Long-term outcome after cognitive and behavioral regression in nonlesional epilepsy with continuous spike-waves during slow-wave sleep
Long-term outcome after cognitive and behavioral regression in nonlesional epilepsy with continuous spike-waves during slow-wave sleep
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DOI：
10.1111/j.1528-1167.2012.03465.x
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发表时间：
2012-06-01
期刊：
影响因子：
5.6
通讯作者：
Roulet-Perez, Eliane
中科院分区：
文献类型：
--
作者：
Seegmueller, Caroline;Deonna, Thierry;Roulet-Perez, Eliane
目的：对10例青少年和青年成人慢性慢波睡眠（CSWS）期间因非病变局灶性癫痫（主要是额叶癫痫）并发连续尖峰波而出现儿童认知和行为倒退的患者进行长期随访。方法：回顾过去的医学和脑电图（EEG）数据，并进行神经心理测试，探索主要的认知功能。主要发现：平均随访15.6年（范围823年）后，10例患者均未完全恢复，但4例恢复了正常智力，基本独立。长期整体智力衰退的患者结果最差，而那些更具体和短期缺陷的患者恢复得最好。除一名患者外，所有患者的明显行为障碍都得到了缓解。执行功能既不严重也不均匀地受到影响。在活跃期（AP）有3例额叶综合征患者仅表现出轻微的残余执行和社会认知缺陷。主要的认知能力提高发生在AP治疗后不久，但质量上的改善继续发生。长期预后与CSWS持续时间相关性最好。意义：我们的研究结果强调，CSWS停止后的认知恢复取决于初始回归的严重程度和持续时间。我们的患者中没有人有严重的执行和社会认知缺陷与保留智力，正如在早期额叶破坏性病变的成人中所报道的那样。早期识别伴有CSWS的癫痫并迅速引入有效的治疗对于获得最佳可能的结果至关重要。
Purpose: To present the long-term follow-up of 10 adolescents and young adults with documented cognitive and behavioral regression as children due to nonlesional focal, mainly frontal, epilepsy with continuous spike-waves during slow wave sleep (CSWS). Methods: Past medical and electroencephalography (EEG) data were reviewed and neuropsychological tests exploring main cognitive functions were administered. Key Findings: After a mean duration of follow-up of 15.6 years (range, 823 years), none of the 10 patients had recovered fully, but four regained borderline to normal intelligence and were almost independent. Patients with prolonged global intellectual regression had the worst outcome, whereas those with more specific and short-lived deficits recovered best. The marked behavioral disorders resolved in all but one patient. Executive functions were neither severely nor homogenously affected. Three patients with a frontal syndrome during the active phase (AP) disclosed only mild residual executive and social cognition deficits. The main cognitive gains occurred shortly after the AP, but qualitative improvements continued to occur. Long-term outcome correlated best with duration of CSWS. Significance: Our findings emphasize that cognitive recovery after cessation of CSWS depends on the severity and duration of the initial regression. None of our patients had major executive and social cognition deficits with preserved intelligence, as reported in adults with early destructive lesions of the frontal lobes. Early recognition of epilepsy with CSWS and rapid introduction of effective therapy are crucial for a best possible outcome.