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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
Objective To summarize the clinical features of children with alternating hemiplegia (AHC). Methods Clinical and laboratory data of 13 children with AHC were analyzed. All 13 patients underwent cranial MRI, electroencephalogram (EEG) examination, blood amino acids, urine organic acid analysis, blood lactate and pyruvate. Some children do head magnetic resonance angiography (MRA), digital subtraction arterial angiography (DSA) and video recording EEG (VEEG) monitoring. Results 13 cases of male 12 cases, 1 female. The age of onset 2d ~ 55 months, an average of 13.1 months. Symptoms manifested as eye movement abnormalities (including strabismus, both eyes up or to the side of the gaze, nystagmus) in 2 cases, ocular motility disorders with tension in 9 cases, hemiplegia in 2 cases. This group have repeated hemiplegia episodes, each episode lasting a few minutes ~ 10d. Seizure frequency from 8 times a day to once every two months. 10 cases of hemiplegia recurrence of ocular motility abnormalities or dystonic posture, accompanied by dance hands and feet Xu move in 2 cases, ataxia in 1 case. Seven patients had a history of quadriplegia. Four patients had dysarthria or aphasia with episodes of quadriplegia with two cases of shortness of breath and dysphagia. Weakness and the above-mentioned seizures reduce during sleep. 9 cases of mental retardation, convulsions in 3 cases. Except for 3 cases of EEG or VEEG abnormalities, the remaining laboratory tests were normal. Among the 12 children treated with flunarizine, the severity of hemiplegia, the duration and the frequency of seizures decreased in 8 patients. Conclusions AHC is a disease characterized by frequent episodes of alternating paralysis with extrapyramidal symptoms and mental retardation. Flunarizine treatment is effective in some cases