Neuropsychiatric Manifestations of Autoimmune Encephalitis in a Tertiary Hospital: A Case Series and Current Perspectives.

Akriti Sinha, Trenton J Smolik, Kamalika Roy, Pradeep C Bollu
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引用次数: 2

Abstract

Importance: Autoimmune encephalitis (AE) refers to a group of neuropsychiatric conditions associated with specific circulating autoantibodies directed against synaptic receptors, neuronal cell surface proteins, and intracellular targets. Increased recognition of these disorders is of value, as affected patients prominently display cognitive impairment, behavioral disturbances, and seizures requiring multidisciplinary teams, with early recognition often impacting prognosis.

Observations: This case series is based on a retrospective record review of adult patients diagnosed with AE between January 1, 2010- January 1, 2020. Cases 1 and 2, demonstrating anti- N-methyl-d-aspartate receptor (NMDAR) encephalitis with initial manifestations of neurologic and psychiatric symptoms, correlate with the literature describing a higher prevalence of this condition in young women. Case 3, despite being seronegative, exhibited classic features of anti-NMDAR encephalitis. Case 4 demonstrates a classic presentation of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis: a middle-aged male with psychosis, altered mentation, and epilepsy. Case 5 had a more indolent neuropsychiatric presentation with mild elevation of N-type voltage-gated potassium channel (VGKC) antibody. Case 6, with glutamic acid decarboxylase 65 (GAD65) antibody, was an elderly female with speech dysfunction and altered mentation, and case 7 was an elderly male with GAD65 antibody who had stiff-person syndrome, ataxia, cognitive decline, and thymoma.

Conclusions: This retrospective case series describes the clinical details of 7 individuals with AE and overlapping neuropsychiatric symptoms. This series is limited in scope, with a small number of cases and observational findings, which prevents specific conclusions from being drawn. Despite this limitation, the present article explores the nuances of variable presentations of this disease to inform better interdisciplinary management and emphasize the gap areas that need rigorous research.

三级医院自身免疫性脑炎的神经精神表现:一个病例系列和当前的观点。
重要性:自身免疫性脑炎(AE)是指与针对突触受体、神经元细胞表面蛋白和细胞内靶标的特异性循环自身抗体相关的一组神经精神疾病。增加对这些疾病的认识是有价值的,因为受影响的患者明显表现出认知障碍、行为障碍和癫痫发作,需要多学科团队,早期认识往往影响预后。观察:本病例系列基于2010年1月1日至2020年1月1日期间诊断为AE的成年患者的回顾性记录。病例1和2表现为抗n -甲基-d-天冬氨酸受体(NMDAR)脑炎,初始表现为神经系统和精神症状,这与文献中描述的年轻女性中高发病率相关。病例3虽然血清阴性,但表现出抗nmdar脑炎的典型特征。病例4是典型的抗富亮氨酸胶质瘤失活1 (LGI1)脑炎的表现:一位中年男性伴有精神病、精神状态改变和癫痫。病例5的神经精神表现较为懒散,n型电压门控钾通道(VGKC)抗体轻度升高。病例6为患有谷氨酸脱羧酶65 (GAD65)抗体的老年女性,伴有语言功能障碍和精神状态改变;病例7为患有GAD65抗体的老年男性,患有僵硬人综合征、共济失调、认知能力下降和胸腺瘤。结论:本回顾性病例系列描述了7例AE患者和重叠神经精神症状的临床细节。该系列的范围有限,只有少数病例和观察结果,因此无法得出具体结论。尽管存在这种限制,本文仍探讨了这种疾病的不同表现的细微差别,以告知更好的跨学科管理,并强调需要严格研究的空白领域。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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