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Challenges in identifying Lennox–Gastaut syndrome in adults: A case series illustrating its changing nature

机译:鉴定成人Lennox–Gastaut综合征的挑战:说明其性质改变的病例系列

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摘要

The variable presentation and progression of Lennox–Gastaut syndrome (LGS) can make it difficult to recognize, particularly in adults. To improve diagnosis, a retrospective chart review was conducted on patients who were diagnosed as adults and/or were followed for several years after diagnosis. We present 5 cases that illustrate changes in LGS features over time. Cases 1 and 2 were diagnosed by age 8 with intractable seizures, developmental delay, and abnormal EEGs with 1.5–2 Hz SSW discharges. However, seizure type and frequency changed over time for both patients, and the incidence of SSW discharges decreased. Cases 3, 4, and 5 were diagnosed with LGS as adults based on current and past features and symptoms, including treatment-resistant seizures, cognitive and motor impairment, and abnormal EEG findings. While incomplete, their records indicate that an earlier LGS diagnosis may have been missed or lost to history. These cases demonstrate the need to thoroughly and continuously evaluate all aspects of a patient's encephalopathy, bearing in mind the potential for LGS features to change over time.
机译:Lennox–Gastaut综合征(LGS)的表现和病情变化会使其难以识别,尤其是在成年人中。为了改善诊断,对诊断为成人和/或在诊断后随访了几年的患者进行了回顾性图表审查。我们提供5个案例,说明LGS功能随时间的变化。病例1和2在8岁时被诊断为顽固性癫痫发作,发育迟缓和SEG放电1.5–2 Hz的异常脑电图。但是,两名患者的癫痫发作类型和发作频率均随时间变化,SSW放电的发生率降低。根据目前和过去的特征和症状,包括难治性癫痫发作,认知和运动障碍以及脑电图异常发现,将病例3、4和5诊断为成人LGS。尽管不完整,但他们的记录表明,早期LGS诊断可能已遗漏或遗忘了。这些案例表明,有必要彻底并持续地评估患者脑病的各个方面,同时要记住LGS功能可能随着时间而改变。

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