Differences and diversity of autoimmune encephalitis in 77 cases from a single tertiary care center

Abstract

BackgroundThe incidence of autoimmune encephalitis has risen globally. There are two general categories of disease-associated antibodies that can be tested for: neuronal surface and intracellular. However, testing both groups of autoantibodies are costly. This study aims to identify differences between groups by comparing clinical presentations, radiological findings and CSF profile of patients, and determine if any parameters are indicative of one group of autoantibodies over another. Additionally, we aim to report the local incidence of less common groups of disease-associated antibodies as well.MethodsSeventy-seven records of autoimmune encephalitis/encephalomyelitis patients admitted to King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between October 2010 and February 2017 were reviewed. Patients with infections or those with classic central nervous system demyelinating features were excluded.ResultsOf 77 patients, 40% presented with neuronal surface antibodies and 33% had intracellular antibodies. The most common autoantibody detected in each group was anti-NMDAr antibody (25/31, 81%) and anti-Ri antibody (7/25, 28%) respectively. In the neuronal surface antibody group, behavioral change was the most common complaint (45%), followed by seizures (39%) and abnormal movements (29%). In the latter group, seizure was the most common presenting symptom (32%), followed by motor weakness (20%), behavioural change (16%) and abnormal movements (16%). Patients with neuronal surface antibodies were younger (35 vs 48 years old, p = 0.04) and more likely to present with behavioral change (45% vs 16%, p = 0.02). Mortality rate was higher in the intracellular group (16% vs 3.2%, p = 0.09). No differences were detected in magnetic resonance imaging (MRI) and CSF profile.ConclusionsIn the early stages of the disease, both groups have comparable clinical outcomes. Although there were significant differences in age and percentage of patients with behavioral change, both groups of autoimmune encephalitis still shared many clinical features and could not be distinguished based on MRI and CSF profiles. Therefore, we recommend that patients with features of autoimmune encephalitis should be screened for both the neuronal surface and intracellular antibodies regardless of clinical presentation.

Search for Viral Infections in Cerebrospinal Fluid From Patients With Autoimmune Encephalitis

“…In the current study, antineuronal autoantibodies were detected in 8 (4%) out of 200 patients with a clinical diagnosis of encephalitis, which is similar to the proportion reported for the United States (4.2%; based on anti-NMDAR) [ 38 ] and only slightly lower than that reported for Hungary (5.8%; based on anti-NMDAR, anti-LGI1, anti-Caspr2, anti-GABABR, anti-AMPAR1, and anti-AMPAR2) [ 39 ] and the United Kingdom (7.9%; based on anti-VGKC and anti-NMDAR) [ 40 ]. In our study, the vast majority of detected autoantibodies were anti-NMDR (87.5%), which is compatible with other reports; for example, in the study by Saraya et al [ 41 ], anti-NMDAR antibodies were present in 81% of AE cases with detectable neuronal surface antibodies.…”

Autoimmune Encephalitis in a Girl With Psychiatric Manifestations

“…AIE consists of a set of syndromes that are characterized by limbic and extra-limbic disorder manifestations, occurring due to the presence of antibodies. When broadly classified, two categories of antibodies cause AIE, which are neuronal surface and intracellular antibodies [ 6 , 7 ]. The most common neuronal autoantibody detected is the anti-N-methyl-D-aspartate receptor (NMDAr) antibody, and the most common intracellular antibody is the anti-Ri antibody [ 7 ].…”

“…When broadly classified, two categories of antibodies cause AIE, which are neuronal surface and intracellular antibodies [ 6 , 7 ]. The most common neuronal autoantibody detected is the anti-N-methyl-D-aspartate receptor (NMDAr) antibody, and the most common intracellular antibody is the anti-Ri antibody [ 7 ]. Other autoantibodies in the neuronal surface group consist of α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, contactin-associated protein-2, large glucagon immunoreactivity-1 (LGI-1) and gamma-aminobutyric acid receptor antibody [ 8 , 9 ].…”

Anti-SOX1 Antibodies in Paraneoplastic Neurological Syndrome

“… 19 Paired CSF and serum samples were available for only 17 patients with SOX1 reactivity. 6 20 21 25 55 59 61 63 65 Among them, 4 patients were negative for anti-SOX1 abs in the CSF, 21 25 while the other 13 patients showed positive anti-SOX1 abs reactivity in the CSF. 6 20 21 55 57 61 63 65 …”

“…Abnormal DNA methylation of the promoter region of SOX1 is a potential pathogenesis. 19,62 Notably, a small group (n=34, 6.5%) of the 520 patients with anti-SOX1 abs developed neurological symptoms without underlying tumors, 7,11,12,18,20,21,25,45,46,49,63 even after years of follow-up, but the mechanism that triggered the autoimmune response remains unknown. A possible explanation is that the tumor was too small to be detected or would have developed in the future.…”