MRI Appearance of Sturge-Weber Syndrome in Tuberous Sclerosis Complex: Is the Neural Crest the Culprit?

被引：6

作者：

Curatolo, Paolo ^{[1
]}

机构：

[1] Univ Roma Tor Vergata, Dept Neurosci, Pediat Neurol Unit, Rome, Italy

来源：

JOURNAL OF CHILD NEUROLOGY | 2009年 / 24卷 / 03期

关键词：

tuberous sclerosis complex; Sturge-Weber syndrome; neurocutaneous syndromes;

D O I：

10.1177/0883073808324220

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Several neurocutaneous syndromes have been reported in association with tuberous sclerosis complex. The coexistence of intracranial features of Sturge-Weber syndrome in patients with a confirmed diagnosis of tuberous sclerosis complex has been recently described. Several hypotheses can he raised to explain this association. The coexistence of signs of both diseases in the same patient could be explained by abnormal neural crest development, and by mutually enhanced common altered pathways. Mutation characterization of tuberous sclerosis complex and a better definition of the clinical and neuroimaging phenotypes could offer a crucial contribution to the etiopathogenetic mechanisms of these disorders.

引用

页码：263 / 266

页数：4

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