| 2 | Clinical features of progressive supranuclear palsy in 105 Chinese patients显示文摘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. | Jing Hou Ruibiao Guo Tong Chen Xiaohong Zhang Weiping Wu Zhenfu Wang | 2011 | Neural Regeneration Research2011，6，2： | 0 |
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