Teaching NeuroImages: NMDA encephalomyelitis with MRI abnormalities isolated to ventral spinal cord gray matter

A 17-year-old girl presented with a month of anxiety followed by acute psychosis, catatonia, choreoathetosis, seizures, autonomic instability, lower extremity spasticity, and hyperreflexia. CSF was positive for the NMDA antibody; additional antibodies and infectious etiologies tested negative. MRI brain was normal. MRI spine revealed symmetric T2 hyperintensities in ventral gray matter (figure). EEG showed extreme delta brush. She received the following sequentially: IV gammaglobulin, methylprednisolone, ovarian teratoma resection, and plasmapheresis, only improving after rituximab. At 3 months, repeat MRI spine and motor examination results were normal. NMDA encephalitis with myelopathy is rare(1,2); spinal cord gray matter involvement has not been reported previously.