case_images: PMC11250063_01
case_images: Clinical timeline (created with BioRender.com). Arrow represents timeline of main clinical symptoms over 12 months. Interventions/treatments are represented below the arrow. AEDs, anti-epileptic drugs; IVMP, intravenous methylprednisolone; IVIG, intravenous immunoglobulin; mAb, monoclonal antibody; PLEX, plasma exchange; sxs, symptoms; TTMP, transtympanic methylprednisolone.
case_images: crn-2024-0016-0001-539186_F01.jpg
case_images: PMC11250063_01_crn-2024-0016-0001-539186_F01.jpg
case_images: F1
case_images: A previously healthy 40-year-old man of Algerian descent experiencing worsening headaches over 6-8 weeks followed by right eyelid twitching and mild confusion for 48 h was hospitalized abroad for generalized tonic-clonic status epilepticus (Fig. 1).
case_images: MRI evolution (2nd and 3rd relapse) in relation to treatment. Axial FLAIR sequences demonstrating the evolution over 8 months in relation to current therapeutic regimen. IVMP, intravenous methylprednisolone; IVIG, intravenous immunoglobulin; PLEX, plasma exchange; MMF, mycophenolate mofetil.
case_images: crn-2024-0016-0001-539186_F02.jpg
case_images: PMC11250063_01_crn-2024-0016-0001-539186_F02.jpg
case_images: F2
case_images: Brain MRI was stable (Fig. 2). CT-angiogram and spinal MRI were unremarkable, but brain MRI showed marked deterioration (Fig. 2). CSF was again normal including absence of oligoclonal bands, and a comprehensive panel for autoimmune/paraneoplastic Abs was ordered. AE in the context of anti-titin was suggested and pulse IVMP 1 g/day for 3 days was added to monthly IVIG. A repeat brain MRI 2 weeks later (Fig. 2) showed new cortical lesions but regression of one lesion. Brain MRI showed progression of multiple lesions with improvement of one lesion (Fig. 2). Follow-up MRI showed improvement of some lesions but evolution of others (Fig. 2). At 1-month and 3-month follow-up the patient demonstrated improvement of memory and language, as well as of left superior visual field at 3 month, and reported no recurrence of seizures or headaches, while MRI showed improvement and then near-complete resolution of lesions (Fig. 2).
case_images: Positivity for anti-GABAAR by mouse brain TIIF and CBA in our patient. Mouse brain tissue indirect immunofluorescence (TIIF) shows staining of (a) the cerebellum and (b) hippocampus compatible with anti-GABAAR. c Confirmatory positivity for anti-GABAAR by EUROIMMUN fixed cell-based assay (CBA).
case_images: crn-2024-0016-0001-539186_F03.jpg
case_images: PMC11250063_01_crn-2024-0016-0001-539186_F03.jpg
case_images: F3
case_images: Using CSF, TIIF staining compatible with anti-GABAAR was observed, and confirmatory serum and CSF testing for anti-GABAAR by EUROIMMUN fixed cell-based assay (CBA) was positive (Fig. 3).