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Multiple system atrophy(MSA)is a serious and rapidly progressive neurodegenerative disease.The currently used diagnostic criteria are the second edition of MSA diagnostic criteria published in 2008.Although widely recognized in clinical research, they lack sensitivity for early diagnosis of the disease.Reliable early diagnosis of MSA is particularly important for patient care and counseling, recruitment for clinical trials of disease-modifying therapies, and development and validation of diagnostic tools.To overcome the shortcomings of the second edition of the diagnostic criteria, the International Parkinson and Movement Disorder Society developed a new version of diagnostic criteria for MSA, which was published in April 2022.This paper interprets the new MSA diagnostic criteria and examines the scientific research areas that need to be further explored in future research, which will be important in guiding the management of elderly patients and related research.
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Objective:To examine the clinical subtypes of patients with multisystem atrophy(MSA)that may indicate the prognosis of patients.Additionally, we aim to compare the ability to perform daily activities among patients of each subtype using cluster analysis.Methods:The retrospective analysis included demographic data, clinical symptoms and signs, scale scores, and ancillary examinations of 94 patients diagnosed with multisystem atrophy at Xuanwu Hospital of Capital Medical University.The study aimed to analyze the clinical characteristics of each subtype obtained through clustering.Additionally, a comparison was made between patients with traditional motor subtypes and those with new subtypes in terms of activities of daily living.The study consisted of 94 MSA patients, with an average age of 61 years and a female representation of 51.1%.Using the data collected on the continuum, a full linkage hierarchical cluster analysis was performed to classify MSA patients into four clinical subtypes: gait disorder(17 cases, 18.1%), malignant tonic hyperkinetic with premature haircut(25 cases, 26.6%), intermediate(43 cases, 45.7%), and autonomic benign type(9 cases, 9.6%).Each subtype exhibited various clinical motor and non-motor symptoms, including UPDRS-Ⅲ( χ2=27.90, P<0.001), gait disturbance( χ2=33.23, P<0.001), MoCA( χ2=10.98, P=0.012), HAMA( χ2=12.14, P=0.007), HAMD( χ2=13.62, P=0.003), smell score( χ2=10.16, P=0.017), postural hypotension( χ2=14.59, P=0.028), and a statistically significant difference in the ability to perform daily living score( χ2=25.35, P<0.001).No statistically significant differences in non-motor symptoms and activities of daily living abilities were observed between the cerebellar and Parkinsonian types of traditional motor typing( P>0.05). Conclusions:The hierarchical clustering analysis conducted in this study reveals that the clinical phenotype of MSA provides a more accurate reflection of patients' clinical characteristics and their impact on quality of life compared to the traditional motor phenotype.Additionally, it may help predict variations in the underlying pathological impairment and the rate of disease progression.These findings offer a foundation for precise diagnostic interventions in patients with MSA.
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Abstract After more than 200 years since its initial description, the clinical diagnosis of Parkinson's disease (PD) remains an often-challenging endeavor, with broad implications that are fundamental for clinical management. Despite major developments in understanding it's pathogenesis, pathological landmarks, non-motor features and potential paraclinical clues, the most accepted diagnostic criteria remain solidly based on a combination of clinical signs. Here, we review this process, discussing its history, clinical criteria, differential diagnoses, ancillary diagnostic testing, and the role of non-motor and pre-motor signs and symptoms.
Resumo Passados mais de 200 anos desde a sua descrição inicial, o diagnóstico clínico da doença de Parkinson (DP) continua a ser um processo muitas vezes desafiante, com amplas implicações que são fundamentais para o manejo clínico. Apesar dos grandes desenvolvimentos na compreensão da sua patogénese, marcadores patológicos, características não motoras e potenciais pistas paraclínicas, os critérios diagnósticos mais aceitos permanecem solidamente baseados numa combinação de sinais clínicos motores. Aqui, revisamos esse processo, discutindo sua história, critérios clínicos, diagnósticos diferenciais, testes diagnósticos complementares e o papel dos sinais e sintomas não motores e pré-motores.
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Objective:To explore the role of α-synuclein (α-Syn) in pathogenesis of Parkinson's disease (PD) and multiple system atrophy (MSA) by observing the ability of α-Syn aggregates incubated with PD and MSA patients' plasma to destroy cell membrane.Methods:Peripheral blood samples were collected from 5 PD patients and 5 MSA patients diagnosed in Department of Neurology, Affiliated Hospital of Guilin Medical University from January 2018 to January 2022, as well as 5 physical examination healthy control subjects (HCs) during the same period. The α-Syn was dissolved in 0.01 mol/L PBS and then incubated with PBS, and plasma from HCs, PD patients and MSA patients at 37 ℃ for 4 d, respectively (named as PBS group, HC group, PD group and MSA group). Small unilamellar vesicles (SUVs) containing calcein were prepared with acidic phospholipid, 1-palmityl-2-oleoyl-Sn-glycerol-3-phospho-L-serine (POPS), by membrane dispersion-ultrasonic method; the particle size and morphology of SUVs were analyzed by Malvern Zetasizer Nano ZS and transmission electron microscope. SUVs and human neuroblastoma cells (SH-SY5Y) were treated with different concentrations of α-Syn aggregates (0.5, 1.0, 2.0, 4.0, 8.0 and 16.0 μmol/L). The abilities of α-Syn aggregates formed under different conditions to destroy liposomes and cell membrane were evaluated by measuring the calcein relative release and intracellular calcein fluorescent value after dialysis.Results:(1) Destructive effect of α-Syn aggregates on SUVs: calcein induced by α-Syn aggregates in each group increasingly released with the increase of protein concentration; at protein concentration of 8 μmol/L, the calcein released from SUVs in PD and MSA groups was significantly higher than that in PBS and HC groups, and the calcein released from SUVs in MSA group was further significantly higher than that in the PD group ( P<0.05). (2) Destructive effect of α-Syn aggregates on cell membrane in SH-SY5Y: the calcein fluorescent value in each group decreased with the increase of protein concentration; at protein concentration of 8 μmol/L, the intracellular calcein fluorescent value in PD and MSA groups were significantly decreased compared with that in PBS and HC groups ( P<0.05); the intracellular calcein fluorescent value in MSA group was further significantly decreased compared with that in PD group ( P<0.05). (3) Effect of α-Syn aggregates on SH-SY5Y cell survival: at protein concentration of 8 μmol/L, the viability of SH-SY5Y cells in each group decreased obviously; PD and MSA groups had significantly decreased cell viability compared with PBS and HC groups ( P<0.05); and the viability in MSA group was further statistically decreased compared with that in PD group ( P<0.05). Conclusion:The ability of α-Syn aggregates incubated with PD and MSA patients' plasma to destroy cell membrane is greater than that with HCs' plasma, especially those with MSA patients' plasma.
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Objective To investigate the diagnostic value of cervical vagus nerve cross-sectional area(CAS)for Parkinson's disease(PD).Methods Thirty patients with PD admitted to the People's Hospital of Zhengzhou University from October 2019 to October 2022 were selected as PD group,25 patients with multiple system atrophy(MSA)admitted to the People's Hospital of Zhengzhou University during the same period were selected as the MSA group,and 30 healthy individuals who underwent physical examination in the People's Hospital of Zhengzhou University during the same period were selected as healthy control group.Cervical vagus CAS of subjects in the three groups were measured by high-resolution ultrasound,and the difference of CAS of cervical vagus nerve was compared among the three groups.The degree of impairment of autonomic nervous function of subjects in the three groups was evaluated by PD autonomic symptom scale(SCOPA-AUT).The diagnostic value of cervical vagus nerve CAS for PD was analyzed by receiver operating characteristic(ROC)curve.Results The CAS of the right cervical vagus nerve of subjects was significantly larger than that of the left in the healthy control group and PD group(P<0.05);there was no significant difference in CAS of bilateral cervical vagus nerve of subjects in the MSA group(P>0.05).The CAS and average CAS of bilateral cervical vagus nerve of subjects in the PD group and MSA group were significantly lower than those in the healthy control group(P<0.01).The CAS of the right vagus nerve of subjects in the MSA group was significantly lower than that in the PD group(P<0.05);there was no significant difference in CAS and the average CAS of the left vagus nerve between the MSA group and the PD group(P>0.05).The total score of SCOPA-AUT and gastrointestinal(GI),cardiovascular(CV),urinary(UR)and sexual(SX)scores of subjects in the PD group and MSA group were significantly higher than those in the healthy control group(P<0.01).The total score of SCOPA-AUT and UR,SX scores of subjects in the MSA group were significantly higher than those in the PD group(P<0.05).There was no significant difference in temperature(TH)and pupil(PU)of subjects among the three groups(P>0.05).Pearson correlation analysis showed that the CAS of cervical vagus nerve of PD patients was not correlated with the total score of SCOPA-AUT and the UR,TH,PU,SX scores(r=-0.143,0.281,0.297,0.265,0.312;P>0.05).The CAS of cervical vagus nerve of PD patients was negatively correlated with GI and CV scores(r=-0.683,-0.373;P<0.05).ROC curve analysis showed that the area under the curve of cervical vagus nerve for diagnosing PD was 0.870(95%confidence interval:0.773-0.966,P<0.05);the critical value was 3.064 mm2,the sensitivity was 96%,and the specificity was 67%.The area under the curve of CAS of cervical vagus nerve in differential diagnosis of PD,MSA was 0.680(95%confidence interval:0.537-0.823,P<0.05).The sensitivity and specificity for the diagnosis of MSA were 68%and 70%when the CAS of the cervical vagus nerve<2.709 mm2.Conclusion The CAS of cervical vagal nerve has high clinical diagnostic value for PD,and it provides a new way to improve the diagnosis rate of PD.
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Objective:To investigate the role of plasma neurofilament light chain (NfL) in diagnosing and differentiating Parkinson's disease (PD) and multiple system atrophy-Parkinsonian subtype (MSA-P).Methods:Forty PD patients and 23 MSA-P patients admitted to Department of Neurology, Henan Provincial People's Hospital from June 2019 to December 2021 were recruited; 27 healthy subjects accepted physical examination during the same period were selected as controls. Ultrasensitive Simoa technology was used to measure the plasma NfL. Differences in clinical data and plasma NfL were compared among all subjects. Correlations of plasma NfL with clinical characteristics, such as disease course, Hoehn-Year (H-Y) staging, Unified Parkinson's Disease Rating Scale (UPDRS), Montreal Cognitive Assessment (MoCA), Scale for Outcomes in Parkinson's Disease for Autonomic Symptoms (SCOPA-AUT) and levodopa equivalent daily dosage (LEDD), were analyzed with Pearson correlations. Receiver operating characteristic (ROC) curve was used to analyze the value of plasma NfL in diagnosing and differentiating PD and MSA-P.Results:Compared with MSA-P group, PD group had significantly longer disease course and statistically lower scores of UPDRS-II and SCOPA-AUT ( P<0.05). The plasma NfL in MSA-P group, PD group and healthy control group was decreased successively ([37.69±10.47] pg/mL, [17.85±4.23] pg/mL, [12.86±3.14] pg/mL, respectively), with statistical differences ( P<0.05). In MSA-P patients, Pearson correlations showed positive correlation between plasma NfL and age ( r=0.442, P=0.035); and Partial correlations showed positive correlations between plasma NfL and scores of UPDRS-I and UPDRS-III ( P<0.05), and plasma NfL showed no significant correlation with H-Y staging, UPDRS-III, MoCA, LEDD or SCOPA-AUT scores ( P>0.05). In PD patients, Pearson correlations showed that plasma NfL was positively correlated with age ( r=0.342, P=0.031); partial correlations showed that plasma NfL was positively correlated with H-Y staging and UPDRS-III, and negatively correlated with MoCA scores ( P<0.05); plasma NfL showed no significant correlation with disease course, scores of UPDRS-I and UPDRS-II, LEDD, and SCOPA-AUT scores ( P>0.05). ROC curve showed that the area under the curve (AUC) of plasma NfL in diagnosing PD was 0.814 (95% CI: 0.712-0.920, P<0.001); AUC of plasma NfL in differentiating and diagnosing PD and MSA-P was 0.980 (95% CI: 0.954-1.000, P<0.001); AUC of plasma NfL in diagnosing MSA-P was 0.998 (95% CI: 0.993-1.000, P<0.001). Conclusions:Plasma NfL is correlated with severity of motor symptoms in MSA-P patients; plasma NfL is correlated with cognitive function and disease course in PD patients. Besides, plasma NfL has high sensitivity and specificity in differentiating PD and MSA-P, therefore, plasma NfL could serve as a biomarker to diagnosis and differentiate PD.
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Multiple system atrophy(MSA)is an adult-onset and progressive neurodegenerative disease,is clinically classified into two subtypes:MSA with predominant parkinsonism(MSA-P)and MSA with predominant cerebellar ataxia(MSA-C),characterized by dysautonomia and dyskinesia.The etiology of MSA is still unknown,multiple pathogenic factors are involved in the pathogenesis of this disease,therefore,in addition to limited symptomatic treatment,there is still a lack of disease modifying therapy to prevent the progress of the disease.Currently,animal and clinical studies targeting α-synuclein,neuroinflammation and neurotrophic supporting are being explored,so this article reviewed the latest progresses to improve the clinical understanding of treatment of MSA.
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@#Objective To investigate the risk factors for fatigue impairment in patients with multiple system atrophy (MSA). Methods A total of 101 patients with MSA were enrolled,and according to the score of Fatigue Severity Scale (FSS),they were divided into non-fatigue group (<4 points) with 41 patients and fatigue group (≥4 points) with 60 patients. A binary logistic regression analysis was used to screen for the risk factors for fatigue in patients with MSA. Results There were significant differences in sex,course of disease,clinical classification,urinary retention,UMSARS-I,UMSARS-II,UMSARS-I+II,UMSARS-IV,bradykinesia,myotonia,ataxia,abnormal gait and posture,and ESS score between the patients with different between fatigue levels (P<0.05). The binary logistic regression analysis showed that UMSARS-I and ESS scores were independent risk factors for fatigue in MSA patients (P<0.05). Conclusion Fatigue impairment in patients with MSA is caused by multiple factors,among which UMSARS-I and ESS scores are independent risk factors for fatigue in MSA.
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Multiple system atrophy (MSA) is a rapidly developing and serious Degenerative disease of the nervous system. It is characterized by different combinations of prominent autonomic dysfunction, Parkinson′s syndrome and cerebellar Ataxia in clinical practice, and its core symptom is extensive and serious autonomic dysfunction in the early stage of the disease. Non motor symptoms of MSA involve nervous system, cardiovascular system, gastrointestinal system, Genitourinary system and many other fields. Early clinical heterogeneity is large. This article describes the non motor symptoms of MSA, including prodromal symptoms, to help clinicians identify MSA earlier.
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Objective:To explore the impact of different segmentation methods on differential diagnostic efficiency of 18F-FDG PET/MR radiomics to distinguish Parkinson′s disease (PD) from multiple system atrophy (MSA). Methods:From December 2017 to June 2019, 90 patients (60 with PD and 30 with MSA; 37 males, 53 females; age (55.8±9.5) years) who underwent 18F-FDG PET/MR in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology were retrospectively collected. Patients were randomized to training set and validation set in a ratio of 7∶3. The bilateral putamina and caudate nuclei, as the ROIs, were segmented by automatic segmentation of brain regions based on anatomical automatic labeling (AAL) template and manual segmentation using ITK-SNAP software. A total of 1 172 radiomics features were extracted from T 1 weighted imaging (WI) and 18F-FDG PET images. The minimal redundancy maximal relevance (mRMR) and least absolute shrinkage and selection operator (LASSO) algorithm were used for features selection and radiomics signatures (Radscore) construction, with 10-fold cross-validation for preventing overfitting. The diagnostic performance of the models was assessed by ROC curve analysis, and the differences between models were calculated by Delong test. Results:There were 63 cases in training set (42 PD, 21 MSA) and 27 cases in validation set (18 PD, 9 MSA). The Radscore values were significantly different between the PD group and the MSA group in all training set and validation set of radiomics models ( 18F-FDG_Radscore and T 1WI_Radscore) based on automatic or manual segmentation methods ( z values: from -5.15 to -2.83, all P<0.05). ROC curve analysis showed that AUCs of 18F-FDG_Radscore and T 1WI_Radscore based on automatic segmentation in training and validation sets were 0.848, 0.840 and 0.892, 0.877, while AUCs were 0.900, 0.883 and 0.895, 0.870 based on manual segmentation. There were no significant differences in training and validation sets between Radiomics models based on different segmentation methods ( z values: 0.04-0.77, all P>0.05). Conclusions:The 18F-FDG PET/MR radiomics models based on different segmentation methods achieve promising diagnostic efficacy for distinguishing PD from MSA. The radiomics analysis based on automatic segmentation shows greater potential and practical value in the differential diagnosis of PD and MSA in view of the advantages including time-saving, labor-saving, and high repeatability.
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Objective:To study the features as well as the diagnosis and differential diagnosis values by conventional MRI morphometrics in different clinical subtypes of progressive supranuclear palsy(PSP).Methods:Forty five patients with PSP were included, comprising three PSP subtypes: 15 cases of Richardson's syndrome(PSP-RS), 15 cases of Parkinson's syndrome(PSP-P)and 15 cases of progressive frozen gait(PSP-PFG). In addition, three control groups were established: 15 cases of multiple system atrophy-Parkinson's syndrome(MSA-P), 30 cases of primary Parkinson's disease(PD)and 40 healthy controls(HC). Midbrain area-to-Pons area ratio(M/P), Magnetic Resonance Parkinsonism Index(MRPI, MRPI2.0), width ratio of middle cerebellar peduncle to superior cerebellar peduncle(MCP/SCP), Midbrain-to-Pons ratio(MTPR), Angle of cerebral peduncle(A cp), third ventricle width/frontal horns width ratio(V 3rd/FH), and Humming bird sign rating scale(HBS-RS)scores were calculated.Diagnostic sensitivity and specificity were performed by ROC curve to assess the accuracy of these imaging indicators in the diagnosis and differential diagnosis of PSP and its subtypes. Results:The MRPI, MRPI2.0, MCP/SCP and HBS-RS scores were significantly higher in PSP group than in other control groups( H=69.351, 66.776, 33.926 and 84.694, all P<0.05), while M/P and MTPR were significantly lower in PSP group than in other control groups(H=60.101 and 77.276, all P<0.05). PSP group also had higher V 3rd/FH compared with PD or HC group( F=17.168, P<0.05), but not with MSA-P group( Z=-1.602, P>0.05). The above differences also existed between each PSP subgroup and control groups.Among PSP subgroups, PSP-PFG subgroup had a larger A cp than did PSP-RS( Z=-2.510, P<0.05), and had higher HBS-RS score than did PSP-P group( Z=-2.380, P<0.05). No significant differences in other MRI morphometric indexes were identified among PSP subtypes.The M/P, MRPI, MTPR, MRPI2.0, HBS-RS score showed good accuracy in diagnosing PSP and its each subgroup, with HBS-RS score being the most accurate indicator, when the cutoff value was 2, the AUC values were all higher than 0.99, and the sensitivity and specificity were all above 90%.PSP and its subtypes were best distinguished from MSA-P by MRPI, when the cutoff value was 9.94, the AUC values were all higher than 0.90, with the sensitivity of 100% and specificity of 86.67%.PSP and its subtypes were best distinguished from PD by MTPR, AUC values were all above 0.95, with slightly different cutoff values.Almost all the morphological measurement parameters failed to show significant sensitivity and specificity in discriminating subtypes of PSP.The sensitivity and specificity of almost all MRI morphometry indicators in differentiating different subtypes of PSP are not high. Conclusions:MRI morphometrics have a high value both in the diagnosis of PSP and its subtypes, and also in specific application fields.MRI morphometrics have a limited value in discriminating PSP subtypes.
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Parkinson's disease(PD)and multiple system atrophy(MSA)are two common Parkinsonian syndromes with overlapping clinical manifestations, and clinical differential diagnosis is difficult.Lower urinary tract symptoms are one of the common non-motor symptoms of the two diseases.The incidence of lower urinary tract symptoms in MSA is higher, the onset is earlier, and the micturition period is more prominent.The urinary dysfunction in patients with PD is mainly caused by the central mechanism, leading to overactive bladder.MSA has more extensive lesions with both central and peripheral involvement, leading to overactive bladder and severe voiding dysfunction.Urodynamics can be used to evaluate bladder and urethral function.MSA has more prominent weak detrusor activity, residual urine volume, and early changes of urethral sphincter.The treatment of lower urinary tract symptoms in patients with PD is mainly based on anticholinergic drugs to improve overactive bladder, while in MSA patients with increased residual urine volume, intermittent catheterization is the main method to improve lower urinary tract symptoms.This article reviewed the epidemiology, pathological mechanism, urodynamics and treatment of lower urinary tract symptoms of the two diseases, so as to assist in their differential diagnosis and treatment.
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Objective:To analyze the cognitive dysfunction and its influencing factors in patients with multisystem atrophy(MSA).Methods:A total of 143 hospitalized patients with MSA in our hospital from January 2015 to January 2020 were selected as the research objects.The cognitive function was evaluated by Mini-Mental State Examination(MMSE)and Montreal Cognitive Assessment(MoCA)scales, meanwhile, the influencing factors of cognitive function were analyzed.Results:The average score of MMSE scale of 143 MSA patients ranged from 9 to 30 points, and the MMSE test identified 86 patients(60.1%)with cognitive impairment, including 58 cases(40.6%)with mild cognitive impairment, 27 cases(18.9%)with moderate cognitive impairment and 1 case(0.7%)with severe cognitive impairment.The average score of MoCA scale of 143 MSA patients ranged from 5 to 30 points, among which 111 cases(77.6%)had cognitive impairment, including 69 cases with mild cognitive impairment(48.3%), 35 cases with moderate cognitive impairment(24.5%)and 7 cases with severe cognitive impairment(4.9%). Both the total scores of MMSE and MoCA scores of MSA patients were not correlated with age of onset, old age, gender, disease duration, type of MSA(MSA-C), orthostatic hypotension(OH), urinary retention, positivity of anal sphincter electromyography(ASEMG), triglyceride(TG), total cholesterol(TC), high density lipoprotein cholesterol(HDL-C), low density lipoprotein cholesterol(LDL-C), fasting blood glucose and glycosylated hemoglobin, serum uric acid and residual urine volume(all P>0.05). Logistic regression analysis showed that fasting blood glucose was a risk factor for cognitive impairment identified by MMSE( OR=2.224, 95% CI: 1.108-4.462, P=0.024); MSA-C( OR=3.905, 95% CI: 1.302-11.713, P=0.015)and TG( OR=3.494, 95% CI: 1.061-11.501, P=0.040)were risk factors for cognitive impairment identified by MoCA.There were no significant differences in above clinical characteristics between moderate to severe cognitive impairment patients and mild cognitive impairment patients identified by MMSE scale( P>0.05). Similarly, there were no significant differences in the above clinical characteristics between moderate to severe cognitive impairment patients and mild cognitive impairment patients by MoCA scale( P>0.05). Logistic regression analysis showed that TG was a risk factor for moderate to severe cognitive impairment identified by MMSE( OR=17.624, 95% CI: 2.596-119.669, P=0.003), while TC was a protective factor( OR=0.006, 95% CI: 0.000-0.714, P=0.036). Logistic regression analysis did not find the influencing factors of moderate to severe cognitive impairment as suggested by MoCA score. Conclusions:There is a high incidence of cognitive impairment in MSA patients.The type of MSA(MSA-C), blood glucose and blood lipid may be influence factors of cognitive impairment in MSA patients.Age of onset, old age, gender, disease duration, autonomic dysfunction, and uric acid have no significant correlation with cognitive impairment of MSA.
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@#Objective To explore the relationship between white matter hyperintensity (WMH) and clinical symptoms in patients with multiple system atrophy (MSA),and the risk factors of WMH in patients with MSA.Methods A total of 88 patients with MSA were included.The periventricular white matter hyperintensity (PVH) and deep white matter hyperintensity (DWMH) were assessed according to MRI and the Fazekas scale.The patients were divided into three groups according to the severity of PVH or DWMH.The baseline data,motor symptoms,cognitive function,urinary symptoms and constipation were compared among different groups.Logistic regression was used to analyze the risk factors of WMH in patients with MSA.Results A significant difference was detected in age at admission,age at onset,hypertension,coronary heart disease,UMSARS total score,UMSARS Ⅱ score and UMSARS Ⅳ score between different PVH grades.A significant difference was detected in age at admission,age at onset,hypertension,coronary heart disease,UMSARS total score,UMSARS Ⅰ score,UMSARS Ⅱ score and UMSARSⅣ score between different DWMH grades.Logistic regression analysis showed that age at admission and hypertension were independent risk factors for WMH in patients with MSA.Conclusion WMH is present in some MSA patients and related to motor symptoms and severity of the disease.Age and hypertension influence the occurrence of WMH.
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Multiple system atrophy (MSA) is a rare and rapidly-progressive neurodegenerative disorder, characterized by the combination of dysautonomia, poor levodopa responsive parkinsonism, cerebellar ataxia, and pyramidal tract signs. Insidious onset, clinical heterogeneity and progression of the disease complicate the difficulty of early diagnosis and challenge, the development of neuroprotective drugs. In order to improve the knowledge of diagnosis and treatment of the disease, this paper reviews advances in its diagnostic criteria, biomarkers of early diagnosis and management of the disease.
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Objective:We aimed to determine the impact of the COVID-19 pandemic on the “body function”, “activity”, and “participation” domains of functioning as defined in the International Classification of Functioning (ICF) for individuals with spinocerebellar degeneration (SCD) and multiple system atrophy (MSA).Methods:A questionnaire survey was administered to 1,000 members of the SCD/MSA patient association in Japan between November and December 2020. Questions related to COVID-19 were extracted from the multipurpose data, and the degree of COVID-19-related effects on each life function was assessed using a 7-point scale. Results were stratified into two groups based on age, care needs based on long-term care insurance coverage, and limited opportunities for rehabilitation. The χ2 test was used to examine differences between pairs of groups regarding impacts on functioning.Results:Data from 460 participants with SCD and MSA were analyzed. Participation restriction was reported in 54% of participants with SCD and 46% of participants with MSA, and impairment and activity limitation in approximately 20% of participants with both conditions resulting from the COVID-19 pandemic. Participants with low care needs reported more impairment in SCD and more participation restriction in MSA. About 24% of subjects reported limited opportunities for rehabilitation. MSA participants with these limitations reported greater impairment.Conclusion:The COVID-19 pandemic has had a particular impact on the participation domain of functioning in participants with SCD and MSA. The domains of body function and participation were found to be affected in participants with low care needs or limited opportunities for rehabilitation.
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Multiple system atrophy(MSA)is a rare and fatal neurodegenerative disease occurring in middle-aged and older people, mainly characterized by autonomic nervous system and motor dysfunction.At present, there is no effective method to prevent or reverse its progression, and its treatment is mostly symptom-based, with limited success.A large number of interventional trials have been conducted to explore new treatments for MSA, but there is no clearly effective disease-modifying therapy, probably as a result of poor understanding of the pathophysiological and physiological mechanisms underlying MSA, which may be influenced by multiple factors.The purpose of this paper is to review the existing intervention trials for disease modification therapy of MSA and to discuss the outlook for new therapeutic targets.
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Objective:To investigate the urodynamic characteristics in parkinsonian-type multiple system atrophy (MSA-P) and Parkinson′s disease (PD) patients with lower urinary tract symptoms (LUTS) and to identify the differential diagnostic ability of urodynamic study.Methods:Sixty-eight MSA-P patients and 85 PD patients with LUTS who underwent urodynamic studies and were hospitalized in the First Affiliated Hospital of Zhengzhou University from February 2017 to December 2020 were reviewed. The receiver operating characteristic (ROC) curve was used to evaluate the discriminatory power of urodynamic parameters.Results:Compared with PD, patients with MSA-P had shorter disease duration [2.70 (2.05, 3.00) years vs 5.00 (4.00, 6.00) years, Z=-7.44, P<0.001]. In free uroflowmetry (UFM) and pressure-flow study (PFS), patients with MSA-P showed lower maximal flow rate [Qmax; UFM-Qmax 6.00 (3.00, 8.75) ml/s vs 9.00 (6.00, 14.00) ml/s, Z=-4.31, P<0.001; PFS-Qmax[6.00 (3.00, 8.75) ml/s vs 9.00 (6.00, 14.00) ml/s, Z=-4.03, P<0.001] and larger postvoid residual [PVR;UFM-PVR 207.50 (113.75, 280.00) ml vs 45.00 (10.00, 117.50) ml, Z =-6.03, P<0.001; PFS-PVR 269.00 (148.75, 337.50) ml vs 75.00 (20.00, 167.50) ml, Z=-6.55, P<0.001)] with decreased compliance [42.65% (29/68) vs 14.12% (12/85), χ 2=15.68, P<0.001], decreased detrusor pressure at maximun flow rate [PdetQmax; 13.00 (6.00, 27.75) cmH 2O vs 27.00 (15.00, 39.50) cmH 2O, Z=-4.65, P<0.001; 1 cmH 2O=0.098 kPa] and impaired contractility [43.00 (34.00, 59.25) vs 79.00 (46.50, 100.00), Z=-5.44, P<0.001]. Compared with the MSA-P patients, detrusor overactivity (DO) was prominent in PD [54.41% (37/68) vs 78.82% (67/85), χ 2=10.34, P=0.001]. The ROC curve results showed that PFS-PVR had highest area under the curve (AUC), which was 0.81 (95% CI 0.74-0.88, P<0.001), followed by UFM-PVR, bladder contractility index and PdetQmax (0.78, 0.76 and 0.72, respectively). Conclusions:Patients with MSA-P showed lower Qmax and larger PVR with decreased bladder compliance and impaired contractility, while patients with PD had higher incidence of DO. The ROC results showed that PFS-PVR had the highest AUC and specificity in the differential diagnosis of MSA-P and PD,indicating that urodynamic study is a useful tool in differential diagnosis of patients with MSA-P and PD with lower urinary tract symptoms.
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Multiple system atrophy can have various forms of sleep disorders, including insomnia, rapid eye movement sleep behavior disorder, sleep-disordered breathing, periodic leg movements during sleep and excessive daytime sleepiness. This article will focus on the concept, classification, pathogenesis, clinical features, diagnosis and treatment, aiming to deepen clinicians′ understanding of the disease, which will be helpful for early diagnosis and treatment.
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Objective:To explore the clinical characteristics of urination disorders in multiple system atrophy (MSA). Methods:The clinical data and imaging urodynamic examination of twelve MSA patients with severe urination disorders from June, 2013 to March, 2021 were retrospectively analyzed. Results:Among twelve cases, there were eight cases with acontractile detrusor, one case with detrusor overactivity, one case with detrusor-sphincter dyssynergia, one case with filling detrusor hyperactivity and voiding-phase dyssynergia (DO+DSD), and one case with normal urodynamics. Conclusion:The clinical symptoms of MSA are complex, and the early diagnosis is difficult. Electrophysiological and MRI examinations can be helpful for diagnosis.