Juvenile Neuropsychiatric Systemic Lupus Erythematosus With Magnetic Resonance Imaging Findings Similar to Acute Necrotizing Encephalopathy
Juvenile Neuropsychiatric Systemic Lupus Erythematosus With Magnetic Resonance Imaging Findings Similar to Acute Necrotizing Encephalopathy
复制标题
青少年神经精神系统性红斑狼疮的磁共振成像结果与急性坏死性脑病相似
DOI：
10.1097/rhu.0000000000001698
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发表时间：
2020
期刊：
影响因子：
--
通讯作者：
Hasegawa Shunji
中科院分区：
文献类型：
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作者：
Korenaga Yuno;Wakiguchi Hiroyuki;Matsushige Takeshi;Inoue Hirofumi;Mizutani Makoto;Furuta Takashi;Kawamura Mai;Nakamura Tamaki;Okazaki Fumiko;Yasudo Hiroki;Maki Takashi;Hasegawa Shunji
急性坏死性脑病(ANE)是一种特殊类型的脑病，死亡率高。1诊断依据是脑磁共振成像显示双侧丘脑、大脑白质、脑干和小脑存在对称性病变。2幼年系统性红斑狼疮(JSLE)未见急性坏死性脑病或血管样脑病的报道。一名15岁的日本女孩因主诉发烧和惊厥后意识障碍而住进本院。她有颧部皮疹和低血清补体水平。免疫学研究显示抗核抗体、抗Sm抗体和抗心磷脂抗体呈阳性，而抗dsDNA和抗肾上腺素B受体2抗体呈阴性。对大脑的磁共振成像扫描没有显示任何异常。入院三天后，由于严重的呼吸困难，患者不得不进行插管，并在我们的重症监护病房进行机械通气。脑磁共振成像显示脑干、双侧丘脑和多个皮质明显异常(图)。她被诊断为JSLE相关的ANE样脑病。我们先用甲基强的松龙冲击治疗，然后用强的松龙治疗；然而，她的神经精神症状并没有改善。然后静脉注射环磷酰胺(CyC)(每月500 mg/m2)。第一次注射两周后，她恢复了知觉。在1年的随访中，除了轻微的认知障碍外，她仍然很好(补充表1，http://links.LWW。COM/RHU/A252)，并准备恢复正常的功能生活，包括上学。
Acute necrotizing encephalopathy (ANE) is a distinctive type of encephalopathy with a high mortality rate. 1 The diagnosis is based on brain magnetic resonance imaging showing the presence of symmetrical lesions in the bilateral thalami, cerebral white matter, brainstem, and cerebellum. 2 Acute necrotizing encephalopathy or ANE-like encephalopathy has not been reported in juvenile systemic lupus erythematosus (JSLE). A 15-year-old Japanese girl was admitted to our hospital with complaints of pyrexia and disturbed consciousness after convulsions. She had a malar rash and low serum complement levels. Immunologic studies were positive for antinuclear, anti-Sm, and anticardiolipin antibodies, but negative for anti-dsDNA and anti–ephrin type B receptor 2 antibodies. Magnetic resonance imaging scans of the brain showed no abnormalities. Three days after admission, the patient had to be intubated, owing to severe dyspnea, and placed on mechanical ventilation in our intensive care unit. Brain magnetic resonance imaging revealed marked abnormalities in the brainstem, bilateral thalami, and multiple cortices (Figure). She was diagnosed with JSLE-associated ANE-like encephalopathy.We administered methylprednisolone pulse therapy followed by prednisolone; however, her neuropsychiatric symptoms did not improve. We then administrated intravenous cyclophosphamide (CYC)(500 mg/m2 per month). Two weeks after the first dose, she regained consciousness. She remained well except for mild cognitive impairment at 1-year follow-up (Supplementary Table 1, http://links. lww. com/RHU/A252) and was ready to resume a normal functional life, including attending school.