Lower urinary tract symptoms in Parkinson's disease and multiple system atrophy / 中华老年医学杂志

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.

Assessment of conventional magnetic resonance morphological measurements in diagnosis and differential diagnosis of progressive supranuclear paralysis / 中华老年医学杂志

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.

18F-FDG hybrid PET/MR radiomics based on different segmentation methods for distinguishing Parkinson′s disease from multiple system atrophy / 中华核医学与分子影像杂志

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.

Research progress on non motor symptoms of multiple system atrophy / 中国医师杂志

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.

The value of urodynamic study in the differential diagnosis of patients with parkinsonian-type multiple system atrophy and Parkinson′s disease with lower urinary tract symptoms / 中华神经科杂志

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.

Disease-modifying therapy for multiply system atrophy / 中华老年医学杂志

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.

The Impact of the COVID-19 Pandemic on Three Domains of Functioning of ICF in Participants with Spinocerebellar Degeneration and Multiple System Atrophy in Japan / The Japanese Journal of Rehabilitation Medicine

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.

Advances in the Diagnosis and Treatment of Multiple System Atrophy / 罕见病研究

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.

Clinical analysis of cognitive dysfunction and its influencing factors in patients with multisystem atrophy / 中华老年医学杂志

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.

Progress in sleep disorders in multiple system atrophy / 中华神经科杂志

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.

Urodynamic Manifestations of Severe Urination Disorders Caused by Multiple System Atrophy / 中国康复理论与实践

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.

Gray matter atrophy in parkinsons disease and the parkinsonian variant of multiple system atrophy: a combined roi- and voxel-based morphometric study

OBJECTIVES: Parkinson's disease (PD) and the parkinsonian variant of multiple system atrophy (MSA-P) are distinct neurodegenerative disorders that share similar clinical features of parkinsonism. The morphological alterations of these diseases have yet to be understood. The purpose of this study was to evaluate gray matter atrophy in PD and MSA-P using regions of interest (ROI)-based measurements and voxel-based morphometry (VBM). METHODS: We studied 41 patients with PD, 20 patients with MSA-P, and 39 controls matched for age, sex, and handedness using an improved T1-weighted sequence that eased gray matter segmentation. The gray matter volumes were measured using ROI and VBM. RESULTS: ROI volumetric measurements showed significantly reduced bilateral putamen volumes in MSA-P patients compared with those in PD patients and controls (p<0.05), and the volumes of the bilateral caudate nucleus were significantly reduced in both MSA-P and PD patients compared with those in the controls (p<0.05). VBM analysis revealed multifocal cortical and subcortical atrophy in both MSA-P and PD patients, and the volumes of the cerebellum and temporal lobes were remarkably reduced in MSA-P patients compared with the volumes in PD patients (p<0.05). CONCLUSIONS: Both PD and MSA-P are associated with gray matter atrophy, which mainly involves the bilateral putamen, caudate nucleus, cerebellum, and temporal lobes. ROI and VBM can be used to identify these morphological alterations, and VBM is more sensitive and repeatable and less time-consuming, which may have potential diagnostic value.

Progress in biomarkers of multiple system atrophy / 上海交通大学学报（医学版）

Multiple system atrophy is a neurodegenerative disease, which is characterized by a combination of autonomic failure, Parkinsonian features, pyramidal and cerebellar dysfunction, and is poorly reactive to levodopa. Few biomarkers with high sensitivity or specificity have been applicable in the diagnosis of multiple system atrophy, making it a challenge to correctly diagnose this disease. Therefore, some researches combined several biomarkers to improve the diagnosis accuracy. Apart from those in body fluid, progress has been made in new biomarkers of neuroimaging and pathology. In this review, the advances in the identification of biomarkers of multiple system atrophy are summarized, and some candidate biomarkers that worth more investigation, including proteins and miRNAs in blood or cerebrospinal fluid, parameters in molecular and functional imaging, and pathologic features of peripheral tissue are described.

Quantitative autonomic function test in differentiation of multiple system atrophy from idiopathic Parkinson disease / 中华医学杂志(英文版)

Background@#Differential diagnosis of idiopathic Parkinson disease (IPD) and multiple system atrophy-Parkinson type (MSA-P) is challenging since they share clinical features with parkinsonism and autonomic dysfunction. To distinguish MSA-P from IPD when the symptoms are relatively mild, we investigated the usefulness of the quantitative fractionalized autonomic indexes and evaluated the correlations of autonomic test indexes and functional status.@*Methods@#Thirty-six patients with parkinsonism (22 with IPD and 14 with MSA-P) in Soonchunhyang University Bucheon Hospital from February 2014 to June 2015 were prospectively enrolled in the study. We compared fractionalized autonomic indexes and composite autonomic scoring scale between patients with IPD and MSA-P with Hoehn and Yahr (H&Y) score ≤3. Parasympathetic indexes included expiratory/inspiratory ratio during deep breathing, Valsalva ratio (VR), and regression slope of systolic blood pressure (BP) in early phase II (vagal baroreflex sensitivity) during Valsalva maneuver. Sympathetic adrenergic indexes were pressure recovery time (PRT) and adrenergic baroreflex sensitivity (BRSa) (BP decrement associated with phase 3 divided by the PRT), sympathetic index 1, sympathetic index 3, early phase II mean BP drop, and pulse pressure reduction rate. Additionally, we compared the unified multiple system atrophy rating scale (UMSARS) and H&Y scores and the autonomic indexes in all patients.@*Results@#PRT was significantly different between the IPD and MSA-P groups (P = 0.004) despite the similar BP drop during tilt. Cutoff value of PRT was 5.5 s (sensitivity, 71.4%; specificity, 72.7%). VR (r = -0.455, P = 0.009) and BRSa (r = -0.356, P = 0.036) demonstrated a significant correlation with UMSARS and H&Y scores.@*Conclusions@#Among the cardiovascular autonomic indexes, PRT can be a useful parameter in differentiating the early stage of MSAP from that of IPD. Moreover, VR, and BRSa may be the optimal indexes in determining functional symptom severity.

Urodynamic evaluation of bladder function in patients with parkinsonism dominant multiple system atrophy with abnormal urination / 中华神经科杂志

Objective@#To investigate the urodynamic characteristics and clinical significance of bladder function in patients with parkinsonism dominant multiple system atrophy (MSA-P) with abnormal urination. @*Methods@#The clinical data of 58 patients with multiple system atrophy (MSA) and urinary dysfunction who were hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2015 to June 2018 were analyzed. The bladder function status of MSA-P patients was evaluated, and the characteristics of urinary motility examination results were analyzed. @*Results@#According to the diagnostic criteria, 51 patients were MSA, and seven patients were suspicious, all diagnosed as MSA-P. All MSA-P patients had abnormal urination with different symptoms, including dysuria (77.6%), frequent urination (62.1%), urgency (62.1%), urinary incontinence (65.5%), urinary retention (12.1%). Of the MSA-P patients with abnormal urination, the maximum flow rate (Qmax) was (12.46±2.85) ml/s, the average flow rate (Qave) was (5.19±2.06) ml/s, the voided volume was (171.73±11.47) ml, postvoid residual urine was (136.15±10.47) ml, maximal detrusor pressure was (20.78±3.55) cmH2O, bladder compliance was (16.59±2.91) ml/cmH2O, normal desire to void was (223.64±11.02) ml, and maximal capacity was (287.57±12.51) ml. The Qmax, Qave, voided volume, maximal detrusor pressure, bladder compliance, normal desire to void, and maximal capacity of patients with MSA-P with abnormal urination were significantly decreased, while the bladder residual urine volume was significantly increased, with statistically significant differences compared to normal reference values (P<0.05). @*Conclusion@#The urinary motility of bladder function in patients with MSA-P and urinary abnormalities is mainly characterized by decreased bladder compliance and detrusor dysfunction, while urodynamic testing is helpful for early diagnosis.

A Survey of Rehabilitation and the Life Situation of Japanese Patients with Spinocerebellar Degeneration and Multiple System Atrophy / The Japanese Journal of Rehabilitation Medicine

Objective：This study aimed to identify the characteristics of daily rehabilitation in patients with spinocerebellar degeneration (SCD), including those with multiple system atrophy, with a focus on physical status and social background.Methods：We analyzed questionnaire responses from 914 patients in Japan and conducted inter-group comparisons of backgrounds and rehabilitation-related information by dividing patients into a rehabilitation group, a self-directed exercise group, and a non-rehabilitation group.Results：Among all subjects, the rehabilitation group accounted for 67.9％, the self-directed exercise group for 17.7％, and the non-rehabilitation group for 14.3％．In the rehabilitation group, the percentage receiving services increased with a decline in the level of independent living and increase in coverage by long-term care insurance. The frequency and duration of rehabilitation showed a maximum distribution of 2-3 sessions/week for 40 minutes per session in the rehabilitation group and 7 sessions/week for 20 minutes per session in the self-directed exercise group. The intent to continue in the rehabilitation group was significantly greater than in the self-directed exercise group (p＝0.018), although subjective changes with rehabilitation showed no difference between groups.Conclusion：The percentage of SCD patients who participated in rehabilitation programs including self-directed exercise was high. However, the subjective effect was not clear. We consider it necessary to develop an environment in which experts can provide rehabilitation to all SCD patients, irrespective of the disease stage.

Analysis of serum biochemical indexes in patients with multiple system atrophy / 中华老年医学杂志

Objective To study changes in hepatic and renal function,serum lipids and homocysteine levels in patients with multiple system atrophy(MSA),and to explore the relationship of serum biochemical indexes with MSA and its severity.Methods A total of 86 hospitalized patients with MSA at our department from January 2014 to June 2017 were retrospectively analyzed.Eightyfive healthy subjects undergoing health physical examinations at our hospital during the same period were enrolled as a control group.Serum lipid levels and liver and renal function were compared between the two groups.After stratified by gender,the correlations of liver and renal function,serum lipids and homocysteine levels with MSA and its severity were analyzed.Results Serum levels of total cholesterol,high-density lipoprotein cholesterol and low-density lipoprotein cholesterol were lower in the MSA group than in the control group(P＜0.01).Serum levels of total cholesterol,high-density lipoprotein cholesterol and low-density lipoprotein cholesterol were lower in male patients with MSA than in male healthy controls(P＜0.01).Serum levels of uric acid were lower in female patients with MSA than in female healthy controls (P ＜ 0.05).Serum levels of total cholesterol and low-density lipoprotein cholesterol were lower in MSA patients aged under 60 years than in healthy controls aged under 60 years (P ＜ 0.01).Serum levels of total cholesterol (r =0.34,P ＜ 0.01),high-density lipoprotein cholesterol(r =-0.27,P ＜ 0.01),low-density lipoprotein cholesterol(r =0.32,P ＜ 0.01)and uric acid(r =0.15,P ＜ 0.05)were correlated with MSA,but they were not independent risk factors.Serum levels of total cholesterol(r =0.27,P ＜0.05) and low-density lipoprotein cholesterol (r =0.23,P ＜0.05)were correlated with the severity of MSA,but they were not independent risk factors (r =-0.381,P ＞0.05).Conclusions Serum levels of lipids and urea are associated with MSA,but they are not independent risk factors for MSA or its severity.Gender may affect the occurrence of MSA,but it needs to be further confirmed.

Clinical Milestones Preceding the Diagnosis of Multiple System Atrophy and Progressive Supranuclear Palsy: A Retrospective Cohort Study

OBJECTIVE: Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead to delayed referral to appropriate specialists and distress to patients, as well as delaying symptomatic treatment and participation in clinical trials. This work aimed to describe the symptoms that patients with MSA and PSP developed and plot their emergence relative to final diagnosis using a median onset in months. METHODS: Forty-seven patients from the United Kingdom with MSA or PSP diagnosed by a movement disorder specialist were interviewed with carers or relatives to establish milestone onset. This was corroborated using clinical notes and letters. RESULTS: In the MSA cohort (n = 23), autonomic symptoms (median 5.5 months before diagnosis) and falls (median 1 month before diagnosis) were the two clinical milestones which occurred before diagnosis. In the PSP cohort (n = 24), falling was the only milestone which occurred before diagnosis (median of 18.5 months). CONCLUSION: This study shows that PSP patients experience falling more than a year and a half an average before receiving a diagnosis and although MSA patients also tended to fall, this was much closer to the time of diagnosis. Further work with larger cohorts may illustrate whether these preliminary findings can be generalised to guide diagnosis and management.

The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases

OBJECTIVE: To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism. METHODS: The radiologic information systems at an academic center and affiliated veterans' hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient's neurologic symptoms. RESULTS: Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11). CONCLUSION: MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).

Spatiotemporal Gait Parameter Comparison for Parkinson's Disease, Multiple System Atrophy, and Other Parkinsonism Diseases

The aim of this study was to compare and quantify the spatiotemporal and gait parameters obtained by foot pressure analysis during the gait in a group of Parkinson's disease (PD) patients compared with other Parkinsonism diseases, especially multiple system atrophy (MSA). Thirty-seven out of ninety-three patients who visited the center of neurology or rehabilitation with features of Parkinsonism were recruited. Spatiotemporal gait parameters were collected using gait analysis system. The results did not differ in terms of the stride length, step width, double stance phase, stride time, cadence, velocity, gait line and single support line differences, anterior-posterior position of center of pressure, and maximal gait line velocity; the lateral symmetry showed a significant difference between the PD and the MSA groups (p < 0.05). The study evaluated the differences in terms of spatiotemporal parameters between the PD and MSA along with other Parkinsonism diseases; it showed that the PD patients had a gait tendency to deviate laterally compared to the MSA patients. The result suggests conducting the gait foot pressure analysis might help distinguish PD from other Parkinsonism diseases in early stage, aiding the early decision for the treatment plans.