Abstract
BackgroundProspective observations of functional recovery are lacking in patients with autoimmune encephalitis defined by antibodies against synaptic proteins and neuronal cell surface receptors.MethodsAdult patients with a diagnosis of autoimmune encephalitis were included into a prospective registry. At 3, 6 and 12 months of follow-up, the patients’ modified Rankin Scale (mRS) was obtained.ResultsPatients were stratified into three groups according to their antibody (Ab) status: anti-NMDAR-Ab (n=12; group I), anti-LGI1/CASPR2-Ab (n=35; group II), and other antibodies (n=24; group III). A comparably higher proportion of patients in group I received plasma exchange/immunoadsorption and second line immunosuppressive treatments at baseline. A higher proportion of patients in group II presented with seizures. Group III mainly included patients with anti-GABABR-, anti-GAD65- and anti-GlyR-Ab. At baseline, one third of them had cancer. Patients in groups I and III had much higher median mRS scores at 3 months compared to patients in group II. A median mRS of 1 was found at all follow-up time points in group II.ConclusionsThe different dynamics in the recovery of patients with certain autoimmune encephalitides have important implications for clinical trials. The high proportion of patients with significant disability at 3 months after diagnosis in groups I and III points to the need for improving treatment options. More distinct scores rather than the mRS are necessary to differentiate potential neurological improvements in patients with anti-LGI1-/CASPR2-encephalitis.
Highlights
Autoimmune encephalitides defined by antibodies against synaptic proteins and neuronal cell surface receptors have emerged as specific diagnostic entities [1,2,3]
Patients with antibodies against neuronal cell surface proteins and the synaptic protein GAD65 in serum and/or CSF were identified at baseline as follows: 12 (16.9%) antiNMDAR-Ab, 24 (33.8%) anti-LGI1-Ab, 10 (14.1%) anti-CASPR2Ab, 1 (1.4%) anti-LGI1-Ab and anti-CASPR2-Ab, 1 (1.4%) antiAMPAR-Ab, 1 (1.4%) anti-GABAAR-Ab, 5 (7.0%) anti-GABABRAb, 9 (12.7%) anti-GAD65-Ab, 1 (1.4%) anti-GABABR-Ab and anti-GAD65-Ab, 7 (9.9%) anti-GlyR-Ab
Patients were stratified according to their antibody status: one group of patients with anti-NMDAR-Ab (n=12), one with antiLGI1/CASPR2-Ab (n=35), and one with all other antibodies (n=24)
Summary
Autoimmune encephalitides defined by antibodies against synaptic proteins and neuronal cell surface receptors have emerged as specific diagnostic entities [1,2,3]. Immunosuppressive treatments improve the patients’ clinical outcome based on uncontrolled observations [4,5,6]. A small randomized trial has shown superiority of intravenous immunoglobulins (IVIG) to placebo in reducing seizure frequency in anti-leucine-rich, glioma-inactivated 1-antibody (anti-LGI1-Ab) and anti-contactin-associated protein-like 2antibody (anti-CASPR2-Ab) seropositive adult patients [7]. We have set up a multicenter prospective observational study for a structured assessment of diagnostics and treatments applied in clinical routine in adult patients with autoimmune encephalitis. Prospective observations of functional recovery are lacking in patients with autoimmune encephalitis defined by antibodies against synaptic proteins and neuronal cell surface receptors
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