Richard-Mornas A, Jousserand G, Camdessanche J-P, Forest F, Paul S, Antoine J-C
Centre de référence maladies rares syndromes paranéoplasiques, CHU de Lyon, hôpital neurologique, Bron, France.
Rev Med Interne. 2012 Jan;33(1):41-5. doi: 10.1016/j.revmed.2011.05.003. Epub 2011 Jun 30.
N-methyl-D-aspartate receptor antibody (anti-NMDA-r AB) encephalitis has been recently identified. We report two cases illustrating the clinical features, response to immunomodulatory treatment and involvement of B-lymphocytes that characterizes this disorder.
These patients illustrated the classic clinical features of anti-NMDA-r AB encephalitis including occurrence in young female, presence of severe neurological and psychiatric manifestations with confusion, seizures, mutism, hypovigilence and involuntary movements, and inflammatory cerebrospinal fluid. Both patients improved after immunotherapy. In case 1, the encephalitis was associated with an ovarian teratoma containing neuronal elements. In case 2, there was no tumor identified. A brain biopsy showed prominent perivascular B-cells infiltrates with some T-cells distributed in the brain parenchyma.
Anti-NMDA-r AB encephalitis is certainly not rare and needs to be promptly recognized and treated. An associated neoplasia is inconstant and the pathophysiology involves humoral immunity.
N-甲基-D-天冬氨酸受体抗体(抗-NMDA-r AB)脑炎最近已被确认。我们报告两例病例,阐述了这种疾病的临床特征、对免疫调节治疗的反应以及B淋巴细胞的参与情况。
这些患者展现了抗-NMDA-r AB脑炎的典型临床特征,包括发病于年轻女性,伴有严重的神经和精神症状,如意识模糊、癫痫发作、缄默、警觉性降低及不自主运动,以及脑脊液炎症。两名患者在免疫治疗后均有改善。病例1中,脑炎与一个含有神经元成分的卵巢畸胎瘤相关。病例2中,未发现肿瘤。脑活检显示脑实质内血管周围有明显的B细胞浸润,并有一些T细胞分布。
抗-NMDA-r AB脑炎肯定并不罕见,需要及时识别和治疗。相关肿瘤形成情况不一,其病理生理学涉及体液免疫。