Maria Camila Arango-White1, Diego Herrera2, and Sergio Alberto Vargas3
1radiology, Universidad de Antioquia, Medellin, Colombia, 2Radiology, CEDIMED, medellin, Colombia, 3Radiology, CEDIMED, Medellin, Colombia
Synopsis
Cortical development malformations are relatively uncommon conditions but have an enormous impact in children with neuro-developmental delay, disability and epilepsy. We aim to present cases from our clinical practice to illustrate the pathology spectrum and review the existing literature about these malformations. We emphasize the importance of an MRI approach to elaborate a differential diagnosis which can be useful to focus genetic tests, and to establish prognosis in these patients.
Purpose
The purpose of this educational E-Poster is to pictorially review the newest classification of malformations of cortical development with an special emphasis on MRI evaluation
Outline of content
Cortical malformations are a leading cause of intractable epilepsy and developmental delay in children who previously were thought to have idiopathic neurological conditions. Neuroimaging techniques have become more sensitive and precise on detecting these brain abnormalities; for example brain MRI is a valuable tool not only for diagnosis, but also for planning in epilepsy surgery. In 2012, Barkovich et al presented the latest update in the classification adding molecular and genetic data. In this Educational E-Poster, we review the imaging findings covering the wide spectrum of malformations of cortical development according to the latest classification including: malformations secondary to abnormal neuronal and glial proliferation or apoptosis such as severe congenital microcephaly, megalenchephaly, and cortical dysgenesis with abnormal cell proliferation; malformations due to abnormal neuronal migration including periventricular heterotopia and other malformations due to abnormal migration; and malformations due to abnormal postmigrational development including polymicrogyria (PMG), cortical dysgenesis secondary to inborn errors of metabolism, and cortical dysplasias. MRI is the most sensitive and specific imaging technique to detect and classify cortical malformations based on its high tissular and spatial resolution. When evaluating cortical gray matter, volumetric T1-weighted images and T2-weighted thin slices allow to establish the diagnosis in most cases as illustrated in our E-Poster pictorial. In selected cases, we perform additional post-processing techniques including 3D volume rendering and curvilinear reconstructions to better define the extension of focal lesions in potential candidates to epilepsy surgery. Additionally, we discuss the utility of an MRI approach to elaborate a differential diagnosis of these entities which can be useful to focus genetic tests, and to establish prognosis.Summary
Accurate knowledge of the classification of malformations of cortical development is essential for a proper diagnosis and prognosis of these conditions. MRI techniques allow a precise diagnosis of these entities which can be present in patients with epilepsy and children with developmental delay.Acknowledgements
Dr. Diego Herrera
Dr. Sergio A. Vargas
References
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