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When Guidelines Aren't Available, How Do You Treat...?
Neurology Turns to Clinical Practice Surveys

ARTICLE IN BRIEF

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For more information about the Practice Current surveys, visit http://cp.neurology.org/site/misc/practice_current.xhtml

Practice Current, a new project spearheaded by Neurology Clinical Practice, was developed to gather survey information about difficult clinical cases whose diagnoses or treatments are based on anecdotal experience and expert opinion.

Neurologists wondering how to diagnose and treat complicated or rare neurological conditions can now participate in an interactive survey and read how their colleagues around the world handle these issues.

Practice Current, a new project spearheaded by Neurology Clinical Practice, was developed to gather survey information about difficult clinical cases whose diagnoses or treatments are based on anecdotal experience and expert opinion.

“Many conditions in neurology don't have strong scientific evidence for treatment,” said Luca Bartolini, MD, a pediatric neurologist who is completing a fellowship in child neurology at Children's National Health System in Washington, DC. “I thought it would be good to build consensus and generate discussion of controversial topics in the field.”

Dr. Bartolini, a Section editor for Neurology Clinical Practice and a member of the Resident and Fellow Section of Neurology, worked with the Neurology Clinical Practice editorial board to develop this project.

Responses to the survey are anonymous, and as a second phase of the projet, an interactive map will include information on where the respondents live and their numbers of years in practice. “This provides readers with a unique perspective on how neurologists at different stages of their careers and in different areas of the world approach the same clinical scenario,” said Dr. Bartolini. Accompanying each clinical question is a brief review and expert opinion on the topic.

Dr. Bartolini said he chose this question because anti-NMDA receptor encephalitis is rare, but it has gained a lot of attention in recent years through a bestselling book called Brain on Fire: My Month of Madness by Susannah Cahalan. The author, a journalist, was diagnosed with the rare encephalitis after landing in the intensive care unit of a New York City hospital with bizarre psychiatric symptoms.

“Many neurologists may have seen a case of anti-NMDA receptor encephalitis that they remember more than others. For me, it's a case of a 10-year-old girl from China that I had followed when I was a pediatric resident at the University of Padua in Italy,” Dr. Bartolini recalled. “She was a normal girl until one day when she started vomiting and acting like she was ‘possessed.’ She was shouting at her father and yelling at her friends with no good reason. She was seeing things that were not there. Then, she became catatonic. Two days later, she started seizing and was brought to the hospital. She had a long and complicated course. She was in a coma in the ICU for two months and received aggressive immunotherapy. She was ultimately diagnosed with anti-NMDA receptor encephalitis.

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DR. LUCA BARTOLINI: “Many conditions in neurology dont have strong scientific evidence for treatment. I thought it would be good to build consensus and generate discussion of controversial topics in the field.”

“This was back in 2009, only two years after the initial reports of this disease. Eight months later, she came back for a follow-up visit. She was walking by herself. She stopped on the doorway, looked at me and said Ciao. I will never forget this moment.”

In 2007, Josep O. Dalmau, MD, PhD, a senior investigator at the Institute of Biomedical Investigations at the University of Barcelona, described a new set of symptoms that is now called anti-NMDA receptor encephalitis. The initial work was done when Dr. Dalmau was at the University of Pennsylvania, where he is still an adjunct professor of neurology.

Dr. Dalmau, who specializes in autoimmune and paraneoplastic neurological condition, was asked to provide his opinion on the treatment of this rare condition, and has outlined the immunotherapy protocols and innovative approaches to treating anti-NMDA receptor encephalitis in the February issue of Neurology Clinical Practice.

According to Dr. Bartolini, 300 physicians have responded to the survey in the first two months; 30 percent were residents or fellows. Most were utilizing a first-line treatment protocol consisting of intravenous immunoglobulin (IVIg) and high dose steroids. A small number or readers administered the comprehensive immunotherapy — IVIg, plasma exchange, steroids, and rituximab — proposed by Dr. Dalmau and outlined in the February issue of Neurology Clinical Practice.

“One of the novel aspects about this project is that there is no correct answer,” Dr. Bartolini said. “We are trying to figure out whether there is a standard of practice and if so, what is the science behind it. In the future, we plan on asking respondents whether their practice has changed.”

The next survey will be on the diagnostic evaluation of cryptogenic stroke. Peter Rothwell, MD, a professor of neurology at the University of Oxford, is reviewing current protocols and will provide the expert opinion on the topic.

EXPERTS COMMENT

Asked to comment on the survey, Gary Gronseth, MD, FAAN, the chief methodologist of the AAN Guideline Developmental, Dissemination, and Implementation (GDDI) Subcommittee of the American Academy of Neurology and a professor and vice chair of the department of neurology at the University of Kansas Medical Center, said: “I think it's valuable to know what other people are doing. How do neurologists deal with difficult situations in the absence of scientific evidence?”

Dr. Gronseth was involved in the initial launch last summer of a similar online survey called Q-PULSE that was created by the American Epilepsy Society. [Read the Neurology Today article, “How Epileptologists Are Using Surveys to Keep a PULSE on Epilepsy,” http://bit.ly/epilepsy-PULSE.]

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DR. GARY GRONSETH: “These types of survey questions allow us to see whether there is a lot of variation in practice and gaps in care. Most conditions in neurology have a lack of strong evidence that guides practice. Expert opinion and judgment are still the main tools we rely on when making decisions.”

“These types of survey questions allow us to see whether there is a lot of variation in practice and gaps in care,” he said. “Most conditions in neurology have a lack of strong evidence that guides practice. Expert opinion and judgment are still the main tools we rely on when making decisions.”

Variations and gaps in care also speak to the value of systematically seeking out evidence that supports a particular approach, experts said.

Cynthia Harden, MD, system director of epilepsy services at the Mount Sinai Health System in New York, agreed. She was involved in the development of Q-PULSE too and is chair of the AAN's GDDI Subcommittee.

“NMDA receptor encephalitis is a good topic for discussion because the best practices have evolved from observational studies and not clinical trials,” she said. “The results from these surveys highlight what we need to address when we are developing new guidelines for neurological conditions.”

The subcommittee is now working on cost and treatment guidelines for disease modifying therapies for multiple sclerosis, she said, as well as guidelines for sudden unexpected death in epilepsy or SUDEP.

LINK UP FOR MORE INFORMATION:

•. Bartolini L. Commentary: How do you treat anti-NMDA receptor encephalitis http://cp.neurology.org/content/early/2016/02/01/CPJ.0000000000000219.short?rss=1. Neurology 2016: Epub 2016 Jan. 29.