de Menezes Márcio A Sotero, Rho Jong M
Department of Neurology, University of Washington School of Medicine, and the Children's Hospital & Regional Medical Center, Seattle 98105, USA.
Epilepsia. 2002 Jun;43(6):623-30.
Few reports detailing the electroclinical features of epileptic spasms persisting beyond infancy have been published. We sought to characterize this unique population further.
We retrospectively reviewed the clinical and video-EEG data on 26 patients (4-17 years; mean, 93 months) with a confirmed diagnosis of epileptic spasms and who were evaluated at our tertiary referral center between 1993 and 2000.
In half of our cases, epileptic spasms were associated with disorders of neuronal migration, severe perinatal asphyxia, and genetic anomalies. Interictal EEGs showed generalized slowing in the majority of patients, and a slow-wave transient followed by an attenuation of the background amplitude was the most common ictal EEG pattern associated with an epileptic spasm (19 cases). Other seizure types (number of cases in parentheses) included tonic seizures with or without a preceding spasm (13), partial seizures (11), myoclonic seizures (11), generalized tonic-clonic seizures (six), atypical absence seizures (two), and atonic seizures (one). Cases with a more organized EEG background (especially with frequencies > or =7 Hz) were more likely to have better cognition. Continued disorganization of the EEG background and persistence of hypsarrhythmia were associated with poor developmental outcome.
Patients with epileptic spasms persisting beyond age 2 years constitute a truly refractory population, one that should be better recognized by clinicians. Interestingly, although many therapies resulted in a >50% reduction in seizures, neither neurocognitive function nor quality of life was substantially improved with intervention. The interictal EEG background is the most helpful in predicting neurologic outcome.
关于婴儿期后仍持续存在的癫痫性痉挛的电临床特征的详细报道较少。我们试图进一步描述这一独特群体的特征。
我们回顾性分析了1993年至2000年间在我们的三级转诊中心接受评估的26例确诊为癫痫性痉挛的患者（4至17岁；平均93个月）的临床和视频脑电图数据。
在我们的一半病例中，癫痫性痉挛与神经元迁移障碍、严重围产期窒息和遗传异常有关。发作间期脑电图在大多数患者中显示为广泛性减慢，慢波瞬变后背景波幅衰减是与癫痫性痉挛相关的最常见发作期脑电图模式（19例）。其他发作类型（括号内为病例数）包括有或无前驱痉挛的强直发作（13例）、部分性发作（11例）、肌阵挛发作（11例）、全身强直阵挛发作（6例）、非典型失神发作（2例）和失张力发作（1例）。脑电图背景更规整（尤其是频率≥7Hz）的病例更有可能具有较好的认知功能。脑电图背景持续紊乱和高峰节律紊乱持续存在与发育不良结局相关。
2岁以后仍持续存在癫痫性痉挛的患者构成了一个真正难治的群体，临床医生应更好地认识这一群体。有趣的是，尽管许多治疗方法使癫痫发作减少了50%以上，但干预后神经认知功能和生活质量均未得到实质性改善。发作间期脑电图背景对预测神经学结局最有帮助。
