Second consensus statement on the diagnosis of multiple system atrophy
Second consensus statement on the diagnosis of multiple system atrophy
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DOI：
10.1212/01.wnl.0000324625.00404.15
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发表时间：
2008-08-26
期刊：
影响因子：
9.9
通讯作者：
Vidailhet, M.
中科院分区：
文献类型：
--
作者：
Gilman, S.;Wenning, G. K.;Vidailhet, M.
背景：1998年关于多系统萎缩（MSA）的共识会议建立了广泛接受的诊断标准。从那时起，临床，实验室，神经病理学和影像学研究已经推进了该领域，需要一个新的诊断标准的评估。我们在2007年举行了第二次共识会议，并介绍了结果here.Methods：专家在临床，神经病理学，影像学方面的MSA被邀请参加为期2天的共识会议。参与者被分为五组，包括帕金森病，小脑，自主神经，神经病理学和成像方面的专家。每个小组在会议之前独立地为其专业领域编写诊断标准。这些标准进行了讨论和调和会议期间使用consensus methods.Results：新的标准保留了诊断类别的MSA与主要帕金森综合征和MSA与主要小脑共济失调指定的主要运动功能，也保留了明确的，可能的，和可能的MSA的名称。明确的MSA需要神经病理学证明CNS α-突触核蛋白阳性胶质细胞胞质包涵体伴纹状体黑质或橄榄脑桥小脑结构的神经退行性变化。可能的MSA需要散发的、进行性的成人发作性疾病，包括严格定义的自主神经功能衰竭和左旋多巴反应不良的帕金森综合征或小脑共济失调。可能的MSA需要一个散发性的，进行性的成人发病的疾病，包括帕金森综合征或小脑共济失调和至少一个功能提示自主神经功能障碍加上一个其他功能，可能是一个临床或neuroimaging abnormality.Conclusions：这些新的标准简化了以前的标准，已纳入目前的知识，并有望提高未来的评估疾病。
Background: A consensus conference on multiple system atrophy (MSA) in 1998 established criteria for diagnosis that have been accepted widely. Since then, clinical, laboratory, neuropathologic, and imaging studies have advanced the field, requiring a fresh evaluation of diagnostic criteria. We held a second consensus conference in 2007 and present the results here.Methods: Experts in the clinical, neuropathologic, and imaging aspects of MSA were invited to participate in a 2-day consensus conference. Participants were divided into five groups, consisting of specialists in the parkinsonian, cerebellar, autonomic, neuropathologic, and imaging aspects of the disorder. Each group independently wrote diagnostic criteria for its area of expertise in advance of the meeting. These criteria were discussed and reconciled during the meeting using consensus methodology.Results: The new criteria retain the diagnostic categories of MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia to designate the predominant motor features and also retain the designations of definite, probable, and possible MSA. Definite MSA requires neuropathologic demonstration of CNS alpha-synuclein-positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structures. Probable MSA requires a sporadic, progressive adult-onset disorder including rigorously defined autonomic failure and poorly levodopa-responsive parkinsonism or cerebellar ataxia. Possible MSA requires a sporadic, progressive adult-onset disease including parkinsonism or cerebellar ataxia and at least one feature suggesting autonomic dysfunction plus one other feature that may be a clinical or a neuroimaging abnormality.Conclusions: These new criteria have simplified the previous criteria, have incorporated current knowledge, and are expected to enhance future assessments of the disease.