Rasmussen-Like Asymmetric Cortical Atrophy in Anti-NMDA Receptor Encephalitis
An 18-year-old man presented with 1 week of insomnia and headache, followed by erratic behavior, encephalopathy, seizures, and left-sided weakness. Brain MRI demonstrated cortical T2/FLAIR hyperintensity predominantly involving the right cerebral hemisphere, followed by progressive right-greater-than-left cortical volume loss over 2 months. FDG-PET showed extensive hypometabolism, predominantly affecting the right cerebral hemisphere and thalami. CSF revealed lymphocyte-predominant pleocytosis (87 cells/μL), with normal protein and glucose. Anti-NMDA receptor IgG was positive in serum and CSF by cell-based assay (titer not available). Body FDG-PET was negative for malignancy. He was treated with intravenous corticosteroids, immunoglobulin, and rituximab. At 6-month follow-up, he followed simple commands, walked independently with a spastic gait, and was seizure-free on 2 anti-seizure medications. Repeat serum anti-NMDA receptor IgG remained positive at 1:40. While asymmetric cortical atrophy is classically associated with Rasmussen encephalitis, autoimmune encephalitis can rarely have a similar radiographic appearance. The presence of milder contralateral imaging abnormalities and persistent anti-NMDA receptor seropositivity after clinical stabilization supports an atypical presentation of anti-NMDA receptor encephalitis. This case expands the literature on Rasmussen-like autoimmune encephalitis and potential overlap syndromes.
Authors
- Yoji Hoshina (ORCID: https://orcid.org/0000-0003-0228-664X)
- Otto Rapalino
- Jenny Linnoila
Institutions
- Harvard University (US)
- University of Pittsburgh (US)
- University of Pittsburgh Medical Center (US)
- Mass General Brigham (US)
Publication Details
- Journal
- The Neurohospitalist
- Published
- 2026-09-11
- DOI
- https://doi.org/10.1177/19418744261489707
- Primary Topic
- Autoimmune Neurological Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00