Department of Biomedical Sciences and Human Oncology, University Hospital of Bari, Bari, Italy.
Department of Basic Medical Sciences Neurosciences and Sense Organs, University Hospital of Bari, Bari, Italy.
Am J Case Rep. 2021 May 17;22:e931104. doi: 10.12659/AJCR.931104.
BACKGROUND Anti-N methyl D-aspartate receptor encephalitis (anti-NMDArE) is a disorder in which triggers such as infectious agents or neoplastic disease can lead to an autoimmune response against the nervous system, although this disorder is usually idiopathic. Some patients with anti-NMDArE have evidence of other autoimmune alterations. Here, we present a case of non-paraneoplastic anti-NMDArE with elevation of serum anti-thyroid antibodies and a literature review of this association. CASE REPORT A 16-year-old girl was admitted in the University Hospital of Bari for a new onset of tonic-clonic seizures. Progressively, the patient manifested also psychomotor agitation, language difficulties, memory impairment, psychotic symptoms, autonomic dysfunction, and psychomotor retardation. Blood evaluation revealed the presence of anti-thyroglobulin, anti-thyroperoxidase, and anti-NMDAr antibodies. Cerebrospinal fluid analysis confirmed the diagnosis of anti-NMDArE. No tumors were found. Treatment with intravenous immunoglobulin, steroids, and plasma exchange relieved symptoms and decreased levels of serum anti-NMDAr antibodies. After 12 months, the patient had full recovery of communicative capacity, with the persistence of slight difficulty of memory and mild tendency to irritability. Blood exams shown persistence of anti-NMDAr positivity and absence of anti-thyroid antibodies. CONCLUSIONS We report a rare case in which an autoimmune involvement of thyroid gland was concurrent with an anti-NMDArE. It would be useful for clinical practice to clarify whether the presence of anti-thyroid antibody an characterize the clinical course, prognosis, and response to treatment of the idiopathic type of anti-NMDArE.
抗 N 甲基-D-天冬氨酸受体脑炎(抗 NMDArE)是一种疾病,其触发因素如感染因子或肿瘤性疾病可导致针对神经系统的自身免疫反应,尽管这种疾病通常是特发性的。一些抗 NMDArE 患者存在其他自身免疫改变的证据。在此,我们报告一例非副肿瘤性抗 NMDArE,伴有血清抗甲状腺抗体升高,并对该关联进行文献复习。
一名 16 岁女孩因强直阵挛性发作而入住巴里大学医院。随后,患者出现精神运动性激越、语言困难、记忆障碍、精神病症状、自主神经功能障碍和精神运动迟缓。血液评估显示存在抗甲状腺球蛋白、抗甲状腺过氧化物酶和抗 NMDAr 抗体。脑脊液分析证实了抗 NMDArE 的诊断。未发现肿瘤。静脉注射免疫球蛋白、类固醇和血浆置换治疗缓解了症状并降低了血清抗 NMDAr 抗体水平。12 个月后,患者完全恢复了沟通能力,但仍存在记忆轻微困难和轻度易怒倾向。血液检查显示抗 NMDAr 持续阳性,而抗甲状腺抗体阴性。
我们报告了一例罕见的病例,其中甲状腺的自身免疫参与与抗 NMDArE 同时发生。阐明抗甲状腺抗体的存在是否能表征特发性抗 NMDArE 的临床病程、预后和治疗反应,对临床实践将很有帮助。