Department of Psychiatry and Psychotherapy, University of Leipzig, Leipzig, Germany.
Department of Neurology, Rostock University Medical Center, Germany; Gehlsheimer Straße 20, 18147, Rostock, Germany.
BMC Psychiatry. 2021 Jul 15;21(1):355. doi: 10.1186/s12888-021-03269-0.
Anti-NMDA receptor encephalitis (NMDAR-E) is an autoimmune encephalitis (AE) mainly affecting young females. It typically presents with isolated psychiatric symptoms (e.g. depressed mood) at first and neurological abnormalities (e.g. seizures, movement disorders) only develop later. Thus, there is a high risk of overlooking NMDAR-E in patients with preexisting psychiatric illness due to symptom overlap in the prodromal period of the disease when treatment is most effective. Although rare, concomitant or sequential development of a demyelinating disorder is increasingly recognized as an associated disease entity (overlap syndrome), with immediate diagnostic and therapeutic implications.
We report a patient with a borderline personality disorder (BPD), which developed NMDAR-E and an overlapping demyelinating disorder with anti-Myelin oligodendrocyte glycoprotein (MOG) -IgG positivity. The initial clinical presentation with predominantly affective symptoms (e.g. mood lability, anxiety, depressed mood) lead us to suspect an exacerbation of the BPD at first. However, acute changes in premorbid behavior, newly developed psychotic symptoms and memory deficits lead us to the correct diagnosis of an AE, which was further complicated by the development of a demyelinating disorder. As a result of impaired illness awareness and psychosis, diagnostic and treatment was difficult to carry out. The symptoms completely remitted after treatment with methylprednisolone 1 g daily for 5 days and 5 cycles of plasma exchange.
Continuous awareness for neuropsychiatric clinical warning signs in patients with a pre-diagnosed psychiatric disorder is important for a timely diagnosis. Therefore, we believe that the diagnostic and therapeutic algorithm provided here, for the first time specifically addressing patients with preexisting psychiatric illness and integrating overlap syndromes, can be a useful tool. Moreover, in order to timely perform diagnostics and treatment, judicial approval should be obtained rapidly.
抗 N- 甲基-D- 天冬氨酸受体脑炎(NMDAR-E)是一种主要影响年轻女性的自身免疫性脑炎(AE)。它通常首先表现为孤立的精神症状(例如情绪低落),仅在疾病的前驱期后才出现神经异常(例如癫痫发作、运动障碍)。因此,由于疾病前驱期的症状重叠,对于存在先前精神疾病的患者,存在忽略 NMDAR-E 的高风险,因为此时治疗效果最佳。尽管罕见,但越来越多的人认识到并发或序贯发展的脱髓鞘疾病是一种相关疾病实体(重叠综合征),具有直接的诊断和治疗意义。
我们报告了一例患有边缘型人格障碍(BPD)的患者,该患者发展为 NMDAR-E 和重叠的脱髓鞘疾病,伴抗髓鞘少突胶质细胞糖蛋白(MOG)-IgG 阳性。最初的临床表现主要为情感症状(例如情绪不稳定、焦虑、情绪低落),导致我们最初怀疑是 BPD 恶化。然而,病前行为的急性变化、新出现的精神病症状和记忆缺陷使我们得出了 AE 的正确诊断,随后又并发了脱髓鞘疾病。由于对疾病的认识不足和精神病,诊断和治疗变得困难。经过每天 1g 甲基强的松龙治疗 5 天和 5 个周期的血浆置换后,症状完全缓解。
对于已经诊断出的精神疾病患者,持续关注神经精神临床警示症状对于及时诊断非常重要。因此,我们认为,这里首次提出的专门针对存在先前精神疾病的患者并整合重叠综合征的诊断和治疗算法,可以作为一种有用的工具。此外,为了及时进行诊断和治疗,应迅速获得司法批准。