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临床症状类似多发性硬化症且具有不寻常病理和超微结构特征的系统性红斑狼疮。

Systemic lupus erythematosus clinically resembling multiple sclerosis and with unusual pathological and ultrastructural features.

作者信息

Allen I V, Millar J H, Kirk J, Shillington R K

出版信息

J Neurol Neurosurg Psychiatry. 1979 May;42(5):392-401. doi: 10.1136/jnnp.42.5.392.

Abstract

A case of systemic lupus erythematosus is described which clinically resembled multiple sclerosis and in which the lesions were restricted to the central nervous system. The necropsy findings of vascular thickening and necrosis in the spinal cord and in a posterior nerve root explain the main clinical abnormalities. Clinical signs of the terminal peritonitis secondary to cholecystitis were absent or minimised probably because of the steroid therapy and spinal cord necrosis. Primary demyelination was not demonstrated though electronmicroscopy revealed lattice fibrillar inclusions within a few myelin sheaths. An unusual ultrastructural feature was the finding of "rod-shaped tubular bodies" in large numbers in the endothelial cells of cerebral blood vessels. The incidence and morphology of these organelles are compared with those of the intracisternal tubuloreticular structures (TRS) commonly found in systemic lupus erythematosus.

摘要

本文描述了一例系统性红斑狼疮病例,该病例临床症状类似多发性硬化症,且病变仅限于中枢神经系统。脊髓和后神经根血管增厚及坏死的尸检结果解释了主要的临床异常情况。继发于胆囊炎的终末期腹膜炎的临床体征不存在或很轻微,这可能是由于类固醇治疗和脊髓坏死所致。尽管电子显微镜检查显示少数髓鞘内有晶格状纤维状包涵体,但未发现原发性脱髓鞘病变。一个不寻常的超微结构特征是在脑血管内皮细胞中大量发现“杆状管状小体”。将这些细胞器的发生率和形态与系统性红斑狼疮中常见的池内管状网状结构(TRS)的发生率和形态进行了比较。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/80cd/490225/1a09d6ecac96/jnnpsyc00085-0006-a.jpg

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