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肾上腺皮质机能低下(原发或继发 Addison氏病)可引起或并发某些中枢及周围神经病变,临床上见到有下述一些类型的神经损害。肾上腺脑白质曹养不良症(adrenoleukodystrophy)。本病是原发性肾上腺皮质机能低下,合并弥散性脑硬化,后者称为 Schilder 病,故本病又称为 Addison-Schilder 病,最初是由 Siemerling(1923)描述。初曾认为是两者偶合,现已确认是一个性连锁隐性遗传病,通过不发病的女性遗传,绝大多数发生于男性。晚近超微结构检查,发现病变的脑组织吞噬细胞及肾上腺皮质细胞中,有特殊的线样胞浆内包涵体。这是本病组织病理学特征。这种包涵体也同样见于周围神经雪旺氏细胞,睾丸间质细胞中。Schaumburg 认为本病是脂类贮存障碍,导致
Adrenal cortex dysfunction (primary or secondary Addison’s disease) can cause or concurrent with certain central and peripheral neuropathy, clinically see some of the types of nerve damage. Adrenoleukodystrophy adrenoleukodystrophy. The disease is a primary adrenocortical hypoplasia, with diffuse sclerosis, the latter known as Schilder disease, so the disease, also known as Addison-Schilder disease, was originally described by Siemerling (1923). Originally thought to be a combination of the two, has now been identified as a sex-linked recessive inherited disease, which is inherited by women without disease, most of which occur in males. Recent ultrastructural examination found that lesions of brain tissue phagocytes and adrenal cortical cells, there are special line-like cytoplasmic inclusion bodies. This is the histopathological features of the disease. This inclusion body is also found in peripheral Schwann cells, testicular stromal cells. Schaumburg believes the disease is caused by lipid storage disorders