PMC9465088_01
Schema for the disease course, treatments, and MRI manifestations of anti-GABAAR encephalitis in a 29-year-old woman. (A-F) Clinical manifestations and treatment options corresponding to each time point in the course of the disease. AEDs, anti-epilepsy drugs; AZP, azathioprine; CTX, cyclophosphamide; IVIG, intravenous immunoglobin; MTP, methylprednisolone.
fneur-13-954494-g0001.jpg
PMC9465088_01_fneur-13-954494-g0001.jpg
F1
MRI of the brain using fluid-attenuated inversion recovery (FLAIR) revealed multiple, asynchronous, cortical-subcortical abnormalities in the frontal, temporal, parietal, occipital, and insular lobes, and mismatched cerebrovascular distribution (Figure 1).
EEG manifestations at the time of right arm paroxysmal clonus occurrence (A) and after intravenous midazolam (B).
fneur-13-954494-g0002.jpg
PMC9465088_01_fneur-13-954494-g0002.jpg
F2
Electroencephalography (EEG) demonstrated periodic epileptiform discharges in chains lasting for 2 min in the left frontal region when right arm paroxysmal clonus occurred (Figure 2A), which was nearly synchronously attenuated by intravenous midazolam (Figure 2B) A second test was performed on August 12, 2020, for antibodies in the plasma and CSF against GABAAR alpha1/beta3 and gamma2 subunits using live HEK293 cells expressing alpha1/beta3/gamma2 subunit and was positive (Figure 3).
Reactivity of the patient's serum (A) and cerebrospinal fluid (CSF) (B) with live HEK293 cells expressing human alpha1/beta3/gamma2 subunits of GABAAR.
fneur-13-954494-g0003.jpg
PMC9465088_01_fneur-13-954494-g0003.jpg
F3
Electroencephalography (EEG) demonstrated periodic epileptiform discharges in chains lasting for 2 min in the left frontal region when right arm paroxysmal clonus occurred (Figure 2A), which was nearly synchronously attenuated by intravenous midazolam (Figure 2B) A second test was performed on August 12, 2020, for antibodies in the plasma and CSF against GABAAR alpha1/beta3 and gamma2 subunits using live HEK293 cells expressing alpha1/beta3/gamma2 subunit and was positive (Figure 3).