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目的探讨Panayiotopoulos综合征(PS)的临床和脑电图特征,以提高对其的认识。方法对首都医科大学附属北京儿童医院神经科门诊2008年4月至2009年12月收治的8例PS患儿临床资料进行分析。结果 8例中男女各4例,发病年龄2岁9个月至5岁11个月,出生史、生长发育史均正常。2例既往有高热惊厥史。8例癫痫发作均以呕吐为起始表现,其中3例伴有面色苍白,四肢发软,3例呕吐前诉恶心,1例诉头晕、恶心,1例呕吐后有便失禁。发作后期6例表现为双眼向一侧斜视,2例表现为双眼上翻。其中2例头向眼斜视一侧扭转,1例伴有一侧上肢抽动,2例继发全身阵挛性抽搐,7例伴有意识丧失。6例患儿发作持续10～20min,持续2～3min或120min者各1例。脑电图显示枕、后颞导联中高幅棘波、棘慢波4例,两侧前额、额散在中高幅棘波、棘慢波2例,中央、中颞区棘波1例,脑电图未见异常1例。结论 PS是发生于儿童早期的年龄相关的特发性良性癫痫综合征。发作起始特征表现为以呕吐为主的自主神经症状,后期多出现眼偏斜、意识障碍,甚至四肢抽搐。脑电图特点是以枕叶为主的多灶性放电。临床表现是诊断本病的重要依据。
Objective To investigate the clinical and electroencephalographic features of Panayiotopoulos syndrome (PS) in order to improve their understanding. Methods The clinical data of 8 children with PS who were admitted to Department of Neurology, Beijing Children’s Hospital Affiliated to Capital Medical University from April 2008 to December 2009 were analyzed. Results There were 4 males and 4 females in 8 cases. The age of onset was from 2 years old to 9 months to 5 years old and 11 months old. The history of birth, growth and development were all normal. 2 cases of history of febrile seizures. 8 cases of seizures were vomiting as the initial performance, of which 3 cases were accompanied by pale, limbs soft, vomiting, vomiting in 3 cases before nausea, 1 case of dizziness, nausea, vomiting after incontinence. Post-attack in 6 cases showed side to side strabismus, 2 cases showed double upturned. Two of them turned to the side of the ocular strabismus, one was accompanied by twitching of the upper limbs, the other two were generalized clonic convulsions and the other seven were loss of consciousness. 6 cases of attack in children lasting 10 ~ 20min, lasting 2 ~ 3min or 120min in 1 case. EEG showed occipital and posterior temporal leads in high amplitude spikes, spikes and slow waves in 4 cases, both sides of the forehead, scattered in the high spikes, spikes and slow waves in 2 cases, the central and temporal spikes in 1 case, EEG Figure no abnormalities in 1 case. Conclusion PS is an age-related idiopathic benign seizure syndrome that occurs in early childhood. The initial onset of seizures manifested as vomiting-based autonomic symptoms, more post-ocular opacity, disturbance of consciousness, and even limbs and convulsions. EEG is characterized by occipital lobe multifocal discharge. Clinical manifestations of the disease is an important basis for diagnosis.