Karatas Hulya, Saygi Serap
Department of Neurology, Institute of Neurological Sciences and Psychiatry, Hacettepe University, Sihhiye 06100, Ankara, Turkey.
Epilepsy Behav. 2009 Jun;15(2):245-8. doi: 10.1016/j.yebeh.2009.02.046. Epub 2009 Mar 5.
Septo-optic dysplasia (SOD) is a rare developmental anomaly first described by Reeves. It is a heterogeneous condition defined loosely by any combination of optic nerve hypoplasia, pituitary hypoplasia, and midline neuroradiological malformations. The etiology of SOD remains unclear. SOD-plus is the association of SOD and disorders of neuronal organization. Herein we describe two patients with SOD-plus syndrome characterized by SOD with epilepsy and multiple skeletal and central nervous system abnormalities. These two cases indicate that SOD results from disorders in multiple developmental stages. In addition, this is the first report of SOD-plus syndrome with mirror hand movements. Advances in neuroradiological evaluation techniques enable the detection of associated central nervous system abnormalities that can be special characteristics for the diagnosis of the spectrum of SOD-plus.
视隔发育不良(SOD)是一种罕见的发育异常,最早由里夫斯描述。它是一种异质性疾病,可宽泛地定义为视神经发育不全、垂体发育不全和中线神经放射学畸形的任意组合。SOD的病因尚不清楚。SOD-plus是指SOD与神经元组织紊乱的关联。在此,我们描述了两名患有SOD-plus综合征的患者,其特征为SOD合并癫痫以及多种骨骼和中枢神经系统异常。这两例病例表明,SOD是由多个发育阶段的紊乱所致。此外,这是首例伴有镜像手运动的SOD-plus综合征报告。神经放射学评估技术的进展使得能够检测出相关的中枢神经系统异常,这些异常可能是诊断SOD-plus谱系的特殊特征。