Globular glial tauopathies (GGT): consensus recommendations
Globular glial tauopathies (GGT): consensus recommendations
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DOI：
10.1007/s00401-013-1171-0
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发表时间：
2013-10-01
影响因子：
12.7
通讯作者：
Kovacs, Gabor G.
中科院分区：
文献类型：
--
作者：
Ahmed, Zeshan;Bigio, Eileen H.;Kovacs, Gabor G.
最近的研究强调了一组4-重复（4 R）tau蛋白病，其神经病理学特征为广泛的球状胶质包涵体（GGIs）。Tau免疫组织化学显示4 R免疫反应性球状少突胶质细胞和星形胶质细胞内含物，后者主要是Gallyas银染色阴性。这些病例与一系列临床表现相关，这些临床表现与基础tau病理学和神经变性的严重程度和分布相关。其异质性的临床病理学特征结合其罕见性和识别不足，导致在文献中使用各种冗余术语描述了以GGIs为特征的病例。在这份报告中，一组神经病理学家形成了一个共识的术语和分类的情况下与GGIs。在研究了先前报告的疑似GGI病例（n = 22）的显微镜图像后，这组在神经退行性疾病诊断方面具有丰富经验的神经病理学家和至少一例GGI病例的既往经验记录一致认为：（1）所有回顾病例中均存在GGI;（2）球状星形胶质细胞包涵体的形态不同于簇状星形胶质细胞;（3）这些病例代表了许多不同的神经病理亚型。他们还一致认为，不同的形态亚型可能是一个独特的疾病实体谱的一部分，他们建议应使用总体术语球状神经胶质tau蛋白病（GGT）。I型病例通常表现为额颞叶痴呆，这与病理学的额颞分布相关。II型病例的特征为反映运动皮质受累和皮质脊髓束变性的锥体特征。III型病例可表现为额颞叶痴呆和运动神经元疾病的组合，其中额颞叶皮质、运动皮质和皮质脊髓束受到严重影响。锥体外系特征可存在于II型和III型病例中，并且白色物质的显著变性是所有GGT亚型的特征。提高检测和分类将是必要的神经病理学和临床诊断研究标准的建立在未来。
Recent studies have highlighted a group of 4-repeat (4R) tauopathies that are characterised neuropathologically by widespread, globular glial inclusions (GGIs). Tau immunohistochemistry reveals 4R immunoreactive globular oligodendroglial and astrocytic inclusions and the latter are predominantly negative for Gallyas silver staining. These cases are associated with a range of clinical presentations, which correlate with the severity and distribution of underlying tau pathology and neurodegeneration. Their heterogeneous clinicopathological features combined with their rarity and under-recognition have led to cases characterised by GGIs being described in the literature using various and redundant terminologies. In this report, a group of neuropathologists form a consensus on the terminology and classification of cases with GGIs. After studying microscopic images from previously reported cases with suspected GGIs (n = 22), this panel of neuropathologists with extensive experience in the diagnosis of neurodegenerative diseases and a documented record of previous experience with at least one case with GGIs, agreed that (1) GGIs were present in all the cases reviewed; (2) the morphology of globular astrocytic inclusions was different to tufted astrocytes and finally that (3) the cases represented a number of different neuropathological subtypes. They also agreed that the different morphological subtypes are likely to be part of a spectrum of a distinct disease entity, for which they recommend that the overarching term globular glial tauopathy (GGT) should be used. Type I cases typically present with frontotemporal dementia, which correlates with the fronto-temporal distribution of pathology. Type II cases are characterised by pyramidal features reflecting motor cortex involvement and corticospinal tract degeneration. Type III cases can present with a combination of frontotemporal dementia and motor neuron disease with fronto-temporal cortex, motor cortex and corticospinal tract being severely affected. Extrapyramidal features can be present in Type II and III cases and significant degeneration of the white matter is a feature of all GGT subtypes. Improved detection and classification will be necessary for the establishment of neuropathological and clinical diagnostic research criteria in the future.