Department of Immunology, Shifa International Hospital, Islamabad, Pakistan.
Department of Neurology, Combined Military Hospital, Lahore, Pakistan.
J Pak Med Assoc. 2024 Aug;74(8):1514-1517. doi: 10.47391/JPMA.10397.
A rare subtype of autoimmune encephalitis consists of antibodies targetting the alpha-amino-3-hydroxy-5- methyl-4-isoxazolepropionic acid receptor in the central nervous system. We describe the clinical presentation and autoimmune profile of the first case of alpha-amino-3- hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis with concurrent anti-acetylcholine receptor antibodies in Pakistan. The patient was a 58-year-old male who presented with the characteristic symptoms of limbic encephalitis with memory loss, irritability, agitation, and confusion. Antibodies against the alpha-amino-3-hydroxy- 5-methyl-4-isoxazolepropionic acid receptor were detected in both serum and cerebrospinal fluid by indirect immunofluorescence. Computerised tomography of the chest showed an anterior mediastinal mass. The patient was treated with high dose Methylprednisolone and five sessions of plasma exchange. There was a short period of improvement; however, the patient now continues to exhibit irritability, aphasia, confusion, and memory loss. Video-assisted thoracoscopic surgery for mediastinal mass resection and histological testing was planned, however after review by the interventional radiologist the associated risks were deemed too high to proceed with the procedure and biopsy was not done.
一种罕见的自身免疫性脑炎亚型由中枢神经系统中靶向α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体的抗体组成。我们描述了巴基斯坦首例同时伴有抗乙酰胆碱受体抗体的α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体脑炎的临床特征和自身免疫特征。该患者为 58 岁男性,表现为以记忆丧失、易激惹、激越和意识混乱为特征的边缘性脑炎。间接免疫荧光法在血清和脑脊液中均检测到针对 α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体的抗体。胸部计算机断层扫描显示前纵隔肿块。该患者接受了大剂量甲基强的松龙和五次血浆置换治疗。有短暂的改善期;然而,患者现在仍表现出易激惹、失语、意识混乱和记忆力丧失。计划进行胸腔镜手术切除纵隔肿块和组织学检查,但介入放射科医生审查后认为手术相关风险过高,无法进行该手术,也未进行活检。