Clinical Observation Of 123 Cases Of Multiple System Atrophy

Multiple system atrophy(MSA),with its name first coined by Graham et al.in 1969,is a sporadic neurodegenerative disease characterized by progressive autonomic failure involving several systems.The cause of MSA is still unclear,although researches indicate that there are highly phosphorylated a-synuclein proteins widely existed in the brain of MSA patients.MLA often presents with autonomic nervous system failure,parkinson’s-like symptoms,ataxia,and damage of pyramidal tract.According to medical professors’ consensus reached in 1998,MSA can be divided into two subtypes based on clinical manifestations,which are MSA-C--mainly characterized by cerebellar ataxia and MAS-P-predominantly with parkinson’s-like symptoms.In both these two subtypes there are always one to several autonomic dysfunctions complicated in the course of disease progression.MSA often occurs with insidious onset and is difficult to diagnose with a high rate of misdiagnosis,posing severe threat to patients’ life and also heavy burden on families and the whole society.Currently,there is no medication or treatment proven to be effective in treating MSA.However,traditional Chinese medicine(TCM)has its advantage in the treatment of MSA and it has been shown in researches that TCM could relieve several MSA symptoms to some extent and there already are preliminary researches carried out in the symptomatology field.Nonetheless,the sample sizes of current researches are generally small and the symptomatological researches also need improvement,which show the understanding and treatment of MSA from the TCM perspective still requires further development.Objectiveto discuss on the regular pattern of MSA progression and TCM pattern identification.MethodsThis research made a retrospective analysis of the information of MSA in-and out-patients treated in the Second Affiliated Hospital of Beijing University of Chinese Medicine,which includes basic information,case history,symptoms and signs,laboratory test results and TCM four examinations(inspection,listening and smelling,inquiry,and palpation).We included 123 cases according to the MSA diagnosis criteria proposed in 2008,and divided the patients into groups of MSA-C and MSA-P based on their main symptoms.We used SPSS 19.0 to analyze the statistics and the data fit the normal distribution using x±s.We used T-test to compare data,frequency n(%)to demonstrate technical information,x2 to test,and we agreed that P<0.05 indicating statistical significance.Results1.General information:43.9%of the 123 included patients are female(male to female ratio--1.28:1);the incident ages are between 34 and 70 years old,and the mean incident age is 53.04±7.56 years old;the average duration is 5years;the misdiagnosis rate is 27.64%;with the mean misdiagnosis time period being 19months and mean diagnosis time being 40 months;6 cases may have family history of MSA;66.67%of the patients don’t have chronic diseases;44.92%of male patients used to drink much alcohol;the mean incident age of patients who drink alcohol is 50.68±8.25 years old,and that of those who don’t drink is 53.85±7.24 years old.2.Symptoms:The most common one of MSA first symptoms is ataxia,followed by urination disorder and sleep behavior disorder in rapid eye movement period and there are other symptoms such as dizziness,parkinson’s-like symptoms,and sexual dysfunction;it usually takes 9 months from onset to autonomic dysfunction,and 18 months to develop motor symptoms.The most common motor symptoms are unsteady gait and slurred speech(88.6%,87.1%),and other symptoms are bucking while drinking,limb rigidity,tremors(62.6%,31.7%,27.6%);the most common non-motor symptoms are sleep disorders and voiding dysfunction(95.1%,94.3%),followed by upright symptoms and orthostatic hypotension(90.2%),and other symptoms of perspiration disorder,skin temperature paresthesia,depression,anxiety,cognitive dysfunction(85.4%,62.6%,68.3%,55.3%);89.9%of male patients suffer from sexual dysfunction.Till the last time of follow-up,68.3%of the patients cannot walk independently,60.2%have urinary retention,65.0%suffer from incontinence of varying degrees,4.9%needs intermittent catheterization,8.1%experienced syncope,and 1.6%are confined to bed due to frequent syncope.The overall average UMSARS score is 44 and the average scores for Part Ⅰ/Ⅱ/Ⅳ are 20.6/20.8/2.6 respectively.The average score for MSA-C patients is 39.0 and the average score for MSA-P patients is 52.7.(P<0.05)There are significant differences between the two groups.3.Laboratory test results:87patients had run the head magnetic resonance imaging examination and 96.6%of them showed characteristic changes,the most common one among which are cerebellum atrophy and pons cross sign most common(93.1%,78.2%);other less-common changes include IV ventricular dilatation,brain stem and basal ganglia atrophy,abnormal signals in lateral and dorsal putamen putamen edge,dentate nucleus.35 patients had run residual urine bladder ultrasound,with average residual quantity being 127mL.There are 55 patients whose supine blood pressure and orthostatic pressure difference were above 30/1SmmHg and the average blood Pressure drop was 39.3/25.5mmHg.25 patients had run anal sphincter EMQ and 84.0%of them showed neurogenic damage.4.From the TCM perspective:In terms of etiology and pathogenesis,there are mainly three causes,including physical deficiency due to aging,pathogen attaching brain collaterals,and injury of the head.Among the 123 Patients,90.2%of them didn’t show symptoms until 50years old,41.5%used to drink much alcohol,smoke or suffered CO poisoning,and 9.8%had injured their head.In terms of symptoms,Patients mainly suffer from yang-deficiency symptoms such as lassitude of loins and knees,muscle weakness of waist and legs,sexual impotence,frequent urination,aversion to cold,profuse dreaming,unsteady movement;kidney yang deficiency symptoms such as dizziness and drowsiness,lassitude,and slow movement;and also symptoms of stirring of wind due to yin deficiency such as limb rigidity,tremor.patients with MSA generally have pale and dusky tongue,thin and white coating,and deep and fine pulse.Conclusion1.The number of male patients is larger than that of female patients.The number of MSA-C patients is larger than that of MSA-P patients.And the male patients with MAS-C are more than female patients,while the female patients with MAS-P are more than the male patients.2.The occurrence time of patients with MAS-C is earlier than those with MAS-P.3.The attack of this disease is hidden without specificity.And it is a sPoradic disease without sPecific familial disease history.4.1t is seldomly combined with chronic disease in heart and cerebral vessels.5.Some risk factors are cranial injury,CO poisoning,pesticide contiguity,smoking and alcohol consumption.The occurrence time of patient with alcohol consumption is earlier than patients without alcohol consumption.6.The initial symptom of this disease is mostly seen cerebellum symptoms,followed by bladder dysfunction,and RBD.The RBD symptom is generally 3 years earlier than other symptoms.Patients with initial symptom of parkinsonian-like syndrome are mostly patients with MAS-P,which suggests that the occurrence of autonomic symptoms is earlier than that of motor symptoms.7.The most commonly seen motor symptom is unsteady walking.Unsteady walking is often occurred in patients with MAS-C,while limb rigidity and tremor are mainly occurred in patients with MAS-P.Both of two subtypes are accompanied with autonomic dysfunction,cognitive disorder and affective disorder with various levels.8.The diseases develoPment can be evaluated by UMSARS scale.The disease is developing faster for patients with MAS-P than that for patients with MAS-C.9.In skull MRI examination,cerebellar atrophy is most commonly seen,followed by pons cross sign,which is the characteristic MRI sign of multiple system atrophy.10.In TCM theory,the disease is located in the brain,with etiology of weak body of elderly people,toxin damaging encephala and craniocerebral trauma.The main pathogenesis is the impotence and deficiency of the liver,the spleen and the kidney.The main symptom factors are yang deficiency with qi deficiency,yin deficiency and qi sinking.The syndrome differentiations are deficiency of the liver and the kidney syndrome,the deficiency of the sPleen yang and the kidney yang,and the yang deficiency and qi sinking syndrome.