ALEXANDERS DISEASE - A DISEASE OF ASTROCYTES
ALEXANDERS DISEASE - A DISEASE OF ASTROCYTES
复制标题
DOI：
10.1093/brain/108.2.367
复制
发表时间：
1985-01-01
期刊：
影响因子：
14.5
通讯作者：
BECKER, LE
中科院分区：
文献类型：
--
作者：
BORRETT, D;BECKER, LE
亚历山大氏病是一种罕见且知之甚少的进行性神经功能恶化的原因。尽管临床病史取决于所涉及的年龄组，但病变的组织学特征和分布往往是一致的。病理学特征有力地证明亚历山大病代表星形胶质细胞的非肿瘤性疾病。许多问题仍未得到解答，包括导致婴儿组疾病严重程度的因素以及继发星形细胞功能障碍的脱髓鞘机制。这一系列的 6 名亚历山大病患者代表了广泛的年龄范围，使得病理和临床异常与星形细胞功能障碍的假设机制有关。
Alexander's disease is a rare and poorly understood cause of progressive neurological deterioration. Although the clinical history depends on the age group involved, the histological character and distribution of lesions tend to be uniform. The pathological features argue strongly that Alexander's disease represents a nonneoplastic disease of astrocytes. Numerous questions remain unanswered, including the factors responsible for the severity of the illness in the infantile group and the mechanism of demyelination secondary to astrocytic dysfunction. This series of 6 patients with Alexander's disease represents a wide spectrum of ages, enabling the pathological and clinical abnormalities to be related to a postulated mechanism of astrocytic dysfunction.