Guarino Miriana, La Bella Saverio, Santoro Marco, Caposiena Daniele, Di Lembo Enza, Chiarelli Francesco, Iannetti Giovanni
Department of Pediatrics, University of Chieti-Pescara "G. D'Annunzio", Via Dei Vestini 5, Ospedale Clinicizzato Chieti (CH), 66100 Chieti, Italy.
Department of Radiology, Pescara Public Hospital "Santo Spirito", 65124 Pescara, Italy.
Brain Sci. 2023 Apr 15;13(4):662. doi: 10.3390/brainsci13040662.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis (NMDARe) is the most common cause of nonviral encephalitis, mostly affecting young women and adolescents with a strong female predominance (F/M ratio of around 4:1). NMDARe is characterized by the presence of cerebrospinal fluid (CSF) antibodies against NMDARs, even though its pathophysiological mechanisms have not totally been clarified. The clinical phenotype of NMDARe is composed of both severe neurological and neuropsychiatric symptoms, including generalized seizures with desaturations, behavioral abnormalities, and movement disorders. NMDARe is often a paraneoplastic illness, mainly due to the common presence of concomitant ovarian teratomas in young women. Abdominal ultrasonography (US) is a key imaging technique that should always be performed in suspected patients. The timely use of abdominal US and the peculiar radiological features observed in NMDARe may allow for a quick diagnosis and a good prognosis, with rapid improvement after the resection of the tumor and the correct drug therapy.
抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎(NMDARe)是非病毒性脑炎最常见的病因,主要影响年轻女性和青少年,女性占主导地位(男女比例约为4:1)。尽管其病理生理机制尚未完全阐明,但NMDARe的特征是脑脊液(CSF)中存在抗NMDAR抗体。NMDARe的临床表型包括严重的神经和神经精神症状,如伴有血氧饱和度下降的全身性癫痫发作、行为异常和运动障碍。NMDARe通常是一种副肿瘤性疾病,主要是因为年轻女性中常伴有卵巢畸胎瘤。腹部超声(US)是一项关键的影像学检查技术,对于疑似患者应始终进行此项检查。及时使用腹部超声以及在NMDARe中观察到的特殊影像学特征,可能有助于快速诊断并获得良好预后,肿瘤切除及正确的药物治疗后病情可迅速改善。