A novel grading scale for striatonigral degeneration (multiple system atrophy)
A novel grading scale for striatonigral degeneration (multiple system atrophy)
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DOI：
10.1007/s007020200025
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发表时间：
2002-01-01
影响因子：
3.3
通讯作者：
Jellinger, K
中科院分区：
文献类型：
--
作者：
Wenning, GK;Seppi, K;Jellinger, K
纹状核变性（SND）通常被认为是多系统萎缩（MSA）患者左旋多巴无反应性帕金森病的神经病理底物。MSA的其他神经病理学特征包括橄榄桥小脑萎缩（OPCA）和神经节前交感脊髓病变。对正在进行的自然史研究或神经保护干预试验中招募的MSA患者的临床病理评估将需要对MSA病理进行标准化分级。基于25例MSA尸检病例，我们提出了一种新的SND分级量表，该量表可以基于黑质、壳核、尾状核和苍白球中神经元丢失、星形胶质增生和α -突触核蛋白阳性胶质细胞质内含物（GCIs）的存在，对病变严重程度进行半定量评估。SND I级定义为致密黑质（SNC）变性，纹状体相对保留，但后壳核有少量胶质增生和gci （MSA最小变化）。SND II级的特征是神经元丢失、星形胶质细胞形成以及SNC和壳核后/背外侧存在gci。尾状核和外苍白球可能表现出轻微的神经胶质瘤。纹状体病变严重，并延伸到前腹内侧亚区。尾状核和苍白球有神经元丢失。II级gci比III级SNC和壳核丰富。初步的临床病理相关性研究表明，与III级患者相比，SND I级和II级患者帕金森残疾程度较轻，初始左旋多巴反应性较好。需要前瞻性临床病理研究来验证拟议的SND分级标准，并可能导致进一步的细分，特别是SND III级。
Striatonigral degeneration (SND) is commonly thought to represent the neuropathological substrate of L-Dopa unresponsive parkinsonism in patients with multiple system atrophy (MSA). Other neuropathological hallmarks of MSA include olivopontocerebellar atrophy (OPCA) and preganglionic sympathetic spinal cord lesions. Clinicopathological evaluation of MSA patients recruited into ongoing natural history studies or neuroprotective intervention trials will require standardized grading of MSA pathology. Based on 25 autopsy cases of MSA, we propose a novel SND grading scale which allows semiquantitative assessment of lesion severity based on neuronal loss, astrogliosis and presence of alpha-synuclein positive glial cytoplasmic inclusions (GCIs) in substantia nigra, putamen, caudate nucleus, and globus pallidus. SND grade I is defined as degeneration of the substantia nigra pars compacta (SNC) with relative preservation of the striatum except for minimal gliosis and GCIs in the posterior putamen ("minimal change MSA"). SND grade II is characterized by neuronal loss, astrogliosis and presence of GCIs in SNC and posterior/dorsolateral putamen. Caudate nucleus and external globus pallidus may exhibit slight gliosis. Striatal pathology is severe and extends to anterior ventromedial subregions in SND grade III. There is neuronal loss in caudate nucleus and globus pallidus. GCIs are more abundant in grade II than grade III SNC and putamen. Preliminary clinicopathologic correlation studies suggest milder parkinsonian disability and better initial L-Dopa responsiveness in SND grade I and II cases compared to grade III cases. Prospective clinicopathologic studies are required to validate the proposed SND grading scale and may result in further subdivisions, particularly of SND grade III.