【摘要】 目的　总结儿童交替性偏瘫 (AHC)的临床特征。方法　对 13例AHC患儿的临床与实验室资料进行分析。 13例均做头颅MRI、脑电图 (EEG)检查 ,血氨基酸、尿有机酸分析 ,血乳酸、丙酮酸测定。部分患儿做头颅磁共振血管成像 (MRA)、数字减影动脉造影 (DSA)及录像脑电图 (VEEG)监测。结果　 13例中男 12例 ,女 1例。发病年龄 2d～ 55个月 ,平均 13 .1个月。起病症状表现为眼的运动异常 (包括斜视、双眼向上或向一侧凝视、眼震 ) 2例 ,眼的运动异常伴肌张力不全姿势 9例 ,偏瘫2例。本组均有反复的偏瘫发作 ,每次发作持续时间数分钟～ 10d。发作频率从每天 8次到每两月 1次。 10例在偏瘫发作时反复出现眼的运动异常或肌张力不全姿势 ,伴舞蹈手足徐动 2例 ,共济失调 1例。 7例曾有四肢瘫的病史 ,在四肢瘫发作时伴构音障碍或失语 4例 ,伴呼吸急促和吞咽困难各 2例。无力和上述发作性症状在睡眠时减轻。有智力障碍者 9例 ,惊厥 3例。除 3例有EEG或VEEG异常外 ,其余实验室检查均正常。 12例用氟桂利嗪治疗的患儿中 ,有 8例偏瘫发作严重程度、持续时间和发作频率有降低。结论　AHC是一种以频繁发作的交替性偏瘫伴锥体外系症状和智力低下为临床特征的疾病 ,氟桂利嗪治疗对部分病例有效更多还原

【Abstract】 Objective To summarize the clinical characteristics of alternating hemiplegia of childhood (AHC). Methods The clinical data of 13 children with AHC were analyzed. Cranial MRI, EEG, analysis of serum amino acids and urinary organic acids, measurement of plasma lactate and pyruvate levels were done in all patients. Other laboratory examinations such as magnetic resonance angio imaging (MRA), digital subtraction arteriography (DSA) and Video EEG were also performed in some patients. Results Of the 13 patients,12 were male, and 1 was female. The age of onset was from 2 days to 55 months (average 13.1 months). The initial symptoms were abnormal ocular movements (AOMs) consisting of ocular deviation, gaze or nystagmus in 2 cases, AOMs and dystonic posturing in 9 cases, hemiplegia in 2 cases. All patients had recurrent alternating hemiplegic episodes. The hemiplegic attacks lasted from a few minutes to 10 days. The occurrence of the attacks ranged from 8 times daily to one time every 2 months. In 10 patients the abnormal eye movements or dystonic posturing, at times, recurred intermittently during the hemiplegic attack. Choreoathetosis was present in 2 cases. Ataxia was present in 1 case; 7 patients also had the episodes of quadriplegia. During the episodes of quadriplegia, dysarthria or aphasia was present in 4 cases, dyspnea and dysphagia was present in 2 cases, respectively. Sleep could relieve both weakness and associated paroxysmal symptoms. Mental retardation was present in 9 cases, seizures in 3 cases. Except for EEG or Video EEG was abnormal in 3 patients, other laboratory investigations were normal in all patients. Twelve patients received flunarizine therapy. Flunarizine reduced the severity, duration, or frequency of hemiplegic attacks in 8 patients. Conclusion The results suggest that AHC is characterized by frequent episodes of alternating hemiplegia with extrapyramidal symptoms and mental retardation, flunarizine is effective in treating some AHC patients.更多还原