Dundar Nihal Olgac, Cavusoglu Dilek, Kaplan Yusuf Cem, Hasturk Mehmet Oytun
Department of Pediatric Neurology, Faculty of Medicine, Izmir Katip Celebi University, Izmir.
Department of Pediatric Neurology, Faculty of Medicine, Afyonkocatepe University, Afyon.
Clin Neuropharmacol. 2019 May/Jun;42(3):88-90. doi: 10.1097/WNF.0000000000000339.
Alternating hemiplegia of childhood (AHC) is an infrequent neurological disorder characterized by recurrent transient attacks of hemiplegia that last minutes to days and impress either side of the body, dystonic or tonic attacks, and nystagmus. Cognitive or neurological deficits with progressive course are another findings. Epileptic seizures may occur in some patients. We report the medical treatment in a case of AHC in a-12-year-old male patient with convulsions. The patient did not respond to available therapies for AHC, except for aripiprazole. After the initiation of aripiprazole therapy, duration and frequency of hemiplegia episodes were decreased. Also, he is currently seizure-free with topiramate treatment for 3 months. On follow-up, a compound heterozygous ATP1A3 mutation c.868C > T (p.R290C)/c.684 + 1G > A was determined. Aripiprazole may reduce the attacks of AHC, which are resistant to other available therapies.
儿童交替性偏瘫(AHC)是一种罕见的神经系统疾病,其特征为反复出现持续数分钟至数天的偏瘫短暂发作,可累及身体任何一侧,伴有张力障碍或强直发作以及眼球震颤。认知或神经功能缺损呈进行性发展是另外的表现。部分患者可能会出现癫痫发作。我们报告了一名12岁男性AHC患者伴有惊厥的药物治疗情况。该患者对除阿立哌唑外的现有AHC治疗方法均无反应。在开始阿立哌唑治疗后,偏瘫发作的持续时间和频率有所减少。此外,他目前接受托吡酯治疗3个月,未再出现癫痫发作。随访时,确定了一个复合杂合ATP1A3突变c.868C>T(p.R290C)/c.684+1G>A。阿立哌唑可能会减少对其他现有治疗方法耐药的AHC发作。