Kondo Akiko, Saito Yoshiaki, Maegaki Yoshihiro, Inoue Takehiko, Seki Ayumi, Harada Yuichiro, Ohno Kousaku
Division of Child Neurology, Institute for Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Tottori.
No To Hattatsu. 2006 Nov;38(6):463-7.
A 1-year-old girl with influenza-associated encephalopathy initially exhibited prolonged febrile convulsions and subsequent drowsiness. She became unconsciousness and experienced a cluster of seizures 4 days later. Diffusion-weighted magnetic resonance imaging (DWI) showed high signal intensity in the bilateral frontal white matter. This signal change migrated to the bifrontal cortical areas and the caudate nuclei within 10 days, when T2 elongation appeared over the gray and white matter of frontal lobes. Choreoathetosis and oculogyric crisis were transiently noted at this period. Frontal lobe signs, including the forded mouth opening response, after diminution of these movement disorders. The DWI signal change subsequently resolved and frontal cortical atrophy appeared thereafter. Levels of inflammatory cytokines in the cerebrospinal fluid were normal during the acute phase of clinical course. The biphasic clinical course with initial prolonged seizure, involvement of the frontal lobes, and the progression of signal change on DWI from white to gray matter, meets the characteristics of "status epilepticus-type acute encephalopathy" suggested by Shiomi et al. Although pentobarbital infusion, steroid pulse therapy and mild hypothermia did not show any apparent effects on the clinical course of this patient, further trial of these therapies may be warranted for the treatment of this type of encephalopathy.
一名患有流感相关性脑病的1岁女孩最初出现了长时间的高热惊厥，随后出现嗜睡。4天后她陷入昏迷并经历了一连串癫痫发作。弥散加权磁共振成像（DWI）显示双侧额叶白质高信号强度。这种信号变化在10天内迁移至双侧额叶皮质区域及尾状核，此时额叶灰白质出现T2延长。在此期间短暂出现舞蹈手足徐动症和动眼危象。在这些运动障碍减轻后出现了包括撅嘴张口反应在内的额叶体征。随后DWI信号变化消失，此后出现额叶皮质萎缩。在临床病程急性期，脑脊液中炎性细胞因子水平正常。最初长时间癫痫发作、额叶受累以及DWI信号从白质向灰质变化的双相临床病程，符合Shiomi等人提出的“癫痫持续状态型急性脑病”的特征。尽管戊巴比妥输注、类固醇冲击疗法和轻度低温治疗对该患者的临床病程未显示出明显效果，但对这类脑病的治疗可能有必要进一步试验这些疗法。
