Anti-N-Methyl-D-Aspartate Receptor Encephalitis With Diffuse Demyelinating Plaques: A Case Report of an Atypical Presentation

Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an autoimmune disease triggered by antibodies against the NR1 subunit of this receptor. It has a wide variety of presentations, including abnormal behavior, psychosis, seizures, abnormal movement, insomnia, and irritability. The diagnosis is confirmed by the presence of one of the six main symptoms and anti-NR1 immunoglobulin G (IgG)-positive antibodies in the cerebrospinal fluid (CSF) after the exclusion of other disorders. We present a case of an 18-year-old female with progressive paresthesia and muscle weakness that compromised walking and psychiatric symptoms. She was admitted to a private institution where magnetic resonance imaging (MRI) revealed pseudotumoral lesions, which led to surgical intervention. The original histopathological diagnosis was of a pleomorphic xanthoastrocytoma (PXA) WHO grade 2. As symptoms persisted, she was referred to our institution where a new MRI was performed, and a biopsy was re-evaluated. It showed perivascular inflammatory infiltrates composed of T cells, intense peripheral gliosis, nodules of macrophages, and reactive astrocytes in the white matter with fragmentation and vacuolation of myelin sheets, suggesting a demyelinating process in contrast to neoplasia. CSF analysis was performed, and it was positive for anti-NMDA antibodies. Immunohistochemical positivity for N-methyl-D-aspartate (NMDA) was observed in the neuronal nuclei, which led to the diagnosis.