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[抗N-甲基-D-天冬氨酸受体脑炎。一种跨学科临床表现]

[Anti-NMDA-receptor encephalitis. An interdisciplinary clinical picture].

作者信息

Prüss H, Dalmau J, Arolt V, Wandinger K-P

机构信息

Experimentelle Neurologie und Klinik und Poliklinik für Neurologie, Charité - Universitätsmedizin Berlin, Berlin.

出版信息

Nervenarzt. 2010 Apr;81(4):396, 398, 400, passim. doi: 10.1007/s00115-009-2908-9.

Abstract

Anti-NMDA-receptor encephalitis is a severe and considerably underdiagnosed form of encephalitis with characteristic clinical features including psychiatric symptoms, decreased levels of consciousness, hypoventilation, epileptic seizures, autonomic dysfunction and dyskinesias. Most patients are primarily seen by psychiatrists, often on the assumption of a drug-induced psychosis. Anti-NMDA-receptor encephalitis had initially been described in young women with ovarian teratoma, but is also common in women without tumour, in men and in children. The diagnosis is based on the characteristic clinical picture, supporting findings of brain MRI, electroencephalogram and cerebrospinal fluid (CSF), and the presence of highly specific autoantibodies directed against the NR1 subunit of NMDA-type glutamate receptors in the serum or CSF. In particular, anti-NMDA-receptor encephalitis must be excluded in patients with 'encephalitis of unknown cause'. In principle, the prognosis is favourable and recovery from symptoms can be expected even after prolonged intensive care treatment and mechanical ventilation. However, improvement correlates with prompt identification of the disorder, early immunotherapy and - in the case of a malignancy - with complete tumour removal. Patient care requires an interdisciplinary approach including neurologists, psychiatrists, paediatricians, oncologists and gynaecologists.

摘要

抗N-甲基-D-天冬氨酸受体(NMDA)脑炎是一种严重且诊断严重不足的脑炎形式,具有特征性临床特征,包括精神症状、意识水平下降、通气不足、癫痫发作、自主神经功能障碍和运动障碍。大多数患者最初由精神科医生诊治,通常被认为是药物性精神病。抗NMDA受体脑炎最初在患有卵巢畸胎瘤的年轻女性中被描述,但在无肿瘤的女性、男性和儿童中也很常见。诊断基于特征性临床表现、脑部磁共振成像(MRI)、脑电图和脑脊液(CSF)的支持性检查结果,以及血清或脑脊液中存在针对NMDA型谷氨酸受体NR1亚基的高度特异性自身抗体。特别是,“病因不明的脑炎”患者必须排除抗NMDA受体脑炎。原则上,预后良好,即使经过长时间的重症监护治疗和机械通气,症状也有望恢复。然而,病情改善与疾病的及时识别、早期免疫治疗以及(如果存在恶性肿瘤)肿瘤的完全切除相关。患者护理需要多学科方法,包括神经科医生、精神科医生、儿科医生、肿瘤学家和妇科医生。

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