Autoimmune Neurology: A New, Rapidly Evolving Subspecialty

Deborah Brauser

December 18, 2017

An emerging new subspecialty is gaining ground as more fellowships and a growing body of research spur interest in the field of autoimmune neurology.

Unlike neuroimmunology, autoimmune neurology's focus is much broader than multiple sclerosis (MS) and includes disorders such as neurosarcoidosis, other "white matter in the brain" conditions, childhood central nervous system diseases, and even leukodystrophy, Stacey L. Clardy, MD, PhD, Department of Neurology at the University of Utah, Salt Lake City, told Medscape Medical News.

Dr Clardy and A. Sebastian López-Chiriboga, MD, Mayo Clinic, Rochester, Minnesota, discuss the history and current status of autoimmune neurology in a paper recently published in Neurology.

Rapidly Evolving Field

"Autoimmune neurology is one of the most rapidly evolving fields in modern neurology," they write. They add that the rate of new antigen discoveries is accelerating and impressive.

"The diversity of neurologic presentations, the unique pathophysiology, and the complexity of treating these disorders justifies dedicated fellowship training to acquire the expertise needed to diagnose and optimally manage these patients," they add.

Autoimmune neurology includes knowledge in all neurologic subspecialties, as well as infectious disease, psychiatry, gastroenterology, and rheumatology.

Currently four dedicated fellowships in this new subspecialty are offered in the United States, along with other programs that include at least some training in the area.

Fellowships in traditional neuroimmunology focus on MS. Training in autoimmune neurology differs by focusing on "a sound diagnostic approach to patients with suspected autoimmune neurologic disease," write the authors.

"Knowledge regarding the risks of emerging immunotherapies is an essential component of training, specifically including pretreatment testing and prophylaxis, as well as long-term monitoring for chronic complications."

Dr López is an incoming autoimmune fellow at Mayo Clinic. He told Medscape Medical News that the field has changed dramatically, with new and different ways to evaluate patients and to treat them with immunotherapy, which often leads to "remarkable improvements."

The first formal fellowship program in this subspecialty was started at Mayo in 2005. In the years before that, many in the field believed that autoimmune neurologic disorders were very rare. Therefore, there was no need to specialize in their diagnosis and treatment, the authors write.

However, "new neural autoantibodies aiding diagnosis," and new test methods, have shown that the conditions are actually not so rare.

While neuroimmunology training may not provide exposure to a sufficient number of patients with neuronal antibody disease, training in autoimmune neurology focuses on diagnosing autoantibody profiles.

"Autoimmune neurology fellowship programs must teach trainees the specific risks and monitoring necessary to safely manage the often aggressive immunomodulation or immunosuppression required for treatment," write Dr López and Dr Clardy.

Fellowship Programs

Dr Clardy, who is a graduate of the autoimmune neurology fellowship program at Mayo, is now the director of the autoimmune neurology clinic at the University of Utah.

In addition to fellowships from Mayo and the University of Utah, Massachusetts General Hospital/Harvard Medical School in Boston and the University of Texas Southwestern Medical Center in Dallas also now offer dedicated autoimmune neurology fellowship programs.

All but Massachusetts General offer both research and clinical training, are open to pediatric neurologists, and include exposure to neuronal antibody testing. All four programs offer inpatient consult services or rotations.

"I think all four really stress a focus on antibody-mediated neurologic syndromes. If you had to pick just one phrase, it's that it focuses on the antibodies needed for a condition," said Dr Clardy.

She noted that there are similarities between the fields of autoimmune neurology and neuroimmunology, with some crossovers in training. However, she reiterated that it's the focus on antibodies that differentiates the two.

"There are more and more of these antibody-mediated conditions being described every year, at the rate of about two to three per year, and each can have a unique and unusual presentation," she said.

"That can be challenging for someone who identifies as, say, an MS specialist to keep up with this exploding field as almost a side practice, if you will."

Dr Clardy said what she most gained from the fellowship she went through, and what she tries to teach her fellows now, is "just exposure to volumes of what is considered rare diseases. One needs to see more than one stiff person syndrome in a year to feel qualified. The four centers with fellowships are referral centers and are seeing these types of conditions in volume."

Career Opportunities

"Neurologists trained in autoimmune neurology are a valuable asset to any neurologic practice," write the authors. They add that "few neurologists are comfortable prescribing and managing the emerging arsenal of immunotherapy utilized in treating these conditions."

After going through an autoimmune neurology fellowship, a neurologist is likely to find a position in a large neuroimmunology clinic or an academic center, they write.

In addition, as awareness of autoimmune encephalitis, autoimmune epilepsy, and other conditions continues to grow, more experts in immunotherapy will be needed both in the United States and internationally.

Also, the authors note that this type of subspecialist has a role in diagnosing cancer.

"Recognition of an informative profile of autoantibodies in serum and spinal fluid focuses the oncologic evaluation and guides appropriate therapy," they write. "A close working relationship among the autoimmune neurologists, neuro-oncologist, and oncologist is ideal for optimal patient management."

Dr López reported that in his earlier training, he was able to see some cases of encephalitis and autoimmune epilepsy. "I saw some remarkable improvements, and that drove me towards this subspecialty," he said.

"I think the novelty, the continuous discovery of new biomarkers, and new treatment approaches — it's all very encouraging and certainly inspiring."

He added that the number of dedicated fellowships, as well as programs that incorporate autoimmune neurology training, will continue to grow.

"Many institutions are actively recruiting people who have this training, and some are in the process of creating new fellowships for more people because there is a huge need," said Dr López.

He pointed out that even if a physician is not subspecializing, it's important to get at least some training in this area. "These disorders are more common than previously thought. The more that we can test, and the more antibodies that are discovered, the more insights we'll be given into the pathogenesis of these conditions."

"I know that all of my mentors and me, we spend a tremendous amount of our free time answering emails from colleagues and responding to cries for help. To me, that makes it clear that there's a need for more of these people in the field," said Dr Clardy.

Dr López is the 2017-2018 autoimmune neurology fellow at Mayo Clinic. Dr Clardy is the director of the autoimmune neurology clinic at the University of Utah and is a graduate of the autoimmune neurology fellowship at Mayo.

Neurology. Published online September 12, 2017. Full article

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