Goldsmith I L, Zupanc M L, Buchhalter J R
Departments of Neurology, Mayo Clinic, Rochester, Minnesota 55901, USA.
Epilepsia. 2000 Apr;41(4):395-9. doi: 10.1111/j.1528-1157.2000.tb00179.x.
To determine if using more stringent criteria for cryptogenic Lennox-Gastaut syndrome (LGS) would result in an improved prognosis for that group. Cryptogenic, symptomatic, and non-cryptogenic LGS patients without etiology (indeterminate) were compared with respect to seizure and cognitive outcome.
Retrospective chart review was performed on 245 patients seen at the Mayo Clinic Rochester from 1976 to 1997, with a diagnosis of either LGS or slow spike wave on EEG. LGS was confirmed in 107 (64 male, 43 female) patients. This group was divided into cryptogenic, symptomatic, and indeterminate groups containing 23, 47, and 37 patients, respectively. In this study, cryptogenic patients all had normal development before onset of LGS, absence of dysmorphic features, normal neurologic examination, and normal magnetic resonance (MRI) brain imaging. Of the 107 patients, 74 had >/=3 years of follow-up.
LGS onset in the 107 patients occurred at a median age of 4.0 years (range, 0.6-28.9 years). When last seen, 63% of those with symptomatic LGS had more than three seizures a day compared with 50% of cryptogenic and 34% of indeterminate patients. The most common seizure types were tonic (77%), atypical absence (61%), and generalized tonic-clonic (56%). Only three patients, all part of the indeterminate group, were seizure free at last follow-up.
Using stringent criteria in defining the cryptogenic subgroup resulted in no significant difference in seizure outcome. Individuals with a normal cognitive outcome did not segregate into one etiologic subgroup, but did have LGS onset at an older age.
确定对隐源性 Lennox-Gastaut 综合征（LGS）采用更严格的标准是否会改善该组患者的预后。比较了隐源性、症状性和无病因（不确定）的非隐源性 LGS 患者的癫痫发作和认知结局。
对 1976 年至 1997 年在罗切斯特梅奥诊所就诊的 245 例诊断为 LGS 或脑电图显示慢棘波的患者进行回顾性病历审查。107 例（64 例男性，43 例女性）患者确诊为 LGS。该组分为隐源性、症状性和不确定组，分别包含 23 例、47 例和 37 例患者。在本研究中，隐源性患者在 LGS 发作前均发育正常，无畸形特征，神经系统检查正常，脑部磁共振成像（MRI）正常。107 例患者中，74 例有≥3 年的随访。
107 例患者 LGS 发作的中位年龄为 4.0 岁（范围 0.6 - 28.9 岁）。最后一次就诊时，症状性 LGS 患者中 63%每天发作超过 3 次，而隐源性患者为 50%，不确定患者为 34%。最常见的癫痫发作类型为强直发作（77%）、非典型失神发作（61%）和全身强直阵挛发作（56%）。最后一次随访时只有 3 例患者无癫痫发作，均为不确定组。
采用严格标准定义隐源性亚组在癫痫发作结局方面无显著差异。认知结局正常的个体并非集中在一个病因亚组，但 LGS 发作年龄较大。
