Repeated Encephalopathy and Hemicerebral Atrophy in a Patient with Familial Hemiplegic Migraine Type 1
Repeated Encephalopathy and Hemicerebral Atrophy in a Patient with Familial Hemiplegic Migraine Type 1
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DOI：
10.2169/internalmedicine.53.0295
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发表时间：
2014-01-01
影响因子：
1.2
通讯作者：
Okamoto, Koichi
中科院分区：
文献类型：
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作者：
Tashiro, Yuichi;Yamazaki, Tsuneo;Okamoto, Koichi
我们在此描述了一名患有家族性偏瘫偏头痛的 38 岁男性病例，其电位依赖性钙离子通道 (CACNA1A) 基因存在 T666M 突变。他的偏头痛还伴有偏瘫和意识障碍。脑磁共振成像显示右皮质半球异常。单光子发射计算机断层扫描显示同侧部位 iomazenil 摄取减少，Tc-99m-半胱氨酸乙酯二聚体摄取增加。正电子发射断层扫描显示同一区域的F-18-氟脱氧葡萄糖摄取减少，随后出现萎缩性变化。接受丙戊酸钠治疗后，患者的脑萎缩停止了。该病例表明，通过充分的预防可以预防脑萎缩的进展。
We herein describe a case of a 38-year-old man with familial hemiplegic migraine with a T666M mutation in the electrical potential-dependent calcium ion channel (CACNA1A) gene. His migraine was accompanied by hemiparesis and impaired consciousness. Brain magnetic resonance imaging revealed abnormalities in the right cortical hemisphere. Single-photon emission computed tomography demonstrated a decrease in iomazenil uptake and an increase in Tc-99m-ethyl cysteinate dimer uptake at the ipsilateral site. Positron emission tomography showed a decrease in F-18-fluorodeoxyglucose uptake in the same area, which later showed atrophic changes. The patient's brain atrophy ceased after treatment with sodium valproate. This case suggests that the progression of brain atrophy can be prevented with adequate prophylaxis.