A Clinical Characteristics Analysis Of 267 Patients With Multiple System Atrophy

ObjectiveMultiple system atrophy(MSA)is a kind of adult onset,sporadic neurodegenerative disease of unknown etiology,the clinical manifestations are Parkinsonism,cerebellar ataxia,autonomic nerve dysfunction,pyramidal tract signs,etc.At present,the cause of MSA is unknown,and the main pathological change is the eosinophilic inclusion bodies found in the cytoplasm of glial cells.In addition,the main symptoms of MSA are easy to be ignored and difficult to differentiate,so the diagnosis and treatment of MSA is relatively difficult.Therefore,retrospective analysis was used in this study,case data of 267 patients with multiple system atrophy diagnosed in our hospital from January 2015 to January 2020 were selected and analyzed to comprehensively understand the clinical characteristics of MSA and improve the clinical diagnosis accuracy of MSA.MethodsCase data of 267 patients with multiple system atrophy diagnosed in our hospital from January 2015 to January 2020 were retrospectively collected.The general data,clinical manifestations,first symptoms,imaging features,autonomic nerve function,anal sphincter function,urodynamics and other aspects of the patients were comprehensively analyzed.According to their motor symptoms,the patients were divided into two subtypes:MSA-C with cerebellar symptoms and MSA-P with parkinsonism.SPSS 19.0 statistical software was used to process data.Measured data are in accordance with the normal distribution of mean ± standard deviation means.The t test of two independent samples was used in the two groups.The counting data is expressed in frequency(%)and rates.Rates were compared using the chi-square.P<0.05 was statistically significant.Results1.General InformationAmong the 267 cases of MSA,165 were males and 102 were females.The onset age ranged from 39 to 78 years.The average age was 56.43±5.37 years.The course of the disease ranges from 4 months to 9 years,with an average of(5.2±2.1)years.Two independent sample t test showed that there was no statistically significant difference in age of onset between MSA-P and MSA-C subsets(t=1.738,P=0.067).2.Clinical featuresThe clinical manifestations of MSA-C include dizziness,walking instability,inarticulate speech,nystagmus,and step-base width.Physical examination can have heel-knee-tibia test,finger-nose test,Romberg test,rotation test and so on positive.Clinical manifestations of MSA-P include bradykinesia,quiescent tremor,postural abnormalities,and increased muscle tension in gear or lead tube rigidity.MSA patients are often accompanied by autonomic nerve dysfunction,symptoms such as dizziness,urination disorders,constipation.3.First SymptomsThe first symptom was cerebellum,followed by extrapyramidal symptoms.Among the 267 patients with MSA,238 patients had motor symptoms as the first symptoms and 29 patients had non-motor symptoms as the first symptoms.There were 182 cases of MSA-C,among which walking instability was the most common,followed by dizziness,syncope and dysarthria.There were 85 cases of MSA-P,the most common of which was bradykinesia,followed by myotonia and limb tremor.4.ImagingIn 182 patients with MSA-C type,the main manifestations of cranial MRI were brain stem atrophy,cerebellar atrophy and pons "cross sign" and so on.In 85 patients with MSA-P type,cranial MRI mainly showed atrophy of the putamen,accompanying T2-weighted sequences hyperintense signal in the dorsolateral margin of the putamen("putaminal slit" sign).In this study,it was found that the probability of cerebellar atrophy in MSA-C patients increased with the duration of the disease.The changes of pons "cross sign" and cerebellar atrophy in MSA-C cranial MRI may be related to the prolonged duration of the disease.5.Dynamic electrocardiogram and ambulatory blood pressureAmong the 267 patients,88 took 24-hour ambulatory blood pressure,81 patients with MSA presented abnormal changes in circadian rhythm of blood pressure,mainly manifested as non-arytenoid and anti-arytenoid blood pressure patterns.In this study,non-arytenoid and anti-arytenoid blood pressure patterns were observed in 63(71.6%)and 21(23.8%)MSA patients,respectively.There were 4 cases of spoon type(3.4%).In this study,the incidence of nocturnal hypertension in 67 patients with MSA was 76.5%.The daytime mean diastolic blood pressure(dDBP),daytime mean systolic blood pressure(dSBP),nocturnal mean diastolic blood pressure(nDBP),and nocturnal mean systolic blood pressure(nSBP)of MSA were expressed as x±s,respectively(68.69±8.95)mmHg,(116.69±12.12)mmHg,(71.03±7.68)mmHg,(126.04±9.02)mmHg.Among the 267 cases of MSA,88 patients were monitored by dynamic electrocardiogram,among which 68 were abnormal.SDNN(ms),SDANN(ms),RMSSD(ms),LF/HF,SDNN Index,LF(ms2)HF(ms2)were expressed as±s.The MSA group was significantly lower than the normal reference level.SDNN Index,LF(ms2)HF(ms2)decreased significantly from the normal reference level.6.Anal sphincter functionOf the 267 patients,43 patients received external anal sphincter electromyography(EAS-EMG).According to the average duration of time extension,it can be divided into three categories:mild duration when the average duration is 10.0-11.9ms,moderate duration when the average duration is 12-13.9ms,and severe duration when the average duration is>14ms.There were 26 cases of msa-p and 17 cases of msa-c.Abnormal results were observed in 38 patients,31(81.6%)of whom had MUP time exceeding 10ms on average,18(47.4%)of whom had multiphase waves,and 13(34.2%)of whom had satellite potentials.31 of the 267 patients with MSA developed abnormal EAS-EMG results within 3 years of onset,but these patients did not develop abnormal head MRI changes.7.Urodynamic determinationThere were 147 patients who underwent urodynamic examination,among which 64 patients had abnormal urodynamic measurement,reduced or weak contractility of bladder detrusor muscle,and urinary retention symptoms.The maximum urine flow rate was(7.46±2.75)ml/s,the average urine flow rate was(5.21±3.17)ml/s,the bladder residual urine volume was(137.34±18.47)ml,the maximum bladder volume was(281.78 ± 15.71)ml,the bladder compliance was(17.52±4.31)ml/cmH2O,and the detrusor systolic pressure was(40.27±4.35)cmH2O(1cmH2O=0.098kPa).Among them,the maximum and average urine flow rate decreased significantly compared with the normal reference value,while the residual urine volume of the bladder increased significantly compared with the normal reference value,suggesting that the detrusor muscle contractility was impaired.Detrusor systolic pressure and bladder compliance were significantly lower than the normal reference value at the time of the maximum urine flow rate,and the maximum bladder volume was significantly lower than the normal reference value,which further confirmed the decline of detrusor function and bladder compliance.Conclusions1.The mean age of onset of MSA-P type and MSA-C type was basically the same.2.The clinical manifestations of msa-p and msa-c overlap,and there is a lack of highly specific laboratory and imaging diagnostic markers.Further clinical analysis and laboratory studies are needed.3.Dynamic blood pressure monitoring at 24h suggested that abnormal circadian rhythm of blood pressure often occurred in MSA,presenting as non-arytenoidal and anti-arytenoidal blood pressure patterns.4.The abnormal results of the external anal sphincter emg of MSA were earlier than those of the imaging changes.Combined with the clinical manifestations and the results of the neuroimaging examination,the early diagnosis accuracy of MSA could be improved.5.The changes of pons cross sign and cerebellar atrophy in msa-c cranial MRI may be related to the course of the disease,especially the occurrence of cerebellar atrophy,which can better predict the progress of the disease.6.Atypical MRI changes of "hummingbird sign" were observed in MSA,and follow-up should be paid attention to.