Alkaissi Hussam, Lee Jung-Hyun, McFarlane Samy I
Internal Medicine, Kings County Hospital Center, Brooklyn, USA.
Internal Medicine, Veterans Affairs Medical Center, Brooklyn, USA.
Cureus. 2023 Jan 27;15(1):e34268. doi: 10.7759/cureus.34268. eCollection 2023 Jan.
Diagnosing autoimmune encephalitis relies on clinical, radiological, and serological studies. Several autoantibodies have been implicated and recognized, with dozens of potential targets identified in the past 20 years. Despite that progress, some patients with encephalitis present a diagnostic dilemma with a seronegative status. The presence of other autoimmune diseases in a patient with encephalitis should provide a clue to the autoimmune nature of a developing neurological syndrome (cognitive, psychiatric, behavioral, and catatonia). In this report, we describe the case of a young man with type 1 diabetes mellitus who was diagnosed with seronegative autoimmune encephalitis after presenting with catatonia. We describe the lengthy clinical course, the various therapeutic trials, and his clinical outcome and response to B-cell depleting agent. This study also discusses the potential pathophysiologic pathways, providing a rationale for the diagnostic workup and therapeutic options for autoimmune encephalopathy in this case presentation.
自身免疫性脑炎的诊断依赖于临床、影像学和血清学研究。多种自身抗体已被证实与该病有关,在过去20年中已确定了数十个潜在靶点。尽管取得了这一进展,但一些脑炎患者仍存在血清学阴性状态的诊断难题。脑炎患者中存在其他自身免疫性疾病,应提示正在发展的神经综合征(认知、精神、行为和紧张症)具有自身免疫性质。在本报告中,我们描述了一名1型糖尿病青年男性的病例,该患者在出现紧张症后被诊断为血清学阴性自身免疫性脑炎。我们描述了其漫长的临床病程、各种治疗试验、临床结局以及对B细胞耗竭剂的反应。本研究还讨论了潜在的病理生理途径,为该病例中自身免疫性脑病的诊断检查和治疗选择提供了理论依据。