By: Grace Huff
A newly published case series led by Matthew Remz, MD, at the Norman Fixel Institute for Neurological Diseases is reshaping how clinicians approach deep brain stimulation (DBS) in patients with DYT-11, a rare form of myoclonus-dystonia caused by a genetic variant. While DBS is typically highly effective for DYT-11, the Fixel team encountered a critical question: What happens when it’s not?
“Our work looks at a tough situation, what do you do when a real-life problem doesn’t behave the way the literature says it should?” said Remz. In DYT-11, the literature says that DBS is very effective. So when it’s not, that’s an incredibly challenging position for both patient and provider.
The study, published in Dystonia, presents three patient cases where standard DBS did not produce the expected benefits. Through advanced programming and, in some cases, surgical revision of DBS leads, the team achieved remarkable improvements. These cases mark the first documented instances of troubleshooting and DBS revision surgery for DYT-11.
The study also explored changes in brain signals, specifically in the beta frequency range, following optimized DBS. The Fixel team is now investigating whether these signals could serve as biomarkers to predict or guide DBS success. “Studying these patients helps us multiply that positive impact. We hope this work can guide others to emulate our success, or, just as importantly, challenge our conclusions, so we can all learn to do better for these patients.”
This research has broader implications for patient care far beyond UF Health. According to Remz, the next steps involve studying a larger group of patients and determining whether brain signal patterns can help fine-tune therapy or predict who will benefit most from DBS. “We hope this case series sparks more conversation and research into refining DBS therapy for rare genetic movement disorders,” said Remz.
With the Fixel Institute’s mission rooted in interdisciplinary innovation, this study exemplifies how clinical observations can lead to breakthroughs for patients who often fall through the cracks of conventional care.