Abstract

Schizencephaly is one of the rare cortical malformations that classify in the group of neurological migration defects. The appearance of the anomaly is in the form of a cleft that passes through the brain parenchyma. In mild types, the cleft does not reach the lateral ventricle. Demonstrating the presence of polymicrogyria, optimal evaluation of the cortex and determining the cleft trace are essential in the radiological evaluation of schizencephaly. Because differential diagnosis should be made with heterotopias and focal cortical dysplasia. We present a case of a 21-yearold female patient with closed-lip schizencephaly accompanied by MRI findings. Polymicrogyria is best detected in sagittal T2 sequences, while the difference of white matter and grey matter are well observed in T1 and FLAIR sequences. The benefit of diffusion-weighted images in schizencephaly is unclear. The ADC values of the grey matter adjacent to the cleft are close to the lower limit of normal. Susceptibility weighted image (SWI) sequence has no place in the evaluation of schizencephaly. It can be beneficial for the exclusion of vascular malformations, haemorrhage and differential diagnosis.

Highlights

  • Is a rare cortical malformation in the form of a cleft that seen throughout the brain parenchyma [1]

  • The type extending to the lateral ventricle is called open-lip, while the type ending in the white matter is called closed-lip. 3⁄4 of the cases are open-lip, and 1⁄4 are closed-lip

  • Is a congenital brain malformation classified among neuronal migration defects together with lissencephaly, heterotopia and polymicrogyria

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Summary

INTRODUCTION

Is a rare cortical malformation in the form of a cleft that seen throughout the brain parenchyma [1]. The prevalence of closed-lip Schizencephaly is 1/400,000 in the normal population It is almost always sporadic, few familial cases have been described. Apart from T1W, T2W turbo spin-echo and FLAIR sequences, Diffusion-ADC, contrast and SWI (magnitude, filtered phase, SWI (combined post-processed magnitude and phase), SWI minimum intensity projection (MIP) views of schizencephaly were demonstrated. Our paper aims to demonstrate the radiological evaluation process and the use of sequences in closed-lip schizencephaly. In the T1-weighted sequence, the cortex band around the cleft was clearly visualized. The band of grey matter was thinner at the end of the cleft (Figure 1A). In contrast-enhanced images, emissary veins along the cleft were observed (Figure 1D). ADC values were at the lower limit of the normal grey matter scale (Figure 3).

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