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首页> 外文期刊>Neuropathology and applied neurobiology >Hirano body-rich subtypes of Creutzfeldt-Jakob disease
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Hirano body-rich subtypes of Creutzfeldt-Jakob disease

机译：Creutzfeldt-Jakob病的平野富人体亚型

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Background: In definite Creutzfeldt-Jakob disease (CJD), morphological and immunohistochemical patterns are useful to identify molecular subtypes. Severe cerebellar pathology and hippocampal involvement helps to identify VV subtypes. The rare VV1 variant (<1%), more frequent in young individuals, is additionally characterized by the presence of ballooned neurones in affected areas. In 1985, Cartier etal. described a family cluster of three individuals with an ataxic CJD form, showing, in addition to severe cerebellar and hippocampal involvement, the presence of frequent Hirano bodies (HB) in CA1 pyramidal neurones. HB are frequently found in aged individuals with Alzheimer pathology although they are not a specific finding. Aims and methods: In this study, we evaluated the presence of HB in hippocampi of 54 genetically and molecularly characterized CJD cases, aiming to elucidate whether additional morphological features could be helpful to point to molecular subtypes. Results: We identified nine cases (four VV1, one out of three MV2K, three out of six MV2K+2C and one MV carrying a 96-base pair insertion) with abundant, partly bizarre and clustered HB in CA1 sector, not observed in other subtypes. The presence of HB was independent of hippocampal involvement by the disease itself. Conclusions: Clusters of abundant HB might be found in rare CJD subtypes such as VV1, MV2K/MV2K+2C and some genetic cases. In addition to histopathological and PrP immunohistochemical deposition patterns, their presence might be a useful additional morphologic feature that could point to the molecular subtype, especially when genetic and/or Western blot analyses are not available.
机译：背景：在确诊的克雅氏病（CJD）中，形态学和免疫组化模式可用于鉴定分子亚型。严重的小脑病理和海马受累有助于识别VV亚型。罕见的VV1变异体（<1％），在年轻人中更常见，其特征还在于受影响区域中存在气球状神经元。 1985年，卡地亚（Cartier）等人。他描述了一个由共济失调CJD形式的三人组成的家庭集群，除了严重的小脑和海马受累外，还显示出CA1锥体神经元中频繁存在的平野体（HB）。 HB经常在老年痴呆症患者中发现，尽管不是特定发现。目的和方法：在这项研究中，我们评估了54例具有遗传和分子特征的CJD病例海马中HB的存在，旨在阐明其他形态学特征是否可能有助于指出分子亚型。结果：我们确定了9例（在CA1区域未发现大量HB），其中9例（其中MV2K中有4例，MV2K中有1例，MV2K + 2C中有3例，带有96个碱基对的MV）。亚型。 HB的存在与疾病本身的海马受累无关。结论：在罕见的CJD亚型中，例如VV1，MV2K / MV2K + 2C和一些遗传病例中，可能会发现大量的HB簇。除了组织病理学和PrP免疫组织化学沉积模式外，它们的存在可能是有用的其他形态学特征，可以指出分子亚型，尤其是在无法进行遗传和/或蛋白质印迹分析时。

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