Department of Neurology, Jubilee Mission Medical College Hospital and Research Institute, Thrissur, Kerala, India.
Department of Neurology, Renai Medicity Multi Super Speciality Hospital, Kochi, Kerala, India.
Pan Afr Med J. 2022 Jan 19;41:52. doi: 10.11604/pamj.2022.41.52.28790. eCollection 2022.
Limbic encephalitis is often due to an autoimmune or paraneoplastic disease and is always a diagnostic challenge. We report a 31-year-old lady who presented with fever and proximal weakness to start with and afterwards developed refractory focal onset seizures and worsening cognition despite optimum treatment. Evaluation revealed hypokalemia with a normal anion gap metabolic acidosis. Magnetic resonance imaging (MRI) brain showed features of limbic encephalitis. Cerebrospinal fluid (CSF) showed lymphocytosis and CSF autoimmune, paraneoplastic and viral encephalitis panel were negative. However a blood ANA profile clinched the diagnosis when SS-A and Ro 52 were strongly positive. She was given steroids and subsequently plasma exchange. A labial gland biopsy confirmed the diagnosis of Sjögren syndrome. In cases of autoimmune limbic encephalitis with no identifiable cause, serological screening for rheumatological disorders is recommended. Sjögren syndrome is a rare aetiology for autoimmune limbic encephalitis. A detailed history and a step wise approach is always the key to the right diagnosis.
边缘性脑炎常由自身免疫或副肿瘤性疾病引起，始终是一项诊断挑战。我们报告了一位 31 岁的女性患者，最初表现为发热和近端肌无力，随后尽管接受了最佳治疗，但仍出现难治性局灶性发作性癫痫和认知功能恶化。检查发现低钾血症合并正常阴离子间隙代谢性酸中毒。脑部磁共振成像（MRI）显示边缘性脑炎的特征。脑脊液（CSF）显示淋巴细胞增多，CSF 自身免疫、副肿瘤和病毒性脑炎检测均为阴性。然而，当抗核抗体（ANA）谱显示 SS-A 和 Ro 52 强阳性时，确诊了该病。她接受了类固醇和随后的血浆置换治疗。唇腺活检证实了干燥综合征的诊断。对于原因不明的自身免疫性边缘性脑炎，建议进行风湿性疾病的血清学筛查。干燥综合征是一种罕见的自身免疫性边缘性脑炎病因。详细的病史和逐步的方法始终是正确诊断的关键。
