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

临床表现类似多发性硬化症,但具有异常的病理和超微结构特征的系统性红斑狼疮

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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.

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