潘尼阿蒂斯综合征的误诊和陷阱。
Misdiagnosis and pitfalls in Panayiotopoulos syndrome.
机构信息
Department of Pediatrics, "G. d'Annunzio" University, Chieti, Italy.
Department of Neuroscience, Imaging and Clinical Sciences, "G. d'Annunzio" University, Chieti, Italy.
出版信息
Epilepsy Behav. 2019 Sep;98(Pt A):124-128. doi: 10.1016/j.yebeh.2019.07.016. Epub 2019 Jul 29.
Panayiotopoulos syndrome (PS) is a frequent (6% among children of 1-15 years) and benign epileptic syndrome, characterized by predominantly autonomic symptoms (emesis, pallor, flushing, cyanosis, mydriasis/miosis, cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal motility) associated with simple motor focal seizures, which can be followed by secondary generalization. Panayiotopoulos syndrome can be extremely insidious, because it can mimic several condition, such as gastroenteritis, gastroesophageal reflux disease, encephalitis, syncope, migraine, sleep disorders, or even metabolic diseases. This peculiar pleiotropism should be kept in mind by child neurologists and pediatricians and general practitioners, because a wrong diagnosis may lead to inappropriate interventions. The consequences are high morbidity, costly mismanagement, and stress for children and their parents. The availability of electroencephalography (EEG) recording in pediatric Emergency Departments might be useful for a prompt and not-cost-consuming diagnosis. On the other hand, it is important to be aware of the possible, multifaceted, clinical presentations of PS and its clinical, radiological, and neurophysiological features in order to improve both recognition and management.
潘氏综合征(PS)是一种常见的(1-15 岁儿童中占 6%)良性癫痫综合征,其特征主要为自主神经症状(呕吐、苍白、潮红、发绀、瞳孔扩大/缩小、心肺和体温调节改变、尿和/或粪便失禁、流涎过多以及肠道运动改变),伴有简单运动性局灶性发作,随后可能出现继发性全面发作。潘氏综合征可能极其隐匿,因为它可能模仿多种病症,如肠胃炎、胃食管反流病、脑炎、晕厥、偏头痛、睡眠障碍,甚至代谢疾病。儿科神经学家、儿科医生和全科医生应牢记这种特殊的多效性,因为错误的诊断可能导致不适当的干预。其后果是发病率高、管理不当费用高、对儿童及其父母造成压力。在儿科急诊部进行脑电图(EEG)记录可能有助于快速、非昂贵的诊断。另一方面,了解 PS 可能出现的、多方面的临床表现及其临床、影像学和神经生理学特征非常重要,这有助于提高识别和管理水平。