| Objective: To research OPCA patients with disease duration, age, age ofonset, clinical symptoms and signs, and olives, pons, cerebellar atrophy imageperformance degree and to analysis the risk factors of the OPCA severegroup,it was contributed to the level of clinical diagnosis and theprognosis.Further to research the relationship between clinical manifestationsand the mental retardation in the OPCA patients. Understanding of the OPCApatients the occurrence of mental retardation and the law of development, itwas contributed to early treatment of clinic.Methods: Collected outpatient and hospitalization of100cases of OPCApatients after diagnosis,treatment and a MRI examination in department ofneurology of second hospital of Hebei medical university between2011and2013, Divided them into two groups, the mild group with increased cerebellarsulci, slightly thinner brainstem,but with no significant change in the pons andthe severe group with visible atrophy pons ventral, severe atrophy medullaoblongata olive, the enlarged fourth ventricle, expanded brainstem basalcistern according to the degree of atrophy of its imaging performance.Recorded of all patients with the following clinical datas: age, sex, age ofonset, duration, years of education, clinical symptoms and signs, and imagingfindings.Detected the patients with the Revised Hasegawa Dementia Scale(HDS-R). The results were divided into normal mental and intellectualdisabilities.Divided the severity of the disease based on the patient’s motorfunction into the following three grades:Grade â… ,the patients were able towalk independently and took care of themselves; Gradeâ…¡, they were able towalk with the assistance from others or with the aid of crutches, Grade â…¢,theywere in bed and could not take care of themselves.1The results were analyzed by SPSS13.0statistical software. Compared age, age of onset, duration,the first symptom, clinical manifestations andmotor function between the mild and severe groups. The logistic regressionwas used to analyze OPCA patients with mild group and severe groupassociated with the initial symptoms and clinical symptoms, signs and motorfunction.2Compared sex and motor function, age, duration, years of educationbetween the intellectual disabilities and normal intelligence of the severegroup. P<0.05was considered statistically significant.Results:1The basic Information of OPCA patients:There were48patients in themild group,26cases were male (54.2%) and22were females (45.8%), agingfrom15to75years old,the mean age was52.1±12.38years old. The durationwas0.5to10years and the average duration was3.3±1.97years.The age ofonset was14to65years old,and the average age of onset was48.9±11.62years old.41cases (85.4%) had instability or weakness of lower limbs,3caseshad dizziness,2cases had the unclear language,2cases had tremor of thelimbs.18cases had instability nose level (37.5%),20cases had languagebarriers(41.7%),24patients had nystagmus (50.0%),24cases had horizontalnystagmus, no case had rotating or vertical nystagmus.15cases had dfficultyswallowing and choking water (31.3%),4patients had extrapyramidal damagesymptoms (8.3%),2patients showed increased muscle tone and2cases hadtremors, No one had orthostatic hypotension (0.0%),8cases had urineobstacles (6.7%),8patients had positive Babinski sign (16.7%),20patientshad tendon hyperreflexia (41.7%),12cases had mental retardation (25.0%).36cases had motor function of â…¡ gradeand below (75.0%).There were52patients in the severe group,24cases were male (46.2%)and28were females (53.8%), aging from20to73years old,the mean agewas52.9±13.18years old. The duration was1to13years and the averageduration was5.1±2.64years,.The age of onset was18to65years old, and theaverage age of onset was47.9±12.28years old.44cases (84.6%) hadinstability or weakness of lower limbs,3cases had dizziness,2cases had the unclear language,3cases had tremor of the limbs.46cases had Instability noselevel (88.5%),36cases had language barriers (69.2%),28patients hadnystagmus (53.8%),24cases had horizontal nystagmus,and24cases hadrotating nystagmus.30cases had difficulty swallowing and choking water(57.7%),8patients had extrapyramidal damage symptoms (8.3%),5patientsshowed increased muscle tone and3cases showed tremors,4case hadorthostatic hypotension (7.7%),16cases had urine obstacles (30.8%),18patients had positive Babinski sign (34.6%),37patients had tendonhyperreflexia (71.2%),26cases had mental retardation (50.0%).37cases hadmotor function ofâ…¡ gradeand below (71.2%).Detected all the patients with the Revised Hasegawa Dementia Scale(HDS-R), including26cases (50.0%) had presence of mental retardation,26cases (50.0%) didn’t have mental retardation.HDS-R scores of smart disorders were from8to30points with an averageof24.0points, There were26patients in the mental retardation group,16cases were male (59.3%) and11were females (40.7%), aging from35to73years old,the mean age was53.5years old. The duration was1to13years, withthe average duration of6.0years,. the years of education was0-12years, theaverage years of education was6.0years. Patients of mental retardation groupwere divided into three grades according to motor function: There were6cases in Grade I, There were9cases in Gradeâ…¡, There were11cases inGrade â…¢.HDS-R scores of patients with smart normal group were from31to31.5points, with an average of31.5points:There were26patients in the smartnormal group,18cases were male (72.0%) and7were females (28.0%), agingfrom20to72years old,the mean age was52.0years old. The duration was1to11years, with the average duration of4.0years,. the years of educationwere0-16years, the average years of education was9.0years. Patients withsmart normal group were divided into three grades according to motorfunction: There were8cases in Grade I, There were11cases in Grade â…¡,There were4cases in Grade â…¢. 2comparison between the mild and severe groupsDisease duration between the two groups were significantly different (P<0.05), Disease duration of patients of mild group was shorter than thepatients of severe group. Instability nose level,language barriers,difficultyswallowing and choking water, intellectual decline,positive Babinski sign, andtendon hyperreflexia between the two groups were significantly different (P <0.05) Severe group was more likely to have instability nose level,languagebarriers,difficulty swallowing, drinking cough and intelligence decline andpyramidal tract damage.3Multivariate analysisLogistic regression analysis of first symptom,clinical symptoms andsigns, the entry standard was0.05, the removalstandard was0.10,and thelanguage barriers (X1), difficulty swallowing and choking water (X2),theintellectual decline (X3) came into the equation, logitP=-2.149+1.156X1+1.424X2+0.601X3,χ2=43.568, P=0.000, Language barriers,difficultyswallowing and choking water, intellectual decline were independent riskfactors for severe atrophy.4Comparison between the normal intelligence and intelligent disorders in thesevere groupAge, years of education and duration between the normal intelligencegroup and the mental retardation group were significantly different (P <0.05),The patients of the mental retardation group was older than the normalintelligence group, the years of education of the mental retardation group wasshorter than normal intelligence group, duration of the mental retardationgroup was longer than the normal intelligence group.There was no significant difference between sex of the normalintelligence group and the mental retardation group (P>0.05), Motor functionof the mental retardation group was no better than the normal intelligencegroup. There were11cases in Gradeâ…¢ of the mental retardation group(42.3%), There were4cases in Gradeâ…¢ of the normal intelligence group(15.4%)。. Conclusion: OPCA patients with longer duration had heavier atrophy.The proportion of clinical signs and symptoms between the mild group andthe severe group were different. OPCA patients with language barriers,difficulty swallowing, drinking cough and intelligence decline may imagingfindings severe atrophy. Patients with severe atrophy of smart decline werelikely to have longer course, be older and have less years of education. |