Highly Effective, Short‐Latency Responses to Pallidal Stimulation in Axial Dystonia Supports Early Deep Brain Stimulation
Eoghan Donlon, Jack Horan, Maeve Bradley, Federica Ruggieri, Marie Kelly, Elaine Reynolds, Conor Fearon, Tim Lynch, Erlick Pereira, Catherine Moran, Richard A. WalshAbstract
Background
Axial dystonia is a rare form of adult‐onset focal dystonia. The majority of cases are idiopathic, however a similar phenotype can be seen in monogenic dystonias and tardive syndromes. Management of axial dystonia can be challenging, and most cases are typically refractory to pharmacotherapy and botulinum toxin injections.
Cases
We describe three cases of axial‐predominant dystonia, two idiopathic and one with a monogenic etiology, each demonstrating an early and excellent response to GPi DBS.
Literature review
We conducted a narrative review of the existing literature on axial dystonia, which remains limited to case reports and small series, with no large cohort studies to guide evidence‐based management.
Conclusions
Consistent with limited previous reports and the three cases presented, our findings suggest that GPi DBS offers favorable outcomes in refractory AD regardless of underlying pathological substrate and should be considered early in disease course.