AUTHOR=Wang Yan , Yu Yi , Hu Yaping , Li Ying , Song Fan , Wang Ying 

TITLE=Clinical and Electroencephalographic Features of the Seizures in Neuronal Surface Antibody-Associated Autoimmune Encephalitis

JOURNAL=Frontiers in Neurology

VOLUME=11

YEAR=2020

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2020.00280

DOI=10.3389/fneur.2020.00280

ISSN=1664-2295

ABSTRACT=<p><bold>Objectives:</bold> To investigate clinical and electroencephalographic features of the seizures in different types of neuronal surface antibody (NSAb)-associated autoimmune encephalitis (AE).</p><p><bold>Methods:</bold> The clinical data of the seizures were analyzed in 18 patients with NSAb-associated AEs diagnosed in the First Affiliated Hospital of Dalian Medical University.</p><p><bold>Results:</bold> From May 2013 to April 2019, a total of 18 cases of NSAb-associated AE were diagnosed, including 9 cases of leucine-rich glioma-inactivated 1 protein (LGI1) antibody-associated encephalitis, 7 cases of anti-<italic>N</italic>-methyl-d-aspartate receptor (NMDAR) encephalitis, and 2 cases of anti-γ-aminobutyric acid B receptor (GABA<sub>B</sub>R) encephalitis. All nine cases (100%) with LGI1 AE had seizures manifesting in three types: faciobranchial dystonia seizure (FBDS) (44.4%), mesial temporal lobe epilepsy (MTLE)-like seizure (66.7%), and focal to bilateral tonic–clonic seizure (FBTCS) (77.8%). Six of nine (66.7%) showed abnormal signal on hippocampus or basal ganglia in brain MRI. Five of seven cases (71%) with anti-NMDAR encephalitis had seizures manifesting in three types: focal aware seizure (40%), focal-impaired awareness seizure (20%), generalized tonic–clonic seizure (GTCS) (100%), and status epilepticus (SE) (40%). Three of seven (42.8%) showed abnormalities in brain MRI. Both patients with anti-GABA<sub>B</sub>R encephalitis had seizures manifesting in two types: GTCS and MTLE-like seizure, one with SE. One showed abnormal signal on left hippocampus in brain MRI. All patients (100%) with three types of AE had abnormalities in electroencephalogram (EEG), showing diffuse (4/18) or focal slow waves (14/18) in background, interictal (10/18), or ictal (6/18) epileptic discharges in the temporal or other regions; two patients with anti-NMDAR encephalitis showed delta activity or rhythm in frontotemporal region. All patients with seizures showed good response to immunotherapy except one with LGI1 AE.</p><p><bold>Conclusions:</bold> Most patients with NSAb-associated AE had seizures; seizure types varied between different types of AE. In LGI1 AE, the hippocampus and basal ganglia were two main targets; the corresponding seizure type was MTLE-like seizure and FBDS, respectively. Anti-NMDAR encephalitis had more generalized than focal seizures. Delta activity or rhythm in the frontotemporal region in EEG was helpful for diagnosis. Anti-GABA<sub>B</sub>R encephalitis was characterized by refractory seizures as initial symptom, mainly GTCS or MTLE-like seizure. Most seizures in NSAb-associated AE showed good response to immunotherapy, and antiepileptic drugs should be considered as an add-on symptomatic treatment.</p>