Clinical characteristics of leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis

Abstract

Objective To analyze the clinical characteristics of leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis. Methods Fourteen patients diagnosed as leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis in Capital Medical University Xuanwu Hospital from January 2012 to January 2015 were recruited. The clinical manifestation, brain magnetic resonance imaging, cerebrospinal fluid findings and biochemical examination of these patients were analyzed. Results The case series of 14 patients had an average age of (48.93±15.60) years (range 27-67 years) with a male to female ratio of 2.5∶1. All patients presented with short-term memory loss. Nine patients experienced dementia. Additionally, among the 14 patients, 12 experienced seizures, 8 experienced faciobrachial dystonic seizures, 10 had psychiatric symptoms, and 8 showed sleep dysfunction. Two patients were transferred into intensive care unit because of deteriorating symptoms and were provided operated mechanical ventilation. Thirteen of 14 patients exhibited abnormalities in their brain magnetic resonance imaging, with lesions in temporal lobe and hippocampus. Six patients had abnormal cerebrospinal fluid findings, 8 patients showed hyponatremia (serum Na+ <135 mmol/L), while 5 patients co-existed with other autoantibodies. Screening for malignant tumors revealed normal findings. During 2 years follow-up, 3 patients relapsed. Conclusions Leucine-rich glioma-inactivated protein 1 antibody-associated encephalitis is an autoimmune encephalitis characterized by short-term memory loss, faciobrachial dystonic seizures and hyponatremia. Lesions in brain MRI always involve in temporal lobe and hippocampus. This disease can relapse and is seldom associated with tumor. Key words: Autoimmune diseases of the nervous system; Encephalitis; Proteins; Magnetic resonance imaging; Recurrence