Optic Nerve Atrophy in N-methyl-D-aspartate (NMDA) Encephalitis

Abstract

N-methyl-D-aspartate receptor (NMDA) encephalitis is a recently described autoimmune disease that typically presents with prodromal symptoms including upper respiratory tract infection, headache, fever, nausea, vomiting and diarrhea. Psychiatric symptoms follow within weeks, including anxiety, insomnia, mania, paranoia and grandiose delusions. The diagnosis is confirmed by the detection of NMDA antibodies in the serum or cerebrospinal fluid (CSF).1 Tumours, especially teratomas, are frequently associated with NMDA encephalitis; however, only 5% of male patients older than 18 years have been found to have an underlying tumour. Optic neuropathy associated with NMDA encephalitis is being increasingly recognised in the literature2–6 and was reviewed most recently by Mugavin et al.2 in 2017. In this report, we present a case of bilateral optic neuropathy in a young man diagnosed with NMDA receptor encephalitis.

Abnormal sensory perception masks behavioral performance of Grin1 knockdown mice

“…A substantial percentage of GRIN1 patients have cortical visual impairment, and we hypothesized that Grin1KD mice might be similarly impaired. Not only are NMDARs expressed in the retina, they are required for the retinotopic organization of the lateral geniculate nucleus and visual cortex 59–63 . Thus, it is not surprising that GRIN disorder patients experience CVI or that mouse models of GRIN disorder would have abnormal vision.…”

“…Not only are NMDARs expressed in the retina, they are required for the retinotopic organization of the lateral geniculate nucleus and visual cortex. 59 , 60 , 61 , 62 , 63 Thus, it is not surprising that GRIN disorder patients experience CVI or that mouse models of GRIN disorder would have abnormal vision. We assessed visual depth perception with the visual cliff test developed to detect aberrant vision in mice.…”