A LIGHT AND ELECTRON MICROSCOPY STUDY OF AN UNUSUAL WIDESPREAD NUCLEAR INCLUSION BODY DISEASE - A POSSIBLE RESIDUUM OF AN OLD HERPESVIRUS INFECTION

A LIGHT AND ELECTRON MICROSCOPY STUDY OF AN UNUSUAL WIDESPREAD NUCLEAR INCLUSION BODY DISEASE - A POSSIBLE RESIDUUM OF AN OLD HERPESVIRUS INFECTION
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DOI:
10.1007/bf00690510
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发表时间:
1968-01-01
影响因子:
12.7
通讯作者:
HAYDON, GB
HAYDON, GB
中科院分区:
医学1区
文献类型:
--
作者:
LINDENBERG, R;RUBINSTEIN, LJ;HAYDON, GB

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病理结果描述在一个弱智的28岁男子进行性痉挛和共济失调,因为儿童。它们包括在整个大脑和内脏器官中广泛存在的嗜酸性核内包涵体,以及更大、染色更深的球状体。纹状体、内囊、苍白球和丘脑受累最严重。球状体似乎来自于核内包涵体的增大:两者都给出了蛋白质的染色和组织化学反应。电镜下见核内包涵体和球状体均由均匀的细颗粒物质组成。在一个这样的团块中,在内囊中,可以看到病毒样颗粒,它们随机分布并以晶体阵列排列。这些颗粒类似于疱疹病毒组的颗粒,并讨论了这些改变可能是早期、出生时或子宫内发生的旧的全身性疱疹病毒感染的残留物。假设的原发性免疫缺陷,或病毒感染之前发生的收购免疫能力,是先进的,以占独特的形态学特征。另一种建议的原发性代谢紊乱影响核蛋白被简要考虑。
The pathologic findings are described in a mentally-retarded 28-year old man with progressive spasticity and ataxia since childhood. They consisted in the widespread presence, throughout the brain and visceral organs, of eosinophilic intranuclear inclusions, with the association of larger, more intensely staining globular bodies. The corpora striata, internal capsule, pallida and thalami were the most severely affected. The globular bodies seemed to be derived from the enlargement of the intranuclear inclusions: both gave the tinctorial and histochemical reactions for protein. There was no evidence of inflammation.Electron microscopy showed that the intranuclear inclusions and the globular bodies were composed of homogeneous finely granular material. In one such mass, in the internal capsule, virus-like particles, both randomly distributed and arranged in crystalline arrays, were seen. The particles resembled those of the herpesvirus group.The possibility is discussed that these alterations may present the residuum of an old generalized herpesvirus infection which could have been incurred in early life, at birth, or in utero. The hypothesis of a primary immunologic deficiency, or of a viral infection having occurred before the acquisition of immunologic competence, is advanced in order to account for the unique morphologic features. The alternative suggestion of a primary metabolic disorder affecting nuclear protein is briefly considered.