Shrestha Balkrishna
West China Hospital, West China School of Medicine, Sichuan University, Department of Neurosurgery, Chengdu, China.
Turk Neurosurg. 2013;23(1):117-21. doi: 10.5137/1019-5149.JTN.4283-11.1.
A rare syndrome, Dyke-Davidoff-Masson Syndrome (DDMS), with a diagnostic conundrum, and the way it was solved is presented. A 13-year-old boy presented with recurrent seizures for the past 10 years. He had been treated with anticonvulsant medication which was satisfactory at first but later the seizures recurred. Recently, the frequency of the seizures increased with preictal dizziness and postictal drowsiness. Physical examination revealed mild left hemiparesis and left deviated gait irregularity. He was mentally alert but had not achieved all the developmental milestones as compared to normal child of his age. CT and MRI scan of the head showed hemiatrophic cerebral parenchyma with prominent sulci and encephalomalacia. 24-hour intensive video EEG monitoring revealed suppression of alpha rhythm and local slow wave activity on the side of the atrophic hemisphere. PET-CT showed highly functional left cerebral hemisphere and less functional right cerebral hemisphere. The patient underwent functional hemispherectomy under neurophysiological monitoring and the nonfunctional brain tissues were resected while selectively preserving the functional areas detected by fMRI and PET-CT scan. During follow up, the patient was seizure free as well as without difficulties in performing his daily activities and communications. Functional hemispherectomy for DDMS patient has a good prognosis.
本文介绍了一种罕见的综合征——戴克-戴维多夫-马森综合征（DDMS），及其诊断难题和解决方法。一名13岁男孩在过去10年中反复出现癫痫发作。他曾接受抗惊厥药物治疗，起初效果良好，但后来癫痫复发。最近，癫痫发作频率增加，伴有发作前头晕和发作后嗜睡。体格检查发现轻度左侧偏瘫和左侧步态偏斜不规则。他精神警觉，但与同龄正常儿童相比，尚未达到所有发育里程碑。头部CT和MRI扫描显示脑实质半萎缩，脑沟增宽，脑软化。24小时强化视频脑电图监测显示萎缩半球一侧的α节律抑制和局部慢波活动。PET-CT显示左侧大脑半球功能高度活跃，右侧大脑半球功能较低。该患者在神经生理监测下接受了功能性半球切除术，切除了无功能的脑组织，同时选择性保留了fMRI和PET-CT扫描检测到的功能区。在随访期间，患者癫痫未发作，日常活动和交流也没有困难。DDMS患者的功能性半球切除术预后良好。
