| Background and Purpose: Multiple system atrophy, also known as multi-systemdegeneration divided into two subtypes:cerebellar-predominant(MSA-C) andparkinsonism--predominant(MSA-P). MSA is commonly regarded a sporadic、rapidlyprog ressive disease, no effective treatment. Studies abroad have shown that mediansurvival of MSA ranging from6-9years, died of multiple system atrophy-relatedcomplications, there was no survival analysis on patients with multiple system atrophy,With survival description in patients with multiple system atrophy in our study, todetermine what clinical factors affect the progression and survival prognosis andprovide evidence-based medicine for the Chinese population.Methods: Follow-up67patients with multiple system atrophy patients likely andpossible diagnosis from April2005to September2009in the General Hospital of PLA,survival description with Kaplan-Meier method, then Cox proportional hazards modelswere used to find the significant factors influencing the prognosis.Results1.lost24cases; followed43patients(24men and19women; mean±SD age at onset,55±8.1years)；due to disease progression or complications，32cases (55.7±8.3)died；11cases (53.2±7.7) survived.2.compared MSA-C with MSA-P,the differences of gender, age of onset, initialsymptoms, autonomic dysfunction type, anal EMG abnormalities and radiographicchanges in the proportion were not statistically significant of the followed43patients,3.32deaths of patients, including MSA-C19cases, MSA-P13cases; mainly because ofinfection and sudden death.4.with Kaplan-Meier survival analysis method, median survival time was7.4years;According to the43patients we followed,3,4,5,7,9-year survival rates were:90.7%，74.4%，55.8%，41.3%，29.9%. 5、Cox proportional hazards model analysis showed that gender (P=0.798), age (≥55, p=0.320), classification (P=0.394) and the first symptom (P=0.270) were notstatistically significant.Conclusion:Multiple system atrophy is a chronic and progressive disease with shortersurvival, mostly because of respiratory system diseases and sudden death, no favorableprognostic factors affected the disease progression were found. Background and objective： Multiple system atrophy is known as sporadic progressiveneurological degeneration disease, the main clinical manifestations are autonomicdysfunction, cerebellar ataxia, Parkinson-like symptoms, in the2008revised diagnosticcriteria,dementia was considered an exclusion criteria[1], but doctors often found MSApatients varying degrees of cognitive dysfunction, especially executive function.Weassess Executive function characteristics in patients with multiple system atrophy byneuropsychological tests and to analyze the factors may be interrelated.Methods: The study was composed of two groups of multiple system atrophy（MSA）patients,21patients with cerebellar-predominant(MSA-C),13patients withparkinsonism-predominant(MSA-P), one group of18idiopathic Parkinson’s diseasepatients，one control group of18cases，four groups were compared with each other byneuropsychological tests.Result:1.MSA-C and PD group had lower MoCA、semantic fluency test scores than thecontrol group，the MSA-C group was more significant (p <0.01).2.There were significant differences in digital-symbol/graphics-symbol conversionquiz between patients and the control (p <0.05)；There was as well significantdifference between MSA patients and the control group in all Stroop tests（p<0.05），which PD patients were worse than the control only in cardC（p<0.01）.3.Trail-making test of MSA-P group was worse than both PD group（p<0.05）and thecontrol(p<0.01), Clock drawing test was worse than the control (p<0.05).4.cognitive impairment in patients with multiple system atrophy was significantlycorrelated with the abnormalities of external anal sphincterelectromyography(EAS-EMG)(p <0.05）. Conclusion:1.cognitive impairment occurs in patients of multiple system atrophy and Parkinson’sdisease, especially the executive dysfunction.,but there are no significant differencesbetween them.2.Stroop tests and digital-symbol/graphics-symbol conversion tests are useful inassessing executive dysfunction in patients with multiple system atrophy andParkinson’s disease.3.cognitive impairment is more severe in patients with multiple system atrophy ofabnormal EAS-EMG but was no significant correlation with age, gender, education,duration of disease, presence or absence of orthostatic hypotension and typicalradiographic changes. Background and objective: Multiple system atrophy (MSA) complex clinical symptom,and exists overlap with other diseases of the nervous system, such as idiopathicParkinson’s disease, especially there is no pons cross sign, putamen fracturecharacteristic changes of traditional imaging in the early period of the disease, thedifferential diagnosis is more difficult, domestic and foreign scholars start to search theevidence of differential diagnosis with functional MRI.This study wa to investigate therole of diffusion tensor imaging(DTI) in identification between multiple system atrophyparkinsonism-predominant (MSA-P) and multiple system atrophy predominantcerebellar ataxia (MSA-C) or Parkinson’s disease (PD).Method: From December2012to November2013,the study was composed of twogroups of multiple system atrophy patients,8patients withparkinsonism-predominant,11patients with cerebellar-predominant,one group of13idiopathic Parkinson’s disease patients in General Hospital of PLA，and one controlgroup of18cases,four groups were compared with each other by scores ofneuropsychological assessment and parameters obtained from diffusion tensor imaging(DTI) examination which included25ROI(region of interests).Results1.MD in both putamen and the left substantia nigra、thalamus、external capsule ofMSA-P group were significantly different with MSA-C group and PD group;Significantly higher MD in both middle cerebellar peduncle、medulla oblongata weredemonstrated in MSA-C group than that of other groups (P <0.01)2.FA in the left external capsule、 thalamus and right frontal lobe、occipital lobe weresignificantly different between MSA-P group and MSA-C group; PD group hadsignificantly different FA in the left occipital lobe、 frontal lobe compared with MSA-Pgroup (P <0.01). 3.MD in the bilateral knees of internal capsule, left external capsule and FA in the leftforelimb of internal capsule, putamenthalamus and right cerebral peduncle of PD groupwere significantly different with the control (P <0.05).Conclusion: Diffusion tensor imaging(DTI) can be used to differ MSA-P type fromMSA-C type or PD. |
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