Transverse myelitis extended to disseminated encephalitis in systemic lupus erythematosus: Histological evidence for vasculitis
Transverse myelitis extended to disseminated encephalitis in systemic lupus erythematosus: Histological evidence for vasculitis
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系统性红斑狼疮中横贯性脊髓炎扩展到播散性脑炎：血管炎的组织学证据
DOI：
10.3109/14397595.2014.948535
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发表时间：
2016
影响因子：
2.2
通讯作者：
S. Hirohata
中科院分区：
文献类型：
--
作者：
Toshihiro Tono;T. Nagai;T. Hoshiyama;Y. Sakuma;T. Wada;S. Tanaka;S. Hirohata
1例42岁女性因系统性红斑狼疮合并肾小球肾炎和肺动脉高压入院治疗。在治疗这些并发症的过程中，她出现了运动性瘫痪和横贯性脊髓炎引起的感觉丧失。尽管接受了甲基强的松龙冲击治疗，但她因播散性脑炎而进一步发展为急性混淆状态，并陷入呼吸骤停。实验室检查发现血清和脑脊液中抗NR2抗体升高。虽然经过大剂量皮质类固醇和静脉注射环磷酰胺的广泛治疗后，她从播散性脑炎中恢复过来，但她突然死于肺动脉高压。尸检结果证实，整个脊髓周围存在液化坏死，伴有内膜增生和小动脉闭塞，并伴有单个核细胞浸润和内弹力板断裂。因此，我们的患者很有可能通过脊髓血管炎发展成纵向横贯性脊髓炎，并延伸到脑干和脑实质，导致播散性脑炎的发展。
A 42-year-old woman was admitted due to systemic lupus erythematosus complicated with glomerulonephritis and pulmonary hypertension. During the treatment for these complications, she presented motor paresis and sensory loss caused by transverse myelitis. In spite of methyl prednisolone pulse therapy, she further developed acute confusional state due to disseminated encephalitis and fell into respiratory arrest. On laboratory examination, elevation of anti-NR2 antibodies in serum as well as in cerebrospinal fluid was noted. Although she recovered from the disseminated encephalitis after extensive treatment with high doses of corticosteroid and intravenous cyclophosphamide, she suddenly died of pulmonary hypertension. Autopsy findings confirmed the presence of liquefaction necrosis in the entire circumference of the whole spinal cord along with intimal hyperplasia and obliteration of the small arteries, accompanied by mononuclear cell infiltration and disruption of internal elastic lamina. It is therefore most likely that our patient developed longitudinal transverse myelitis through spinal cord vasculitis, which extended to brainstem and brain parenchyma, leading to the development of disseminated encephalitis.