Abstract
BackgroundA unified clinical approach to diagnose autoimmune encephalitis was published in Lancet Neurology in 2016. Purpose of our study is to examine the feasibility and reasonability of the 2016 “A clinical approach to diagnosis of autoimmune encephalitis” in China with a retrospective study.MethodsWe retrospectively collected 95 cases of autoimmune encephalitis and non autoimmune encephalitis cases with detailed clinical data from Beijing Tongren Hospital and the China National Knowledge Infrastructure (CNKI). All cases were analysed stepwise according to the approach in Lancet Neurology to compare the new diagnosis with the final clinical diagnosis.ResultsThe disease course of these 95 cases ranged from 2 to 540 days. Initial symptoms include fever, headache, seizure, mental and behavioral disorders, memory deterioration and illusion. Based on symptoms and signs when the patient came to the hospital, the sensitivity and specificity of criteria were as follows: possible autoimmune encephalitis (pAE) 84% and 94%, definite autoimmune limbic encephalitis (dALE) 38% and 96%, probable anti-N-methyl-D-aspartate receptor encephalitis (prNMDARE) 49% and 98%. The sensitivities of the above three criteria and the specificity of pAE were low during early disease stage, while the specificities of dALE and prNMDAER remained relatively high in different time periods.ConclusionsThis new autoimmune encephalitis diagnostic approach can recognize possible autoimmune encephalitis. The chances of a case being autoimmune-mediated following classification as autoimmune encephalitis with the new criteria are high. The flowchart is recommended to use as a whole. At the early disease stage, criteria with low sensitivity and high specificity, such as dALE and prNMDARE, lead most cases to enter subsequent diagnosis steps, namely autoantibody detection in the flowchart. Final diagnoses can only be made by autoantibody tests. These factors may make it challenging for clinicians to make diagnosis promptly and to begin immune-modulating therapy immediately. Moreover, the criteria for patients with paraneoplastic syndromes (PNSs) should be considered to avoid diagnosis omission. For Chinese patients, a multi-centre, prospective study on the clinical manifestations, laboratory diagnostic technology, therapy, and prognosis is greatly needed.
Highlights
A unified clinical approach to diagnose autoimmune encephalitis was published in Lancet Neurology in 2016
There were 64 autoimmune encephalitis cases, and 39 cases were diagnosed as anti-NMDA receptor encephalitis, 13 cases as leucine-rich, glioma inactivated 1 (LGI1) Ab(+)-associated limbic encephalitis, 6 cases as gamma-aminobutyric acid-B receptor (GABAbR) Ab(+)-associated limbic encephalitis, 1 case as α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) Ab(+)-associated limbic encephalitis, 1 case as Ma2 Ab(+)-associated limbic encephalitis, 2 cases as paraneoplastic limbic encephalitis, 1 case as autoimmune limbic encephalitis with negative autoantibody, and 1 case as Bickerstaff’s brainstem encephalitis
Among the remaining 43 cases, 16 cases were diagnosed as probable autoimmune encephalitis, the other 27 cases only can be diagnosed by antibody tests
Summary
A unified clinical approach to diagnose autoimmune encephalitis was published in Lancet Neurology in 2016. The discovery of autoimmune antibodies targeting proteins in the brain has been applied to the diagnosis of encephalitis, psychiatric disease, seizures, dyskinesia and cognitive impairment. As early as the 1960s, limbic encephalitis was described as a subacute onset of episodic memory loss, confusion and agitation. Neurologists proposed that this disease was typically associated with neoplasms. Antibodies against nuclear and cytoplasmic proteins, such as Hu, Ma, and Ri, represent specific malignancies. Novel autoantibodies against cell surface or synaptic proteins can alter the function, structure, or density of neuronal antigens, causing nervous system dysfunction. Early identification and diagnosis of this disease are very important
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