Staff Neurologist, Mellen Center for Multiple Sclerosis Treatment and Research, Cleveland Clinic

Amy Kunchok, MD

 Autoimmune encephalitis is very rare. The way that we recognize it is, clinically, we can identify a clinical phenotype, but we now have access to psychological biomarkers, so autoimmune encephalitis antibody biomarkers and these are neuro-autoantibodies that can be tested for and these can help us diagnose and also clinically stratify patients and understand their clinical phenotype and their outcomes. So these diagnostic biomarkers have really revolutionized our understanding of autoimmune encephalitis. 

Although it's increasingly recognized, so far, most of the studies in autoimmune encephalitis come from single centers, and often they are small descriptive cohorts of patients that give us this clinical information, which has really paved a lot of the understanding so far of autoimmune encephalitis. 

In this study, we tried to take a big picture perspective, or a big data approach, and tried to take a large population that had been tested for autoimmune encephalitis. So we took over 40,000 patients over a 2-year period, to get that big picture perspective to try and understand what are the most frequent antibodies that are detected in patients that have tested for autoimmune encephalitis; what are the age and sex associations that stand out in this very large population that have been tested; and what might what sort of clinical implications or pathophysiological inferences are we obtaining from looking at that data? That's the scope of this study.

Videos

The staff neurologist at Cleveland Clinic’s Mellen Center for Multiple Sclerosis Treatment and Research offered her insight into improving the recognition of autoimmune encephalitis. [WATCH TIME: 2 minutes]

Improved Recognition of Autoimmune Encephalitis

"So far, most of the studies of autoimmune encephalitis have come from single centers. They are often small, descriptive cohorts that give us clinical information, which has paved a lot of the understanding so far. In this study, we tried to take a big picture or big data perspective."

Although uncommon, autoimmune/paraneoplastic encephalitis/encephalopathy (AE) has become an increasingly recognized cognition with the use of antibody biomarkers. Due to its rarity, it has been hard for clinicians to gather large-scale data, making it one of the more difficult conditions to research. Although, a new study led by Amy Kunchok, MD, the largest of its kind to date, included 42032 patients who were tested for AE antibody biomarkers (AE-Abs) in serum and/or cerebrospinal fluid (CSF).

Americas Committee for Treatment and Research in Multiple Sclerosis (ACTRIMS) Forum 2022

, Feb 24-26, in West Palm Beach, Florida, showed that age and sex were determinants of the frequency of AE-Ab detection, with notable trends observed. In adults, the most frequency AE-Abs detected were NMDA-R-IgG, GAD65-IgG, and LGI1-IgG; whereas in children, the most frequent AE-Abs were NMDA-R-IgG, GAD65-IgG, and MOG-IgG1. Female sex was associated with NMDA-R-IgG and GAD65-IgG while male sex was associated with LGI1-IgG.

Kunchok, staff neurologist, Mellen Center for Multiple Sclerosis Treatment and Research, 

, concluded that age and sex associations of autoimmune/paraneoplastic biomarkers may aid in the prognostication and provide inference to disease pathophysiology. 

® sat down with Kunchok at the ACTRIMS Forum to discuss why this is an important area of research, along with some of the specific findings she observed. Additionally, she provided insight on which findings may contrast what has been previously known about autoimmune disorders.

For more coverage of ACTRIMS Forum 2022, 

REFERENCE
1. Kunchok, A, McKeon A, Zekeridou A, et al. Autoimmune encephalitis antibody biomarkers: frequency, age and sex associations. Presented at: ACTRIMS Forum 2022; February 24-26; West Palm Beach, Florida. Abstract CE2.4

The staff neurologist at Cleveland Clinic’s Mellen Center for Multiple Sclerosis Treatment and Research discussed the results of a new study on age and sex as determinants of autoimmune encephalitis. [WATCH TIME: 3 minutes]

Understanding Frequency, Age, and Sex Associations With Autoimmune Encephalitis Biomarkers: Amy Kunchok, MD

“In the talk, we discussed a little bit about the clinical phenotype of aquaporin-4 [IgG] NMOSD. We went through the clinical features and radiological features, and then compared also, the clinical and radiological features of MOGAD.”

Institutional Perspectives in Neurology: Multiple Sclerosis

 event, Amy Kunchok, MD, staff neurologist, Mellen Center for Multiple Sclerosis Treatment and Research, Cleveland Clinic, provided an overview of the clinical and radiological features of aquaporin-4 antibody (AQP4) neuromyelitis optica spectrum disorder (NMSOD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD), as well as diagnostic approaches. Discussing AQP4-IgG NMOSD with 

, Kunchok highlighted that it is mostly seen in adult females, and patients often have peri-ependymal lesions, ring enhancing, and spinal cord lesions. Compared to patients with MOGAD, these patients also had more severe attacks and greater disability. 

In her talk, Kunchok also discussed the most common phenotype for MOGAD, optic neuritis, which is often a bilateral optic neuritis involving the anterior optic nerve. Additionally, patients with MOGAD are younger and lack systemic autoimmunity and area postrema syndrome. Discussing brain and brainstem lesions, Kunchok noted that they are often large, fluffy acute disseminated encephalomyelitis-type lesions or cerebellar peduncle lesions. Less commonly observed were cortical encephalitis lesions seen with cortical FLAIR lesions, she said. 

The staff neurologist at the Cleveland Clinic Mellen Center for Multiple Sclerosis outlined key findings from her talk at a recent Institutional Perspectives in Neurology: Multiple Sclerosis event. [WATCH TIME: 3 minutes]

Amy Kunchok, MD

Articles

Improved Recognition of Autoimmune Encephalitis

Understanding Frequency, Age, and Sex Associations With Autoimmune Encephalitis Biomarkers: Amy Kunchok, MD

Clinical, Radiological Features of NMOSD and MOGAD: Amy Kunchok, MD

Latest News