Kachlmeier André, Adams Rolf, Zahalka Tobias
St. Katharinen-Hospital Frechen, Department of Neurology, Kapellenstraße 1-6, 50226 Frechen, Germany.
Epilepsy Behav Rep. 2023 Aug 12;24:100618. doi: 10.1016/j.ebr.2023.100618. eCollection 2023.
Anti-NMDA receptor (Anti-NMDAR) encephalitis is an autoimmune disease that presents with diverse symptoms. Since literature is scarce on the overlap with multiple sclerosis (MS), this report aims to elucidate the distinctive clinical presentation and diagnostic challenges of anti-NMDAR encephalitis in MS patients. A 73-year-old woman with secondary progressive multiple sclerosis, after experiencing status epilepticus and subsequent non-convulsive status epilepticus, presented with neuropsychiatric symptoms and autonomic nervous dysfunction. Notably, the patient had not received any immunomodulatory therapy. The clinical picture together with diagnostics (MRI, EEG, cerebro-spinal fluid) let us suspect HSV-meningoencephalitis and empirically treat the patient with IV acyclovir. Due to a lack of clinical improvement, we reconsidered the diagnosis and found the diagnostic criteria for autoimmune encephalitis to be met. Antibodies in blood and CSF were positive and we diagnosed the patient with anti-NMDAR encephalitis. The patient responded well to IV prednisolone treatment, leading to a stable outcome in a six-month follow-up. This case highlights the difficulties in diagnosing anti-NMDAR encephalitis in patients with multiple sclerosis. The presence of epileptic seizures can serve as a crucial diagnostic indicator to distinguish between an MS relapse and an overlapping disease. Compared to patients with other demyelinating diseases, patients with overlapping MS appear to have a higher risk of motor seizures.
抗N-甲基-D-天冬氨酸受体(Anti-NMDAR)脑炎是一种具有多种症状的自身免疫性疾病。由于关于其与多发性硬化症(MS)重叠的文献较少,本报告旨在阐明MS患者中抗NMDAR脑炎独特的临床表现和诊断挑战。一名73岁患有继发进展型多发性硬化症的女性,在经历癫痫持续状态及随后的非惊厥性癫痫持续状态后,出现神经精神症状和自主神经功能障碍。值得注意的是,该患者未接受任何免疫调节治疗。结合临床表现及诊断检查(MRI、脑电图、脑脊液检查),我们怀疑为单纯疱疹病毒性脑膜脑炎,并经验性地给予患者静脉注射阿昔洛韦治疗。由于临床症状无改善,我们重新考虑诊断,发现符合自身免疫性脑炎的诊断标准。血液和脑脊液中的抗体呈阳性,我们诊断该患者为抗NMDAR脑炎。患者对静脉注射泼尼松龙治疗反应良好,在六个月的随访中病情稳定。该病例凸显了在多发性硬化症患者中诊断抗NMDAR脑炎的困难。癫痫发作的出现可作为区分MS复发和重叠疾病的关键诊断指标。与其他脱髓鞘疾病患者相比,重叠MS的患者似乎有更高的运动性癫痫发作风险。