Alves-Leon Soniza Vieira, Cardoso Maria Fátima Bento de Souza, Pereira Valéria Coelho Santa Rita, Meira Isabela D'Andrea
Universidade Federal do Estado do Rio de Janeiro.
Arq Neuropsiquiatr. 2009 Dec;67(4):986-94. doi: 10.1590/s0004-282x2009000600005.
Epileptic syndromes with absence seizures (AS) possess unique clinical and electroencephalographic (EEG) characteristics. In typical or atypical AS, ictal phenomenology may include various characteristics. Vídeo-EEG monitoring enables findings to be correlated with ictal phenomenology.
To evaluate the different AS in a cohort of patients with drug-resistant epilepsy (DRE) based on the International League against Epilepsy (ILAE)'s 2006 classification, to correlate with ictal phenomenology recorded and to apply the Panayiotopoulos criteria.
This study included patients with criteria of AS followed up at the Epilepsy Clinic. A dual, cross-sectional cohort study was carried out between 2005 and 2008. Patients receiving care in the Epilepsy Program of the HUCFF-UFRJ, who had been investigated by video-EEG and who presented clinical and EEG criteria for absence seizures, typical or atypical, according to the criteria defined by the ILAE, were included in the study, independent of age onset, the review of clinical history, age onset, family history, epilepsy onset and evolution, seizures phenomenology, antiepileptic drugs response and neuroimaging studies were used to classify the patients among the different epileptic syndrome associated to absence seizures.
Typical absences were more frequent (71.4%) than atypical absences. Cases of juvenile absence epilepsy were the most frequent (19%) in this series, followed by childhood absence epilepsy (14.4%) and juvenile myoclonic epilepsy (4.8%). In 14 patients (66.67%), diagnosis was modified from focal epilepsy to primary generalized epilepsy. Clinical and EEG diagnosis of absence epilepsy resulted in a dramatic improvement in the control of seizures following modification of diagnosis and indication of an appropriate antiepileptic drug.
Our results show that typical AS are more frequent than atypical. AS was successfully defined in 10 patients following application of Panayiotopoulos' criteria. The consequent change in diagnosis and therapy resulted in resolution of refractoriness in 9 patients. We concluded that in DRE, AS associated to unusual ictal phenomenology improve dramatically when diagnosed by video-EEG, permitting seizures to be controlled. Clinical and EEG evaluation confirm that myoclonus, automatisms and autonomic disorders are involved and that the consciousness may be affected to different degrees.
伴有失神发作(AS)的癫痫综合征具有独特的临床和脑电图(EEG)特征。在典型或非典型AS中,发作期现象学可能包括各种特征。视频脑电图监测能够使检查结果与发作期现象学相关联。
根据国际抗癫痫联盟(ILAE)2006年的分类,评估一组耐药性癫痫(DRE)患者中的不同AS,将其与记录的发作期现象学相关联,并应用帕纳约托普洛斯标准。
本研究纳入了在癫痫诊所随访的符合AS标准的患者。在2005年至2008年期间进行了一项双重横断面队列研究。纳入了在里约热内卢联邦大学胡西尼奥·科雷亚·德法里亚基金会癫痫项目接受治疗、接受过视频脑电图检查且根据ILAE定义的标准呈现典型或非典型失神发作的临床和脑电图标准的患者,不受发病年龄影响,通过回顾临床病史、发病年龄、家族史、癫痫发作的起始和演变、发作期现象学、抗癫痫药物反应以及神经影像学研究来将患者分类到与失神发作相关的不同癫痫综合征中。
典型失神发作比非典型失神发作更常见(71.4%)。本系列中青少年失神癫痫病例最常见(19%),其次是儿童失神癫痫(14.4%)和青少年肌阵挛癫痫(4.8%)。在14例患者(66.67%)中,诊断从局灶性癫痫改为原发性全身性癫痫。失神癫痫的临床和脑电图诊断在诊断改变及给予适当抗癫痫药物指示后,癫痫发作控制情况有显著改善。
我们的结果表明典型AS比非典型更常见。应用帕纳约托普洛斯标准后,在10例患者中成功定义了AS。诊断和治疗的相应改变使9例患者的难治性得到解决。我们得出结论,在DRE中,与不寻常发作期现象学相关的AS通过视频脑电图诊断时会有显著改善,从而使癫痫发作得到控制。临床和脑电图评估证实肌阵挛、自动症和自主神经功能障碍都有涉及,且意识可能受到不同程度的影响。