Myoclonic epilepsy in Down syndrome and Alzheimer disease
Myoclonic epilepsy in Down syndrome and Alzheimer disease
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DOI：
10.1016/j.nrl.2014.12.008
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发表时间：
2017-03-01
期刊：
影响因子：
3.9
通讯作者：
Alvarez-Sabina, J.
中科院分区：
文献类型：
--
作者：
Aller-Alvarez, J. S.;Menendez-Gonzalez, M.;Alvarez-Sabina, J.
简介：患有唐氏综合征（DS）的阿尔茨海默病（AD）患者与年龄相关。这两种具有共同神经病理基础的疾病都与迟发性肌阵挛性癫痫（LOMEDS）有关。这个实体提出了脑电图功能的广义polyspike-wave dischars.Method：我们提出了一系列的11例诊断为DS或AD谁开发肌阵挛性癫痫发作或全身强直阵挛性癫痫发作。在所有情况下，临床和神经影像学研究和多导脑电图monitoring.Results：在所有情况下，认知功能障碍进展迅速癫痫发作后造成的依赖程度增加。EEG中最常见的发现是大脑活动减慢，θ和δ节律，加上8名患者出现临界间广义多棘波。在神经影像学检查中发现大脑皮质萎缩。在这一系列中最有效的药物是左乙拉西坦。结论：泛发性癫痫与老年DS的关联代表了一种附带现象的演变，这是与认知和运动功能的逐步恶化。这种癫痫具有一些电临床特征，表现为进行性肌阵挛性癫痫，这可能与表征DS与AD进化相似性的结构变化有关。认识到这种综合征是重要的，因为它具有预后意义，需要适当的治疗。(C)2014年西班牙神经病学学会。出版社：Elsevier Espana，S.L.U
Introduction: Patients with Down syndrome (DS) who exhibit Alzheimer disease (AD) are associated with age. Both diseases with a common neuropathological basis have been associated with late-onset myoclonic epilepsy (LOMEDS). This entity presents electroencephalogram features as generalized polyspike-wave discharges.Method: We present a series of 11 patients with the diagnosis of DS or AD who developed myoclonic seizures or generalized tonic-clonic seizures. In all cases, clinical and neuroimaging studies and polygraph EEG monitoring was performed.Results: In all cases, cognitive impairment progressed quickly after the onset of epilepsy causing an increase in the degree of dependence. The most common finding in the EEG was a slowing of brain activity with theta and delta rhythms, plus intercritical generalized polyspike-waves were objectified in eight patients. In neuroimaging studies was found cerebral cortical atrophy. The most effective drug in this series was the levetiracetam.Conclusions: The association of generalized epilepsy with elderly DS represents an epiphenomenon in evolution which is associated with a progressive deterioration of cognitive and motor functions. This epilepsy has some electroclinical characteristics and behaves as progressive myoclonic epilepsy, which is probably related to the structural changes that characterize the evolutionary similarity of DS with AD. Recognition of this syndrome is important, since it has prognostic implications and requires proper treatment. (C) 2014 Sociedad Espanola de Neurologia. Published by Elsevier Espana, S.L.U