Ohmura Kayo, Suzuki Yasuhiro, Saito Yoshiaki, Wada Takahito, Goto Mikio, Seto Shiro
Department of Pediatrics, Kishiwada City Hospital, Kishiwada, Japan.
Brain Dev. 2012 Sep;34(8):691-5. doi: 10.1016/j.braindev.2011.11.002. Epub 2011 Dec 1.
A 10-year-old boy with psychomotor developmental delay and cerebellar vermis atrophy developed right hemiplegia with vomiting, unconsciousness, convulsions, and late-onset fever. Slow delta activity was noted over the left hemisphere on electroencephalography, and neuroimaging revealed swelling of the left temporo-occipital cerebral cortex with restricted diffusivity, successive transient cortical atrophy, and hyperperfusion over the left cerebral hemisphere. Interleukin-6 was elevated in the cerebrospinal fluid. The acute symptoms resolved completely within 3 weeks after onset, but hypoperfusion persisted in the left posterior cortex thereafter. Another episode with transient left hemiplegia appeared 7 months later, followed by recurrence of migraine attacks. Analysis of the CACNA1A gene revealed a mutation of c.1997 C>T (p.T666M). None of his family members had migraine. This case represents an unusual evolution of sporadic hemiplegic migraine with manifestations of acute encephalopathy, for which the role of migraine-related inflammatory process is assumed.
一名患有精神运动发育迟缓及小脑蚓部萎缩的10岁男孩出现了右侧偏瘫，并伴有呕吐、意识丧失、抽搐及迟发性发热。脑电图显示左半球出现慢波δ活动，神经影像学检查发现左侧颞枕叶脑皮质肿胀，弥散受限，相继出现短暂性皮质萎缩，左侧大脑半球血流灌注增多。脑脊液中白细胞介素-6升高。急性症状在发病后3周内完全缓解，但此后左侧后皮质持续存在灌注不足。7个月后出现另一次短暂性左侧偏瘫发作，随后偏头痛发作复发。CACNA1A基因分析显示存在c.1997 C>T（p.T666M）突变。他的家庭成员均无偏头痛病史。该病例代表了散发性偏瘫性偏头痛的一种不寻常演变，伴有急性脑病表现，推测与偏头痛相关的炎症过程有关。
