Globular glial tauopathy Type II: Clinicopathological study of two autopsy cases
Globular glial tauopathy Type II: Clinicopathological study of two autopsy cases
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DOI：
10.1111/neup.12532
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发表时间：
2019-04-01
期刊：
影响因子：
2.3
通讯作者：
Takahashi, Hitoshi
中科院分区：
文献类型：
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作者：
Tanaka, Hidetomo;Kawakatsu, Shinobu;Takahashi, Hitoshi
球状神经胶质tau蛋白病（GGT）是四重复tau蛋白病，其特征在于存在两种类型的tau阳性球状神经胶质包涵体（GGI）：球状少突胶质细胞包涵体和星形胶质细胞包涵体（GOI和盖斯）。GGT分为三种不同的神经病理亚型：I型、II型和III型。我们报告了两例GGT患者-一名76岁的女性和一名70岁的男性-疾病持续时间分别为5年和6年。两名患者均表现出上下运动神经元体征和不自主运动，后者还患有痴呆，磁共振成像显示额颞叶脑萎缩。在神经病理学上，在这两种情况下，中央前回受到最严重的影响，在灰白质交界处，贝茨细胞几乎完全丧失，并出现GOI和卷曲体，其中有许多神经纤维。这两名患者还表现出许多其他中枢神经系统区域（包括基底神经节）的神经元丢失和GGI（主要是GOI）。除了区域严重程度外，两种情况下的分布模式基本相同。然而，盖斯在任何受影响的地区都不明显。基于GGIs的形态和分布模式，我们诊断目前的两个病人为GGT II型。前者的电镜和生化检查结果与诊断一致。据报道，II型病例的特征在于反映主要运动皮质和皮质脊髓束变性的锥体特征。目前的观察结果表明，各种神经功能，包括痴呆，可以发生在GGT II型，反映广泛的变性超出运动神经元系统。
Globular glial tauopathies (GGTs) are four-repeat tauopathies characterized by the presence of two types of tau-positive globular glial inclusions (GGIs): globular oligodendrocytic and astrocytic inclusions (GOIs and GAIs). GGTs are classified into three different neuropathological subtypes: Types I, II and III. We report two patients with GGTs - a 76-year-old woman and a 70-year-old man - in whom the disease duration was 5 and 6 years, respectively. Both patients exhibited upper and lower motor neuron signs and involuntary movements, and the latter also had dementia with frontotemporal cerebral atrophy evident on magnetic resonance imaging. Neuropathologically, in both cases, the precentral gyrus was most severely affected, and at the gray-white matter junction there was almost complete loss of Betz cells and occurrence of GOIs and coiled bodies with numerous neuropil threads. Both patients also showed neuronal loss and GGIs (mostly GOIs) in many other central nervous system regions, including the basal ganglia. Apart from the degree of regional severity, the distribution pattern was essentially the same in both cases. However, GAIs were not conspicuous in any of the affected regions. Based on the morphology and distribution pattern of the GGIs, we diagnosed the present two patients as having GGT Type II. Electron microscopic and biochemical findings in the former were consistent with the diagnosis. Type II cases are reported to be characterized by pyramidal features reflecting predominant motor cortex and corticospinal tract degeneration. The present observations suggest that a variety of neurological features, including dementia, can occur in GGT Type II reflecting widespread degeneration beyond the motor neuron system.