儿童交替性偏瘫的临床特征和脑血流灌注改变

目的探讨儿童交替性偏瘫的临床特点及脑血流改变。方法对近23年收治的11例患儿的临床资料进行分析,并对6例患儿应用单光子发射计算机断层扫描进行脑血流灌注观察,其中4例在发作间期,1例在发作期,1例在发作间期和发作期均进行测定,以兴趣区法作半定量分析。结果11例患儿的临床特征一般为18个月内起病,表现为频繁发作的交替性偏瘫、短暂的眼球震颤、肌张力异常、舞蹈徐动样动作,常伴自主神经功能紊乱和认知功能减退;睡眠可缓解上述症状。2次发作期单光子发射计算机断层扫描均示偏瘫对侧脑血流减少,5次发作间期均正常。结论本病的主要临床特征为一般18个月内起病的反复发作的交替性偏瘫,伴锥体外系症状及智能障碍;发作期偏瘫对侧脑血流减少。

Observation on clinical features and regional cerebral blood flow in patients with alternating hemiplegia of childhood

Objective To explore the clinical features and regional cerebral blood flow changes in alternating hemiplegia of childhood. Methods Eleven children with alternating hemiplegia of childhood in recent 23 years were studied prospectively. Single photon emission computed tomography was performed in 6 patients, including 4 interictal patients, 1 ictal patient, and 1 patient both in interictal and ictal phase. Regional cerebral blood flow was semi-quantitatively analyzed by regions of interest. Results Patients were characterized by onset before 18 months of age universally, and frequent episodes of alternating paralysis,together with transient nystagmus, dystonia, choreoathetosis, autonomic dysfunction and development of cognitive impairment. Sleep consistently relieved both hemiplegia and associated symptoms. Regional cerebral blood flow was decreased in contralateral to the hemiplegic side examined during 2 ictal phases. Five performed during interictal phase were normal. Conclusion Alternating hemiplegia of childhood occurs universally before 18 months of age, and be associated with complicated paroxysmal phenomena and cognitive impairment. Regional cerebral blood flow should be decreased during ictal phase.