Aicardi J
Department of Pediatrics, Hopital des Enfants-Malades, Paris, France.
Brain Dev. 1991;13(1):1-8. doi: 10.1016/s0387-7604(12)80289-0.
Agyria-pachygyria is a relatively common brain malformation in humans as shown by the recent experience with CT scan and MR imaging. However, the specificity of imaging is not completely established and it is not known whether all cases with a thick cortical ribbon and a smooth surface correspond histologically to actual agyria-pachygyria or may belong to different pathological anomalies such as polymicrogyria. The extent, distribution and detailed structure of agyria-pachygyria vary widely and the clinical features are accordingly very diverse. Rather than one entity, pachygyric cortical abnormalities form a broad spectrum that raises different clinical, nosological and genetic problems. Therefore, a precise analysis of each case is necessary to establish the prognosis and risk of recurrence.
无脑回-巨脑回畸形在人类中是一种相对常见的脑畸形，最近的CT扫描和磁共振成像经验表明了这一点。然而，成像的特异性尚未完全确立，尚不清楚所有具有厚皮质带和平滑表面的病例在组织学上是否对应于实际的无脑回-巨脑回畸形，或者是否可能属于不同的病理异常，如多小脑回畸形。无脑回-巨脑回畸形的范围、分布和详细结构差异很大，临床特征也因此非常多样。厚脑回皮质异常并非单一实体，而是形成了一个广泛的谱系，引发了不同的临床、疾病分类学和遗传学问题。因此，对每个病例进行精确分析对于确定预后和复发风险是必要的。
