Have You Heard of the Neurologist Behind Louis-Bar Syndrome?
Now You Will.
By Dawn Fallik
March 19, 2020
Article In Brief
The woman who is associated with the eponymous Louis-Bar syndrome is the subject for discussion—and an award—about the challenges for a woman in neurology from an earlier era.
When Elizabeth A. Coon, MD, started researching Denise Louis-Bar, MD, the neurologist who helped define the eponymous pediatric neurologic syndrome, Dr. Coon wasn't sure if she'd chosen the right subject.
As an adult neurologist, Dr. Coon, an assistant professor of neurology at Mayo Clinic in Rochester, MN, had never met someone with the disease Dr. Louis-Bar helped discover—ataxia telangiectasia. But that very afternoon in her clinic, she met a gentleman with familial ataxia and ocular telangiectasia, which has symptoms very similar to those in Louis-Bar syndrome.
“It was meant to be,” Dr. Coon said.
Her paper on Dr. Louis-Bar was published in Neurology in 2018. But this year, Dr. Coon is receiving the AAN McHenry Award in History for her research on the neurologist behind ataxia telangiectasia. The disease is caused by mutations in the ATM gene and appears as an early-onset multisystem neurodegenerative disorder, characterized by a progressive lack of coordination that appears in toddlers when they are starting to learn to walk. Patients also have a high rate of leukemia or lymphoma.
Dr. Louis-Bar first described the disorder in 1941 in the international journal Confinia Neurologica, published in Switzerland. She was 27 years old at the time. But as the Neurology article noted, she never really received credit for naming the disorder. Indeed, a citation in the Neurology article pulled from the literature underscores how being a woman in medicine at the time was perceived: “Dr. M. Reznik recalled Louis-Bar during her time in Liège: ‘I have really known Mrs. Louis-Bar when, myself a young student, she was a senior assistant at the Brull service. One recognized her by her lush red hair.’” As the article noted Dr. Louis-Bar had become a “neuropsychiatrist” within the department of internal medicine with renowned physiologist Professor Lucien Brull at the State University of Liège.
Dr. Coon discussed with Neurology Today what she learned about Dr. Louis-Bar and how it has influenced her own role as a neurologist today. Her excerpted remarks appear below.
In your research, you write about how Dr. Louis-Bar became interested in treating children because she saw the health inequities and health problems in young children. How did you become interested in neurology?
I've been interested in medicine since I was a teenager. My uncle was in family medicine and I knew I wanted to care for people, but also be involved in a scientific and rigorous field. My interest in neurology arose from personal experiences. I remember as an undergrad, I was working in a research lab studying DNA sequences in zebrafish that had neurological disorders. I was also shadowing Hank Paulson [Henry Paulson, MD, PhD] who was caring for patients with Huntington's disease in a multidisciplinary clinic. So in the morning I was looking at genetic code and in the afternoon, I was seeing patients deal with significant symptoms stemming from mutations in their genetic code.
What surprised you about your research into Dr. Louis-Bar?
I knew about the disease, ataxia telangiectasia, but I wasn't sure who she was. I thought the disease was named after two different people at first and assumed they were men. But what became really interesting to me was how the disease became named after her. She was not really the first person to describe the disease—it was two Czech physicians in 1926, but they described it as more of a “dystonia-type of disorder” and so it wasn't linked with Louis-Bar.
The group who really did the most work on ataxia telangiectasia was out of California in the mid-20th century, led by Dr. Elena Boder, an American pediatric neurologist. There was some controversy about calling the disease “Louis-Bar syndrome” versus ataxia telangiectasia, because it had been written about by other people, and there was some issue with Dr. Louis-Bar's pathophysiological description of AT. But when you look at the literature, her name still is frequently used.
Do you think that the syndrome should not be named after Dr. Louis-Bar?
It doesn't really bother me, as Elena Boder did advance the field significantly and I think that ataxia telangiectasia is a good descriptive name.
What did the research show about how Dr. Louis-Bar was regarded in the field?
Reading between the lines of the historical writing, you get a sense that she wasn't fully part of the medical community of that time. There was a follow-up to the journal article she had written in 1941 from her coworkers and mentor, and they didn't even use her name; there was no description of her work. I feel like maybe she didn't have all the support she could have used.
Were there any other clues about what being a woman in medicine was like at that time?
She was clearly a very accomplished physician and researcher, but in the articles about her, they describe her appearance, particularly that she had “lush, red hair.” Those physical descriptors bothered me as we know that women are more frequently judged by appearance. She didn't stay in academia; her husband went to Belgium, and she left the academic world when she moved. She continued to practice and developed subspecialty clinics caring for disabled children and adults, which is noble.
What else did you take away from your research about her?
When she was in academia, it was a very productive time for her. She later devoted her time to multidisciplinary care, particularly with mentally disabled children and she did amazing work in that regard. She was a very observant clinician and she really did a lot of work in internal medicine and in neurologic disorders, really emphasizing patient care, which demonstrated her diverse abilities and interests.
How do you relate Dr. Louis-Bar's story with your experience being a woman in Neurology Today?
This is a wonderful time to be a woman in medicine. Women are increasingly in leadership positions and I'm grateful to be in a department led by a woman, Claudia Lucchinetti, MD. Finding mentors who support and help you navigate your career is vital, and I'm also grateful for my many mentors. As a woman, there will be microaggressions and roadblocks along the way, but finding mentors who help you manage and continue moving forward is quintessential.
How did the research inform your thinking about working with patients with movement disorders?
I would say patients come with a variety of different symptoms and backgrounds. All patients need help in different ways. You have to learn to tailor your approach to the patient. It's not just knowing that a patient has a specific type of disease; it's about having to think about how that disease is affecting that particular patient, how the symptoms are impacting every aspect of their life. I was taught this is in residency with the Sir Williams Osler quote: “It is much more important to know what sort of a patient has a disease than what sort of a disease a patient has.”
Dr. Coon had no disclosures.