Spectrum of epilepsy in Wilson's disease with electroencephalographic, MR imaging and pathological correlates
Spectrum of epilepsy in Wilson's disease with electroencephalographic, MR imaging and pathological correlates
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DOI：
10.1016/j.jns.2010.01.007
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发表时间：
2010-04-15
影响因子：
4.4
通讯作者：
Shankar, S. K.
中科院分区：
文献类型：
--
作者：
Prashanth, L. K.;Sinha, S.;Shankar, S. K.
背景资料：目的：分析WD患者的癫痫发作特征，并与EEG和MRI检查结果进行相关性分析。结果：癫痫发作的年龄为12.8 ± 5.7岁。癫痫发作-在WD特征性特征发作之前（19.5%）;同时发生（46.3%）;或在去铜治疗后（29.2%）和作为终末事件发生（4.8%）。癫痫发作的类型有：全身强直阵挛-29，简单部分性-8，复杂部分性-6，部分性发作伴继发全身性-2和周期性肌阵挛-1。6例患者有多种癫痫发作类型，4例有癫痫持续状态。EEG异常常见（19/24），包括背景减慢和癫痫样放电。MRI（n= 20）显示不同程度的萎缩和信号改变，涉及基底节，脑干和白色物质。随访时间为8.1 +/- 9.2年。癫痫发作结局：无复发-68.3%，突破性发作-17.1%，控制不良-9.7%，未随访-4.9%。其中两人在集束炸弹袭击后死亡。尸检发现在额叶、颞叶和顶叶区域的白色物质中存在空洞性病变，伴有不同程度的皮质带受累。有癫痫发作的患者较无癫痫发作的患者更常出现白色改变。还注意到，癫痫发作不受控制的患者有MRI提示空洞的白色的问题，虽然反过来是不正确的。结论：这是最大的系列癫痫在WD。癫痫发作并不罕见，可能发生在任何阶段。白色物质束从皮层传入神经可能有助于WD的癫痫发作。(C)2010 Elsevier B. V.保留所有权利。
Background: Seizures are uncommon in Wilson's disease.Objective: To analyze profile of seizures in WD and to correlate with EEG and MR imaging observations.Subjects and methods: 41/490 patients (8.3%) of WD were documented to have seizures. Autopsy observations were available in 3 cases.Results: The age at onset of seizures was 12.8 +/- 5.7 years. Seizure - preceded the onset of characteristic features of WD (19.5%); occurred concurrently (46.3%); or, followed de-coppering therapy (29.2%) and occurred as terminal event (4.8%). The types of seizures were: generalized tonic-clonic -29, simple partial -8, complex partial -6, partial seizures with secondary generalization -2 and periodic myoclonus -1. Six patients had multiple seizure types and 4 had status epilepticus. EEG abnormalities were frequent (19/24) consisting of background slowing and epileptiform discharges. MRI (n= 20) revealed varying degree of atrophy and signal changes involving basal ganglia, brainstem and white matter. The duration of follow-up was 8.1 +/- 9.2 years. The outcome of seizure was: no recurrence -68.3%, breakthrough seizures -17.1%, poor control -9.7% and no follow-up -4.9%. Two of them succumbed following cluster attacks. Autopsy revealed cavitatory lesions in white matter in frontal, temporal and parietal areas with varying involvement of cortical ribbon. Patients with seizures had more often white matter changes than those without. It was also noted that patients whose seizures were not controlled had MRI suggestive of cavitation of white matter, though the reverse was not true.Conclusions: This is the largest series regarding epilepsy in WD. Seizures are not uncommon and could occur at any stage. Deafferentation of white matter tracts from cortex may contribute for seizure in WD. (C) 2010 Elsevier B.V. All rights reserved.