Park Kyung-Il, Chung Jae-Myun, Kim Ji-Young
Department of Neurology, Inje University College of Medicine, Seoul Paik Hospital, Seoul, Korea.
J Epilepsy Res. 2014 Jun 30;4(1):24-7. doi: 10.14581/jer.14006. eCollection 2014 Jun.
Dyke-Davidoff-Masson syndrome (DDMS) has cerebral hemiatrophy and compensatory ipsilateral skull thickening, and is manifested by recurrent seizures and hemiparesis. We present one case with typical DDMS, who had a brother suffering from epilepsy with mild imaging abnormality relevant to DDMS and similar seizure semiology. A 26-year-old man had a history of developmental delay, mental retardation, hemiparesis and recurrent seizures. His brother, 23-year-old man had also experienced recurrent seizures, but he had no neurological deficits. Older brother experienced focal motor seizures with/without secondary generalization. Sometimes, he noted an auditory aura. MRI demonstrated the hemispheric atrophy with the adjacent bony hypertrophy. The seizures of younger brother were mainly of the auditory type and the MRI showed mild hemispheric atrophy with hippocampal sclerosis without any bony change. Our sibling cases might have a familial predisposition and support the idea that clinical courses and radiological findings of DDMS are varied even within one family.
戴克-戴维多夫-马森综合征（DDMS）存在大脑半球萎缩及同侧颅骨增厚代偿，表现为反复发作的癫痫和偏瘫。我们报告一例典型的DDMS病例，其兄弟患有癫痫，影像学检查有与DDMS相关的轻度异常，且癫痫发作的症状学相似。一名26岁男性有发育迟缓、智力低下、偏瘫和反复发作癫痫的病史。他23岁的兄弟也有癫痫反复发作，但无神经功能缺损。哥哥经历了伴有或不伴有继发全身性发作的局灶性运动性癫痫。有时，他会注意到听觉先兆。磁共振成像（MRI）显示半球萎缩伴相邻骨质增生。弟弟的癫痫发作主要为听觉型，MRI显示轻度半球萎缩伴海马硬化，无任何骨质改变。我们的这对兄弟病例可能存在家族易感性，支持了即使在一个家族中，DDMS的临床病程和影像学表现也存在差异的观点。
