Sporadic hemiplegic migraine presenting as acute encephalopathy
Sporadic hemiplegic migraine presenting as acute encephalopathy
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DOI：
10.1016/j.braindev.2011.11.002
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发表时间：
2012-09-01
影响因子：
1.7
通讯作者：
Seto, Shiro
中科院分区：
文献类型：
--
作者：
Ohmura, Kayo;Suzuki, Yasuhiro;Seto, Shiro
一个10岁的男孩，患有精神发育迟缓和小脑蚓部萎缩，出现右侧偏瘫、呕吐、意识不清、惊厥和晚发性发热。在脑电图上观察到左半球的缓慢δ活动，神经影像学显示左颞枕叶大脑皮质肿胀，扩散受限，连续一过性皮质萎缩，左大脑半球灌注过度。脑脊液中白细胞介素-6升高。急性症状在发病后3周内完全消退，但此后左后皮质持续灌注不足。7个月后出现另一次短暂性左侧偏瘫发作，随后偏头痛复发。对CACNA 1A基因的分析揭示了c.1997 C>T（p.T666M）的突变。他的家人都没有偏头痛。这个病例代表了散发性偏瘫性偏头痛的一个不寻常的演变，表现为急性脑病，偏头痛相关的炎症过程的作用被假定。(C)2011年日本儿童神经病学学会。Elsevier B. V.出版，保留所有权利。
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. (C) 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.