Abstract
Autoimmune encephalitis (AE) is one kind of encephalitis that associates with specific neuronal antigens. Most patients with AE likely suffer from seizures, but data on the characteristics of seizure and antiepileptic drugs (AEDs) utilization in this patient group remains limited. This study aimed to report the clinical status of seizure and AEDs treatment of patients with AE, and to evaluate the relationship between AEDs discontinuation and seizure outcomes. Patients with acute neurological disorders and anti-N-methyl-D-aspartate receptor (NMDAR), γ-aminobutyric acid B receptor (GABABR), leucine-rich glioma inactivated 1, or contactin-associated protein-like 2 (CASPR2) antibodies were included. As patients withdrew from AEDs, they were divided into the early withdrawal (EW, AEDs used ≤3 months) and late withdrawal (LW, AEDs used >3 months) groups. Seizure remission was defined as having no seizures for at least 1 year after the last time when AEDs were administered. Seizure outcomes were assessed on the basis of remission rate. The factors affecting the outcomes were assessed through Spearman analysis. In total, we enrolled 75 patients (39 patients aged <16 years, male/female = 39/36) for follow-up, which included 67 patients with anti-NMDAR encephalitis, 4 patients with anti-GABABR encephalitis, 2 patients with anti-voltage-gated potassium channel encephalitis, and 2 patients with coexisting antibodies. Among the 34 enrolled patients with anti-NMDAR encephalitis who were withdrawn from AEDs, only 5.8% relapse was reported during the 1-year follow-up, with no significant difference in the percentage of relapse between the EW and LW groups (P = 0.313). Fifteen patients (an average age of 6.8, 14 patients with anti-NMDAR encephalitis and 1 patient with anti-CASPR2 encephalitis) presented seizure remission without any AEDs. Seventy five percent of patients with anti-GABABR antibodies developed refractory seizure. Other risk factors which contributed to refractory seizure and seizure relapse included status epilepticus (P = 0.004) and cortical abnormalities (P = 0.028). Given this retrospective data, patients with AE have a high rate of seizure remission, and the long-term use of AEDs may not be necessary to control the seizure. Moreover, seizures in young patients with anti-NMDAR encephalitis presents self-limited. Patients with anti-GABABR antibody, status epilepticus, and cortical abnormalities are more likely to develop refractory seizure or seizure relapse.
Highlights
Autoimmune encephalitis (AE) is kind of encephalitis which associates with humoral or cellular responses against specific neuronal antigens [1, 2]
Among the 75 patients, 12 continued taking antiepileptic drugs (AEDs), 15 were not given any AEDs, and 37 patients who withdrew from AEDs were followed up for at least 1 year (Figure 1)
To evaluate the AEDs utilization associated with AE, we focused on seizure in a cohort of patients with anti-N-methyl-D-aspartate receptor (NMDAR), antiGABABR, anti-leucinerich glioma inactivated 1 (LGI1), and contactin-associated protein-like 2 (CASPR2) encephalitis
Summary
Autoimmune encephalitis (AE) is kind of encephalitis which associates with humoral or cellular responses against specific neuronal antigens [1, 2]. Despite the severity of the disease in acute phase, most patients recover after proper immunotherapy and intensive support [4]. Seizure is a highly prevalent symptom, and parts of patients develop status epilepticus (SE) [5,6,7]. Multiple anti-epileptic drugs (AEDs) are often necessary to control the attacks. Based on the previous studies and data from our center, most patients likely recover completely after adequate immunotherapy, and seizure is rarely reported in the chronic phase [5, 8, 9]. An instructional database describing the long-term use of AEDs with AE is lacking
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