Verma Rajesh, Anand Rohit
Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India.
J Neurosci Rural Pract. 2020 Oct;11(4):658-660. doi: 10.1055/s-0040-1715997. Epub 2020 Sep 4.
Sjogren's syndrome (SS) is an autoimmune disorder characterized by lymphocytic and plasma cell infiltration of exocrine glands, resulting in dry mouth and keratoconjunctivitis sicca. The clinical symptoms may range from mucosal dryness to more systemic complaints. We report a 42-year-old man presenting with rapid cognitive decline and memory impairment for 2 months. Magnetic resonance imaging of the cranium revealed abnormal signals at the bilateral medial temporal lobe and hippocampal region. The autoimmune panel and paraneoplastic antibodies were negative. The patient was labeled as a case of primary SS based on the vasculitis profile and pathological examination of lip biopsy. This case illustrates a rare, heralding manifestation of SS as limbic encephalitis.
干燥综合征(SS)是一种自身免疫性疾病,其特征是外分泌腺出现淋巴细胞和浆细胞浸润,导致口干和干燥性角结膜炎。临床症状范围可从黏膜干燥到更多的全身性症状。我们报告一名42岁男性,出现快速认知衰退和记忆障碍2个月。头颅磁共振成像显示双侧内侧颞叶和海马区有异常信号。自身免疫指标和副肿瘤抗体均为阴性。根据血管炎特征和唇部活检的病理检查,该患者被诊断为原发性SS病例。本病例说明了SS作为边缘性脑炎一种罕见的先兆表现。