Neuro-Ophthalmic Features of Autoimmune Encephalitides

Abstract

Background: Over the past decade, there has been a remarkable advancement in the understanding of autoimmune etiologies of encephalitis. The first identified generation of paraneoplastic encephalitis tends to occur in older populations, responds poorly to immunotherapy, and is mediated by T-cell damage with antibodies directed toward intracellular antigens. A new generation of autoimmune encephalitides has been described, which are mediated by antibodies to cell-surface proteins, tend to occur in younger individuals, are less frequently associated with malignancy, and often respond better to treatment compared to their intracellular antigen-related paraneoplastic counterparts. This review will focus on several specific antibody-mediated autoimmune encephalitides with neuro-ophthalmic pertinence. Evidence Acquisition: Literature review and personal clinical experience. Results: Several of the antibody-mediated encephalitides, specifically N-methyl-D-aspartate receptor, dipeptidyl-peptidase-like protein 6, glial fibrillary acidic protein, metabotropic glutamate receptor 1 (mGluR1), gamma-aminobutyric acid receptor, glutamic acid decarboxylase 65 (GAD65), collapsing response mediator protein 5 (CRMP5), and kelch-like protein 11 (KLHL11), contain features of neuro-ophthalmic interest. Conclusions: The novel cell-surface protein-directed autoimmune encephalitis group can present with a wide range of afferent and efferent neuro-ophthalmic manifestations. Neuro-ophthalmologists should be familiar with these antibody-associated syndromes, which are treatable and often require a high index of suspicion for diagnosis.

Anti Zic4 Paraneoplastic Neurological Syndrome Presenting as Oscillopsia

“…Oscillopsia has been described in PNS caused by anti DPPX, anti NMDAR, anti GAD65, mGluR1 and anti KLHL11 antibodies though not as the initial feature 3 as in our unique case. Pursuit eye movements checked in the same direction (Video segment 1) of head movements (ie, following a head‐fixed target) require inhibition of the vestibulo‐ocular reflex (VOR), mediated by a class of vestibular neurons namely floccular‐target neurons (FTN) which have substantial input from the floccular complex of the cerebellum 4 .…”

Neuro-Ophthalmic Features of Autoimmune Encephalitides: Letter to the Editor

“…Encephalitides: Letter to the Editor I enjoyed reading the recent article regarding the neuroophthalmic features of autoimmune encephalitides (1). In 2018, I evaluated a 20-year-old woman referred for pseudotumor cerebri (PTC).…”

A Pediatric Case of Anti-N-methyl-D-aspartate Receptor Encephalitis Associated With Optic Neuritis

“…Following the recent review of neuro-ophthalmic features of autoimmune encephalitis by Bohm et al, we present a pediatric case of NMDAR encephalitis with complete ophthalmic assessment to highlight one of the suggested associations (1). Pediatric ON in the context of NMDAR encephalitis is uncommon.…”

“…Anti-NMDAR encephalitis is a rare autoimmune encephalitis affecting 1 in 1.5 million people per year. Presenting features include a flu-like prodromal phase followed by neuropsychiatric findings, including headaches, seizures, movement disorders, agitation, and paranoia (1). Associations include tumors, mainly antibody-producing ovarian teratomas.…”

“…Associations include tumors, mainly antibody-producing ovarian teratomas. Anti-NMDAR antibody production may also follow viral encephalitis, particularly from HSV (1). First-line treatment includes immunosuppressive therapy with corticosteroids, IVIG, plasmapheresis, and tumor removal (1).…”