Nov. 23, 2023
Mayo Clinic is strengthening its commitment to cutting-edge research and care for individuals with autoimmune encephalitis and neuromyelitis optica. As a pioneer in the evolving field of autoimmune neurology, Mayo Clinic is one of a handful of centers with experience in the diagnosis and treatment of these challenging conditions.
"We know how to manage autoimmune encephalitis and neuromyelitis optica. In such a rapidly growing field, caring for these patients properly requires clinical experience with other similar patients," says Sarosh R. Irani, M.B., Ch.B., D.Phil., a neurologist who recently joined Mayo Clinic in Jacksonville, Florida.
Dr. Irani and his team were the first to describe some of these conditions, including LGI1- and CASPR2-antibody encephalitis. He and his team also have made major contributions to the understanding of clinical features and causes of other autoimmune neurological conditions, as well as optimal treatment methods.
"The number of patients we've seen is really quite huge, and the experience we've gained by doing that is massive," Dr. Irani says.
Rapid onset of symptoms is a hallmark of autoimmune encephalitis. Accurate diagnosis during this acute phase is key. "Delaying treatment unequivocally adversely affects outcomes," Dr. Irani says.
A multidisciplinary approach helps to optimize outcomes over what is often lifelong treatment. "Relapses in this disease are devastating, and our goal is to prevent that," Dr. Irani says.
Clinical experience
Autoimmune neurological conditions are less rare than previously thought. Neuromyelitis optica is more common than multiple sclerosis in many countries. What's more, in a groundbreaking study published in 2018 in the Annals of Neurology, Mayo Clinic researchers reported that autoimmune encephalitis is as common as infectious encephalitis.
"The number of patients we've seen is really quite huge, and the experience we've gained by doing that is massive."
But establishing a diagnosis of autoimmune encephalitis is challenging. Patients typically are young adults who initially present with psychotic delusions, increased anxiety or insomnia — symptoms that can be mistakenly attributed to other conditions.
Within weeks, most patients experience seizures, movement disorders or other focal neurological deficits. The rapid onset of symptoms, with no other apparent cause, warrants testing for antibodies.
"Mayo Clinic Laboratories gives us an excellent and rapid set of test results that exclude other conditions but also include autoimmune neurological conditions," Dr. Irani says. "However, the diagnostic methods and test results are nuanced. Clinical experience with these patients — knowing what you're looking for and what you're not looking for — is just as important."
Patients generally respond to immunomodulatory therapies. But individual treatment plans are complex. The immune system response that leads to autoimmune encephalitis is sometimes triggered by a tumor, which must be treated. Immunosuppression also must be managed carefully.
"Proper patient care requires expertise from multiple different sources," Dr. Irani says. Mayo Clinic's care team includes specialists in radiology, ophthalmology, neurophysiology, oncology, hematology, psychology and psychiatry.
"In the acute setting, our focus is on rapid diagnosis and treatment and on giving patients and their families accurate prognostic information," Dr. Irani says. "That includes indications of how long patients will need to be hospitalized and when we might see some recovery. Rapid diagnostics and a period in hospital can make a massive difference in patient outcomes."
Transformational research
Mayo Clinic's clinician-researchers are engaged in multiple efforts to improve understanding of autoimmune neurological diseases, with the goal of enhancing treatment.
"Directly seeing so many patients contributes a lot to the understanding of these diseases," Dr. Irani says. "We use their precious insights to describe and refine clinical phenotypes and to learn more about disease immunopathogenesis."
Formal clinical trials are being developed to test therapeutics for neuromyelitis optica and autoimmune encephalitis. Another avenue of research is evaluating patients' long-term outcomes.
"Nobody has yet measured exactly how patients do in the long term and if there are, for example, reasons that we should be treating them more intensively earlier or for longer," Dr. Irani says. "We go on clinical intuition because this is such a new field. Understanding longer term outcomes, as well as developing acute interventional clinical trials, is important to us."
These research efforts are bolstered by Mayo Clinic physicians' close ties to patient advocacy groups. Dr. Irani serves on the Encephalitis Society Scientific Advisory Panel and on the Autoimmune Encephalitis Alliance Medical Advisory Board.
"The last thing a clinician wants to do is miss these conditions — because they are treatable," Dr. Irani says. "There is excitement around our efforts because the research can directly translate to transforming patients' lives."
For more information
Dubey D, et al. Autoimmune encephalopathy epidemiology and a comparison to infectious encephalitis. Annals of Neurology. 2018;83:166.
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