苏丹青少年肌阵挛性癫痫的临床表现和延迟诊断的因素

S. Abdalla, M. Elmagzoub, H. Babikir
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引用次数: 1

摘要

青少年肌阵挛性癫痫(JME)是一种常见的特发性癫痫,表现为肌阵挛性抽搐,通常在儿童早期没有意识障碍,然后全身性强直-阵挛性发作,缺席发作并不罕见。支持诊断的JME综合征的突出和主要EEG特征是正常脑背景活动下的广义3.5-6 Hz单、双峰和多峰慢波复合体。目的:目的是评估人口统计学特征,发现苏丹JME患者中最常见的临床表现,并解释诊断延迟的可能原因。方法和患者:该研究包括所有在国立Ribat大学医学院和El-Magzoub神经科学中心就诊的患者,他们的脑电图显示JME的特征(回顾性)。所有患者均为前瞻性招募,进行脑电图复核,并通过预先填写的访谈表进行临床病史和检查。所得数据已使用SPSS进行分析,结果以表格形式说明。结果与讨论:JME患者诊断时的平均年龄为19.55±8.98岁。约91%的患者确认有肌阵挛性抽搐。61.4%的患者睡眠不足是诱发MJs的因素。77.27%的JME患者出现失神发作,84.1%的患者出现全身性强直-阵挛性发作,平均发病年龄13.92±5.65岁。抽搐与GTC的间隔时间为4.35±3.61年。结论:睡眠不足是苏丹JME患者的常见症状,睡眠不足是最常见的加重因素。全身性强直-阵挛在JME患者中很常见,这一重大事件可能会使MJs模糊不清,并使医生在询问时感到困惑。
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Clinical Presentations and Factors Responsiblefor Delays in Diagnosis of Juvenile MyoclonicEpilepsy among Sudanese Patients
Introduction: Juvenile Myoclonic Epilepsy (JME) is common idiopathic epilepsy manifested by myoclonic jerks that commonly noticed in early childhood without consciousness disturbance, then the generalised tonic – clonic overwhelms the scene, absence attacks are not uncommon. The prominent and cardinal EEG features of JME syndrome that supports the diagnosis is the generalized 3.5-6 Hz single, bifid and polyspikes slow-wave’s complexes on normal brain background activity. Aim: Aims were to evaluate the demographic features and to detect the most common clinical presentations among Sudanese patients with JME and explain the possible causes of diagnosis delaying. Methods and patients: The study included all patients attended to National Ribat University – Faculty of Medicine and El-Magzoub Neurosciences centres whom their EEGs showed the characteristics features of JME (retrospectively). All the patients had been recruited (prospectively) and their EEGs have been repeated and clinical history and examination through pre-formed interview forms had bee done. The obtained data have been analysed using the SPSS and results were illustrated in by tables. Results and discussion: The mean age of JME patients at diagnosis was 19.55 ± 8.98 years. Myoclonic jerks were confirmed in about 91% of the patients. Sleep deprivation was the triggering factor for MJs in 61.4% of the patients. Absence attacks were confirmed in 77.27% of JME patients, and generalised tonic-clonic seizures in 84.1% of patients with a mean age of onset 13.92 ± 5.65 years. The elapsed time between jerks and GTC was 4.35 ± 3.61 years. Conclusion: MJs is common among Sudanese patients with JME and the sleep deprivation is the most frequent aggravating factors with the early awakening. Generalised tonic-clonic is common among JME patients and this great event may obscure MJs and confused the doctors to ask about them.
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