years was 1.8 (95% CI 1.4 to 2.3) compared with patients between 31 and 49 years. Cerebellar features were associated with marginally increased survival (6.1 years versus 5.4 years; p = 0.04). There were no difference in survival according to gender, parkinsonian, or pyramidal features or whether the patient was classified as striatonigral degeneration or olivopontocerebellar atrophy type. These results demonstrate the poor prognosis for patients with multiple system atrophy but may be biased toward the worst cases. Future research needs to recruit more representative samples.
Topics: Adult; Aged; Atrophy; Autonomic Nervous System Diseases; Brain Diseases; Cerebellar Diseases; Corpus Striatum; Female; Humans; Male; Middle Aged; Olivopontocerebellar Atrophies; Parkinson Disease
PubMed: 9040727
DOI: 10.1212/wnl.48.2.384