Rajalakshmi P Prathiba, Gadodia Ankur, Priyatharshini P
Department of Radiology, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India.
Goyal MR and Diagnostic Center, New Delhi, India.
J Pediatr Neurosci. 2015 Jul-Sep;10(3):244-6. doi: 10.4103/1817-1745.165678.
Middle interhemispheric variant (MIH) of holoprosencephaly (HPE) or syntelencephaly is a rare variant of HPE characterized by abnormal midline union of the posterior frontal and parietal lobes with variable fusion of thalami. It varies from classic HPE in embryopathogenesis, severity of fusion of brain structures, associated craniofacial anomalies and clinical presentation. We report a case of MIH in a 5-year-old girl, who presented with severe developmental delay and discuss the features differentiating it from other more common forms of HPE.
前脑无裂畸形（HPE）或融合性全前脑的中间脑间变异型（MIH）是一种罕见的HPE变异型，其特征是额后叶和顶叶的中线联合异常以及丘脑不同程度的融合。它在胚胎发病机制、脑结构融合的严重程度、相关的颅面畸形和临床表现方面与典型的HPE有所不同。我们报告了一例5岁女孩的MIH病例，该女孩表现为严重发育迟缓，并讨论了将其与其他更常见的HPE形式区分开来的特征。
