Dan Bernard, Boyd Stewart G
Department of Neurology, Hôpital Universitaire des Enfants Reine Fabiola, Free University of Brussels (ULB), Belgium.
Epileptic Disord. 2006 Jun;8(2):143-50.
Status epilepticus refractory to sequential trials of multiple medication is a rare but significant problem in children. We describe stimulus sensitivity arising during the treatment of convulsive status epilepticus in children (stimulus-sensitive burst-spiking in burst-suppression). We reviewed retrospectively clinical and EEG features in six children (three months to ten years), with status epilepticus requiring intensive care, in whom tactile, auditory and visual stimulation induced myoclonic jerks and bursts of EEG spikes. Sensitivity was not present at onset, but appeared after 24 hours as myoclonic jerks of the eyes, face and limbs, irrespective of the modality and site of stimulation. These were associated with burst-suppression in the EEG, the induced spiking forming the burst component. Various antiepileptic drugs, including GABAergic and NMDA blockers had no effect, but halogenated agents (used in two patients) abolished the sensitivity. Two children died, but the remainder returned to their previous clinical state. We conclude that stimulus sensitivity may appear in the context of refractory status epilepticus treated with high-dose barbiturates. Outcome may be more favorable than previously reported in adults, mostly in the context of post-anoxic or toxic coma. Evaluation of ventilated children in status epilepticus should include electroclinical assessment using sensory stimulation. If present, the drug regime should be reviewed and halogenated agents considered.
在儿童中，对多种药物序贯试验均难治的癫痫持续状态是一个罕见但严重的问题。我们描述了在儿童惊厥性癫痫持续状态治疗期间出现的刺激敏感性（爆发抑制中的刺激敏感爆发性尖波）。我们回顾性分析了六名儿童（年龄从三个月至十岁）的临床和脑电图特征，这些儿童患有需要重症监护的癫痫持续状态，触觉、听觉和视觉刺激会诱发他们的肌阵挛性抽搐和脑电图尖波爆发。刺激敏感性在发病时并不存在，而是在24小时后出现，表现为眼、面和肢体的肌阵挛性抽搐，与刺激的方式和部位无关。这些与脑电图中的爆发抑制相关，诱发的尖波形成爆发成分。包括GABA能和NMDA阻滞剂在内的各种抗癫痫药物均无效，但卤化剂（两名患者使用）消除了这种敏感性。两名儿童死亡，但其余儿童恢复到了之前的临床状态。我们得出结论，在使用高剂量巴比妥类药物治疗难治性癫痫持续状态的情况下，可能会出现刺激敏感性。其结果可能比之前在成人中报道的更有利，主要是在缺氧后或中毒性昏迷的情况下。对处于癫痫持续状态的通气儿童进行评估应包括使用感觉刺激的电临床评估。如果存在刺激敏感性，应重新审查药物治疗方案并考虑使用卤化剂。
