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1880年,法国医生 D.M.Bourneville 报道了一例从3岁起经常出现癫痫发作,并且在颜面、鼻、额部、面颊出现斑疹的女孩,这位女孩在15岁时死亡。尸检发现该病人的脑回区有白色的硬化区域和肿瘤样结节,在肾脏表面有多发性“山丘样”隆起,Bour-neville 将肾脏和大脑的病变联系到了一起,认为是同一种疾病在不同部位的表现,并将其称之为“脑回结节性硬化”(tuberous sclerosis of the cerebral convolu-tions),这是人类最早对该病的病理学特征进行详尽的记录。并将该病命名为 Bourneville 病。目前人们对结节性硬化症的主要临床症状特征及病理改变已有了较全面深入的认识,其最特征性的临床表现为面部血管纤维瘤、癫痫发作和智能障碍,病理改变
In 1880, the French doctor D.M. Bourneville reported on a girl who had had a seizure since the age of 3 and had herpes on the face, nose, forehead and cheeks, and died at the age of 15. An autopsy revealed a white sclerosis and tumor-like nodules in the back of the patient with multiple “hill-like” ridges on the surface of the kidney. Bour-neville linked the kidney and brain lesions to the same The manifestation of the disease at different sites is called “tuberous sclerosis of the cerebral convolu- tions,” which is the earliest record of the pathology of the disease in humans . And the disease is named Bourneville disease. At present, people have a more comprehensive and in-depth understanding of the main clinical symptoms and pathological changes of tuberous sclerosis. The most characteristic clinical manifestations are facial fibroses, seizures and mental disorders, pathological changes