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线状皮脂腺痣综合征的计算机断层扫描表现。

Computed tomography appearances in the linear sebaceous naevus syndrome.

作者信息

Levin S, Robinson R O, Aicardi J, Hoare R D

出版信息

Neuroradiology. 1984;26(6):469-72. doi: 10.1007/BF00342683.

Abstract

The appearance of C.T. of the head in 11 cases of the linear sebaceous naevus syndrome are presented. From these it is argued that the pathology represents a disorder of neuronal migration and organisation rather than differentiation. Since three of the cases had evidence of CNS tumour in addition to two in the literature, it would appear that a further aspect of this disorder is a failure of growth constraint. This is paralleled by a similar tendency in the skin lesion which should be considered premalignant. Normal intelligence is rare in this condition and co-exists only with a normal C.T. scan. The early onset of fits is not necessarily associated with subsequent mental handicap.

摘要

本文报告了11例线性皮脂腺痣综合征患者的头部CT表现。由此认为,其病理表现为神经元迁移和组织紊乱,而非分化异常。由于其中3例除文献报道的2例之外还有中枢神经系统肿瘤的证据,看来这种疾病的另一个方面是生长抑制失败。皮肤病变也有类似倾向,应视为癌前病变。在这种情况下,智力正常很少见,仅在CT扫描正常时才会出现。癫痫发作早发不一定与随后的智力障碍相关。

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