Mujawar Quais Mohammad, Sen Santanu, Ali Mir Dilshad, Balakrishnan Pramod, Patil Shekhar
Department of Emergency Pediatrics, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.
Pediatr Emerg Care. 2011 Aug;27(8):754-7. doi: 10.1097/PEC.0b013e318226e0a9.
Panayiotopoulos syndrome is early-onset benign childhood epilepsy, now classified as an electroclinical syndrome. The original description in 1989 focused on the triad of nocturnal seizures, tonic eye deviation, and vomiting. With available data from the long-term studies, a wide variety of manifestations have been described, with recognition of autonomic features as being the most prominent aspect of this epilepsy. The presenting symptoms are usually focal seizures comprising autonomic symptoms and/or behavioral changes. Majority of these seizures are in sleep, with half of the seizures progressing to become secondarily generalized. Occasionally, these seizures can present with prominent autonomic features such as ictal vomiting, pallor, flushing/cyanosis, and tachycardia with prolonged thermoregulatory changes lasting for hours, constituting autonomic status epilepticus. Recovery from this autonomic status epilepticus is within hours and is always complete. Autonomic status epilepticus has been previously reported in this epilepsy syndrome, but ictal cardiorespiratory arrest is extremely rare, with only 4 cases being reported in literature. All 4 cases reported in literature recovered spontaneously and did not require resuscitation. Here we present a 3½-year-old male child with Panayiotopoulos syndrome who presented with status epilepticus and ictal cardiorespiratory arrest requiring cardiopulmonary resuscitation for revival.
潘纳约托普洛斯综合征是一种早发性儿童良性癫痫，现被归类为一种电临床综合征。1989年的最初描述聚焦于夜间发作、强直性眼球偏斜和呕吐三联征。根据长期研究的现有数据，已描述了各种各样的表现，其中自主神经特征被认为是这种癫痫最突出的方面。呈现的症状通常是包括自主神经症状和/或行为改变的局灶性发作。这些发作大多数发生在睡眠中，其中一半的发作会进展为继发性全身性发作。偶尔，这些发作可表现出突出的自主神经特征，如发作性呕吐、面色苍白、潮红/发绀以及心动过速，伴有持续数小时的长时间体温调节变化，构成自主神经癫痫持续状态。从这种自主神经癫痫持续状态中恢复在数小时内即可完成，且总是完全恢复。自主神经癫痫持续状态此前已在这种癫痫综合征中被报道，但发作性心肺骤停极为罕见，文献中仅报道了4例。文献报道的所有4例均自发恢复，无需复苏。在此，我们报告一名3岁半患有潘纳约托普洛斯综合征的男童，他出现癫痫持续状态和发作性心肺骤停，需要进行心肺复苏才能苏醒。
