A Case of Acute Limbic Encephalitis with Sjögren's Syndrome

• Published in: Brain and nerve = Shinkei kenkyū no shinpo Vol. 68; no. 5; p. 567
• Main Authors: Shinmura, Hiroyuki, Nagasaka, Takamura, Tsuchiya, Mai, Onohara, Akiko, Takaki, Ryusuke, Takiyama, Yoshihisa
• Format: Journal Article
• Language: Japanese
• Published: Japan 01.05.2016
• Subjects: Acute Disease; Female; Hippocampus - pathology; Humans; Limbic Encephalitis - diagnosis; Limbic Encephalitis - drug therapy; Limbic Encephalitis - etiology; Limbic Encephalitis - pathology; Magnetic Resonance Imaging; Middle Aged; Sjogren's Syndrome - complications; Sjogren's Syndrome - drug therapy; Sjogren's Syndrome - pathology; Temporal Lobe - pathology
• ISSN: 1881-6096
• DOI: 10.11477/mf.1416200439

A 52-year-old woman developed abnormal behavior and disturbance of consciousness subsequent to several days with a cold. On admission, she was very confused, with incoherent speech, and an inability to recognize family faces. Diffusion weighted MRI showed high intensity signal change in the bilateral medial temporal lobes, including the hippocampus. Cerebrospinal fluid examination was normal. Tests including various viral antibody titers provided no evidence of infection. Several neuronal antibodies including anti-VGKC and -NMDA receptor antibody were absent. Evidence of malignancy was not apparent. She was diagnosed with acute limbic encephalitis complicated by Sjögren's syndrome (SjS), due to the fact that she had a past history of SjS, elevation of anti-SS-A antibody, pleuritis and pericarditis. Her symptoms gradually improved after administration of steroids including pulse therapy; however, her amnesia remained for a long time. In diagnosing acute limbic encephalitis, we should consider SjS as an underlying disease, even though it is rare.