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一例急性脑炎伴难治性、重复性部分性癫痫发作,呈现针对谷氨酸受体Gluε2的自身抗体。

A case of acute encephalitis with refractory, repetitive partial seizures, presenting autoantibody to glutamate receptor Gluepsilon2.

作者信息

Ito Hiromichi, Mori Kenji, Toda Yoshihiro, Sugimoto Mayumi, Takahashi Yukitoshi, Kuroda Yasuhiro

机构信息

Department of Pediatrics, Institute of Health Biosciences, The University of Tokushima Graduate School, 3-18-15, Kuramoto-Cho, Tokushima 770-8503, Japan.

出版信息

Brain Dev. 2005 Oct;27(7):531-4. doi: 10.1016/j.braindev.2004.12.005.

Abstract

An 11-year-old male was admitted to our hospital because of high-grade fever, repetitive seizures, and prolonged impairment of consciousness (Glasgow coma scale E1, M5, V1). His seizures were repetitive complex partial seizures that expanded from the unilateral face to the corresponding side of the body. He sometimes developed secondary generalized seizures. While most seizures lasted 1 or 2 min, intractable seizures also frequently (about 5 times/h) occurred. We diagnosed him as encephalitis/encephalopathy, and treated him with artificial respiration, thiamylal sodium, mild hypothermia therapy, steroid pulse therapy, massive gamma-globulin therapy, etc. Afterwards, he had sequelae, such as post-encephalitic epilepsy (same seizures continued to recur), hyperkinesia, impairment of immediate memory, change in character (he became sunny and obstinate), dysgraphia, and mild atrophy of the hippocampus, amygdala, and cerebrum. However, he could still attend a general junior high school. He was diagnosed as acute encephalitis with refractory, repetitive partial seizures (AERRPS). In this case, he was positive for autoantibody to glutamate receptor Gluepsilon2 IgG or IgM in an examination of blood and spinal fluid, and we presumed that this may have influenced his sequelae. In this case, a combination of mild hypothermia therapy, steroid pulse therapy, and massive gamma-globulin therapy was effective.

摘要

一名11岁男性因高热、反复癫痫发作及意识障碍延长(格拉斯哥昏迷量表E1、M5、V1)入院。他的癫痫发作类型为反复复杂部分性发作,从单侧面部扩展至身体相应一侧,有时还会发展为继发性全身性发作。多数癫痫发作持续1至2分钟,但难治性癫痫也频繁发作(约每小时5次)。我们诊断他为脑炎/脑病,并给予人工呼吸、硫喷妥钠、亚低温治疗、类固醇冲击治疗、大剂量丙种球蛋白治疗等。此后,他出现了后遗症,如脑炎后癫痫(相同的癫痫发作持续复发)、运动过多、即刻记忆障碍、性格改变(变得开朗且固执)、书写障碍以及海马体、杏仁核和大脑轻度萎缩。然而,他仍能就读普通初中。他被诊断为急性脑炎伴难治性、反复部分性癫痫发作(AERRPS)。在该病例中,血液和脑脊液检查显示谷氨酸受体Gluε2 IgG或IgM自身抗体呈阳性,我们推测这可能对他的后遗症产生了影响。在这个病例中,亚低温治疗、类固醇冲击治疗和大剂量丙种球蛋白治疗联合应用是有效的。

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