Recognizing the Symptoms and Diagnostic Workup in NMDA Receptor Encephalitis: Gregory Day, MD, MSc, MSCI, FAAN

WATCH TIME: 5 minutes

“When seeing a patient who's coming in with new psychiatric symptoms, or new seizures, or new movement disorders, or new complaints from a memory and thinking standpoint, maybe even new changes in sleep patterns, it's probably wise to stop and ask, ‘is this the start of something? Is there a continuation of symptoms that have been there in the past? Do we need to think about diseases that could relate to inflammation?’”

Autoimmune encephalitis (AE) is a rare disease which occurs when the immune system produces antibodies and identify healthy brain cells as foreign. Thus, the autoimmune system gets triggered and attacks the brain cells. This then causes inflammation leading to symptoms of seizures, hallucinations, memory loss, psychosis, impaired cognition, and paranoia, among others. The most common of antibodies causing AE are anti-NMDA (N-methyl-D-aspartate) receptors and LGI1 (leucine-rich glioma inactivated 1) receptors.¹

Early diagnosis is one of the main methods in preventing further brain cell damage and mortality. Additionally, after aggressive treatment and 1-2 years of rehabilitation, approximately 80% of patients diagnosed with anti-NMDA AE make a full recovery.¹ In an upcoming trial (NCT04372615), lead investigator Gregory Day, MD, MSc, MSCI, FAAN, and colleagues plan to investigate the safety and efficacy of 300 mg of inebilizumab (Uplizna; Horizon)as a therapy for moderate-to-severe NMDAR encephalitis.²

Recently, Day, a neurologist at Mayo Clinic in Jacksonville Florida, sat down with NeurologyLive® in an interview to talk about the bias and his thoughts on the different outcomes that occur in research for patients with anti-NMDA receptor autoimmune encephalitis. He also spoke about early detection and prevention with the disease such as knowing the appropriate time to intervene with treatment.

REFERENCES
1. The International Autoimmune Encephalitis Society’s #AEAware Campaign. News Release. International Autoimmune Encephalitis Society. Accessed April 17, 2023. https://autoimmune-encephalitis.org/press-release/
2. Day G, Titulaer M, Wong KH, et al. The ExTINGUISH Trial: A Phase-2B Randomized Placebo-Controlled Trial of Inebilizumab in Anti-NMDA Receptor Encephalitis (P5-1.004). Neurology. Published May 2022, 98 (18 Supplement) 1651.