Piña-Garza Jesus Eric, Chung Steve, Montouris Georgia D, Radtke Rodney A, Resnick Trevor, Wechsler Robert T
The Children's Hospital at TriStar Centennial Medical Center, 330 23rd Ave North, Suite 450, Nashville, TN 37203, USA.
Banner University Medical Center, 1012 E Willetta Street, Suite 54, Phoenix, AZ 85006, USA.
Epilepsy Behav Case Rep. 2016 Feb 11;5:38-43. doi: 10.1016/j.ebcr.2016.01.004. eCollection 2016.
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.
伦诺克斯 - 加斯东综合征（LGS）的表现和进展具有多样性，这使得其难以识别，在成人中尤为如此。为了改善诊断，我们对成年后被诊断出患有该综合征和/或诊断后随访数年的患者进行了一项回顾性病历审查。我们呈现了5个病例，这些病例说明了LGS特征随时间的变化。病例1和病例2在8岁时被诊断出患有难治性癫痫、发育迟缓以及伴有1.5 - 2赫兹慢棘波放电的异常脑电图。然而，两名患者的癫痫发作类型和频率都随时间发生了变化，慢棘波放电的发生率也有所下降。病例3、病例4和病例5根据当前及既往的特征和症状在成年后被诊断为患有LGS，这些特征和症状包括难治性癫痫、认知和运动障碍以及异常脑电图结果。虽然记录并不完整，但它们表明可能之前漏诊了LGS或者其诊断已被历史遗忘。这些病例表明，需要全面且持续地评估患者脑病的各个方面，同时要牢记LGS特征可能随时间发生变化的可能性。
