Department of Neurology, National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez", Mexico City, Mexico.
Neuropsychiatry Unit, National Institute of Neurology and Neurosurgery "Manuel Velasco Suárez", Mexico City, Mexico.
Cogn Behav Neurol. 2024 Sep 1;37(3):154-164. doi: 10.1097/WNN.0000000000000375.
The clinical features of neuropsychiatric systemic lupus erythematosus (NPSLE) are heterogeneous. Furthermore, therapeutic decision-making for NPSLE depends on the recognition of clinical syndromes that have not been sufficiently studied. This report describes the case of a 36-year-old woman with NPSLE who exhibited severe cognitive dysfunction and affective psychosis with persistent nihilistic delusions such as those described in the Cotard delusion. The patient insisted for several months that she was already dead. CSF analysis showed elevated levels of anti-ribosomal P antibodies and a positive determination of oligoclonal bands. Additionally, 18F -FDG PET/CT imaging revealed severe bilateral frontal hypermetabolism suggestive of brain inflammation and occipital hypometabolism. Results from the Systematic Lupus Erythematosus Disease Activity Index 2000 and the Systemic Lupus Erythematosus Disease Activity Score were consistent with an active state of the immunological disease. We then determined by an algorithm that this neuropsychiatric event could be attributed to the activity of the underlying immunological disease. Despite immunosuppressive and symptomatic treatment, only a partial improvement in cognition was achieved. The psychopathological features of the Cotard delusion remained unchanged 4 months after onset. However, we observed rapid remission of affective psychosis and significant improvement in cognition following electroconvulsive therapy. Subsequent follow-up examinations showed a sustained remission. This case describes a protracted form of the Cotard delusion, the diagnostic challenges that arise in the context of SLE, and treatment dilemmas that necessitate collaboration between neurology, psychiatry, and rheumatology.
神经精神性狼疮(NPSLE)的临床特征具有异质性。此外,NPSLE 的治疗决策取决于对尚未充分研究的临床综合征的认识。本报告描述了一例 36 岁的 NPSLE 女性患者,其表现为严重的认知功能障碍和情感性精神病,伴有持续的虚无妄想,如科塔尔妄想所描述的那样。该患者坚持认为自己已经死亡,持续了数月。CSF 分析显示抗核糖体 P 抗体水平升高,寡克隆带阳性。此外,18F-FDG PET/CT 成像显示双侧额叶严重代谢亢进,提示存在脑炎症和枕叶代谢低下。系统性红斑狼疮疾病活动指数 2000 版和系统性红斑狼疮疾病活动评分的结果与免疫性疾病的活动状态一致。我们随后通过一种算法确定,这种神经精神事件可能与潜在免疫性疾病的活动有关。尽管进行了免疫抑制和对症治疗,但认知功能仅部分改善。科塔尔妄想的精神病理学特征在发病后 4 个月仍未改变。然而,我们观察到电休克治疗后情感性精神病迅速缓解,认知功能显著改善。随后的随访检查显示持续缓解。本病例描述了一种持续时间较长的科塔尔妄想,以及在 SLE 背景下出现的诊断挑战和需要神经病学、精神病学和风湿病学之间合作的治疗困境。