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OBJECTIVE:To thoroughly investigate clinical characteristics of progressive supranuclear palsy(PSP)in a Chinese population.METHODS:Computer-based online searches through China National Knowledge Infrastructure and Weipu Periodical Database were performed to collect case reports of PSP published between 1980 and 2009.Clinical characteristics were analyzed.RESULTS:A total of 58 studies comprising 105 patients(76 males and 29 females)were included.All cases were sporadic and free of family history.The mean age at onset was 60.6±9.1 years,and the mean course from onset of symptoms to diagnosis was 3.4±2.4 years.The male-to-female ra-tio was approximately 3:1.Onset was characterized by akinetic-rigid features and accounted for 34.3%of all cases,followed by early postural instability(25.5%),pseudobulbar palsy(9.8%),cogni-tive impairment(9.8%),and vertical supranuclear ophthalmoplegia(7.8%).With disease progres-sion,vertical supranuclear ophthalmoplegia was reported in 95.1%of cases,followed by aki-netic-rigid features(83.3%),pseudobulbar palsy(82.4%),axial dystonia(75.5%),cognitive impair-ment(72.5%),and early postural instability(69.6%).A total of 70.5%of patients exhibited abnormal electroencephalograms,and 21.4%exhibited mild abnormalities in cerebrospinal fluid.Brain CT scanning results of 37 patients showed 37.8%with midbrain and concurrent cerebral hemisphere atrophy,and 5.4%and 24.3%with midbrain and cerebral hemisphere atrophy,respectively.Brain MRI scanning results of 55 patients revealed a total of 16.4%patients with midbrain atrophy,23.6% with midbrain and concurrent cerebral hemisphere atrophy,32.7%with cerebral hemisphere atrophy,and 11%with brainstem atrophy.The percentage of midbrain atrophy revealed by MRI was greater than by CT.All 11 patients subjected to Mini-Mental State Examination scored<23.A total of 10 patients underwent brain electrophysiological examination,and 80%presented with abnormalities of cerebral-evoked potential.A total of 33.3%patients presented with neurogenic damage of anal sphincter electromyography.Nine cases were neuropathologically diagnosed,and all displayed tau-positive argyrophilic globous neurofibrillary tangles,tuft-shaped astrocytes,and coiled bodies.Gene diagnosis was not performed.A total of 40 patients were misdiagnosed,including 23(58%)as Parkinson’s disease and seven(18%)as Parkinsonism;44 patients were treated by madopar,ar-tane,or amantadine,and only seven(16%)improved in movement.No patients exhibited improved eye movement.Of 4 patients treated with madopar and dopa agonists,2 improved in movement.In addition,movement improved in 3 patients treated with madopar and monoamine oxidase B.CONCLUSION:PSP is a sporadic disease that often strikes middle or elderly individuals.There are no significant differences in age of onset between male and female patients,although disease pro-gression is more rapid in female patients,likely because the time from symptom onset to disease diagnosis in females is less than in male.Akinetic-rigid features and early postural instability are the most common symptoms of onset.With disease progression,the incidence of vertical supranuclear ophthalmoplegia and akinetic-rigid features increases.MRI is a common,sensitive,and noninva-sive supplemental test.However,PSP is frequently misdiagnosed as Parkinson’s disease or Park-insonism during the early stage,and anti-Parkinson treatments remain ineffective.
OBJECTIVE: To thoroughly investigate clinical characteristics of progressive supranuclear palsy (PSP) in a Chinese population. METHODS: Computer-based online searches through China National Knowledge Infrastructure and Weipu Periodical Database were conducted to collect case reports of PSP published between 1980 and 2009. Clinical A total of 58 studies comprising 105 patients (76 males and 29 females) were included. All cases were sporadic and free of family history. The mean age at onset was 60.6 ± 9.1 years, and the mean course from onset of symptoms to diagnosis was 3.4 ± 2.4 years. The male-to-female ra-tio was approximately 3: 1. Onset was characterized by akinetic-rigid features and accounted for 34.3% of all cases, followed by early postural instability (25.5 %), pseudobulbar palsy (9.8%), cogni-tive impairment (9.8%), and vertical supranuclear ophthalmoplegia (7.8%). With disease progres- sion, vertical supranuclear ophthalmoplegia was reported in 95.1% of cases, followe A total of 70.5% of the patients were diagnosed with dystonia (75.5%), cognitive impairment (72.5%), and early postural instability (69.6%) by aki-netic- rigid features (83.3% of patients showed abnormal abnormalities in cerebrospinal fluid, and 21.4% exhibited mild abnormalities in cerebrospinal fluid. Brain CT scanning results of 37 patients showed 37.8% with midbrain and concurrent cerebral hemisphere atrophy, and 5.4% and 24.3% with midbrain and cerebral hemisphere atrophy, respectively. Brain MRI scanning results of 55 patients revealed a total of 16.4% patients with midbrain atrophy, 23.6% with midbrain and concurrent cerebral hemisphere atrophy, 32.7% with cerebral hemisphere atrophy, and 11% with brainstem atrophy. Percentage of midbrain atrophy revealed by MRI was greater than by CT. All 11 patients subjected to Mini-Mental State Examination scored <23. A total of 10 patients underwent brain electrophysiological examination, and 80% presented with abnormalities of cerebral-evoked potential. A total of 33.3% patient spresented with neurogenic damage of anal sphincter electromyography. Nine cases were neuropathologically diagnosed, and all displayed tau-positive argyrophilic globous neurofibrillary tangles, tuft-shaped astrocytes, and coiled bodies. General diagnosis was not performed. A total of 40 patients were misdiagnosed, including Of the 42 Parkinson’s disease and seven (18%) as Parkinsonism; 44 patients were treated by madopar, ar-tane, or amantadine, and only seven (16%) improved in movement. 4 patients treated with madopar and dopa agonists, 2 improved in movement. Addition, movement improved in 3 patients treated with madopar and monoamine oxidase B. CONCLUSION: PSP is a sporadic disease that often strikes middle or elderly individuals.There are no significant differences in age of onset between male and female patients, although disease pro-gression is more rapid in female patients, likely because the time from symptom onset to disease diagnosis in females is less than in male.Akinetic-rigid features and early postural instability are the most common symptoms of onset. With disease progression, the incidence of vertical supranuclear ophthalmoplegia and akinetic-rigid features increases. MRI is a common, sensitive, and noninva-sive supplemental test .Where, PSP is frequently misdiagnosed as Parkinson’s disease or Park-insonism during the early stage, and anti-Parkinson treatments remain ineffective.