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抗N-甲基-D-天冬氨酸受体脑炎伴磁共振成像显示多灶性皮质下白质病变:1例报告并文献复习

Anti-N-methyl-D-aspartate receptor encephalitis concomitant with multifocal subcortical white matter lesions on magnetic resonance imaging: a case report and review of the literature.

作者信息

Wang Rui-Jin, Chen Bu-Dong, Qi Dong

机构信息

Department of Neurology, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong-An Road, Xicheng District, Beijing, 100050, People's Republic of China.

Department of Radiology, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong-An Road, Xicheng District, Beijing, 100050, People's Republic of China.

出版信息

BMC Neurol. 2015 Jul 8;15:107. doi: 10.1186/s12883-015-0366-5.

Abstract

BACKGROUND

Anti-N-methyl-D-aspartate receptor encephalitis is a severe autoimmune disorder characterized by severe psychiatric symptoms, seizures, decreased consciousness, autonomic dysregulation, and dyskinesias. Multifocal subcortical white matter lesions on fluid-attenuated inversion recovery and diffuse weighted images have rarely been reported in previous literature, and serial magnetic resonance imaging changes after plasma exchange have not been presented before.

CASE PRESENTATION

A previously healthy 24-year-old Chinese woman presented with acute psychiatric symptoms characterized by fear and agitation followed by decreased consciousness, dyskinesias, and seizures. Magnetic resonance imaging revealed hyperintense lesions on fluid-attenuated inversion recovery and diffuse weighted images in bilateral subcortical white matter. Cerebrospinal fluid analysis revealed a mild pleocytosis with lymphocytic predominance. Protein and glucose levels were normal. Aquaporin-4 antibodies in serum and cerebrospinal fluid were negative. Identification of anti-N-methyl-D-aspartate receptor antibodies in serum and cerebrospinal fluid confirmed the diagnosis of anti-N-methyl-D-aspartate receptor encephalitis. She was initially treated with combined intravenous immunoglobulin and methylprednisolone without improvement. Plasma exchange was then initiated with good response; the patient made a full recovery after several cycles of plasma exchange. Repeat magnetic resonance imaging performed 1 month after plasma exchange showed partial resolution of the hyperintense lesions in bilateral subcortical white matter, and follow-up magnetic resonance imaging 2 months after plasma exchange showed complete resolution.

CONCLUSION

Anti-N-methyl-D-aspartate receptor encephalitis may be concomitant with multifocal subcortical white matter lesions. Such lesions may resolve after appropriate immunotherapy.

摘要

背景

抗N-甲基-D-天冬氨酸受体脑炎是一种严重的自身免疫性疾病,其特征为严重的精神症状、癫痫发作、意识减退、自主神经功能失调和运动障碍。以往文献中很少报道液体衰减反转恢复序列和弥散加权成像上的多灶性皮质下白质病变,且此前未呈现血浆置换后磁共振成像的系列变化。

病例报告

一名既往健康的24岁中国女性,最初出现以恐惧和激越为特征的急性精神症状,随后出现意识减退、运动障碍和癫痫发作。磁共振成像显示双侧皮质下白质在液体衰减反转恢复序列和弥散加权成像上有高信号病变。脑脊液分析显示轻度细胞增多,以淋巴细胞为主。蛋白和葡萄糖水平正常。血清和脑脊液中的水通道蛋白4抗体均为阴性。血清和脑脊液中抗N-甲基-D-天冬氨酸受体抗体的鉴定确诊为抗N-甲基-D-天冬氨酸受体脑炎。她最初接受静脉注射免疫球蛋白和甲泼尼龙联合治疗,但病情无改善。随后开始进行血浆置换,反应良好;经过几个周期的血浆置换后,患者完全康复。血浆置换1个月后复查磁共振成像显示双侧皮质下白质高信号病变部分消退,血浆置换2个月后的随访磁共振成像显示病变完全消退。

结论

抗N-甲基-D-天冬氨酸受体脑炎可能伴有多灶性皮质下白质病变。经适当的免疫治疗后,此类病变可能消退。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c792/4495850/ba5e965e08df/12883_2015_366_Fig1_HTML.jpg

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