Objective: To evaluate long-term results of Deep Brain Stimulation (DBS) targeting the dentate nucleus (DN) in patients with cerebellar ataxia

Background: Cerebellar ataxia remains a neurological symptom orphan of treatment interventions, despite being prevalent and incapacitating. In a previous study, we evaluated safety and clinical outcomes of patients with cerebellar ataxia and DN DBS, comparing ON and OFF-stimulation in a double-blind manner [1]

Method: This is a randomized, prospective, double-blind, cross-over clinical trial registered at clinicaltrials.gov (protocol NCT03341416) and conducted in University of São Paulo to evaluate long-term outcomes of dentate nucleus DBS in ataxic patients. Primary outcomes are changes in the Scale for the Assessment and Rating of Ataxia (SARA) and in the Fahn Tolosa Marin Tremor Rating Scale (FTM) comparing ON and OFF stimulation. Patients arrived at the hospital with DBS ON, and the device was immediately turned OFF. After 30 minutes, patients were randomized to have DBS turned ON or remain OFF. After additional 30 minutes, patients were evaluated with clinical scales mentioned above, and switched groups, waiting for 30 minutes more. Finally, patients were again evaluated using clinical scales, and dismissed from the study protocol with DBS ON (Figure)

Results: Six patients from our original cohort were included. ON and OFF clinical data (Table) failed to demonstrate an acute beneficial effect of DN-DBS.

Conclusion: ON and OFF evaluations of DN-DBS with a 30-minutes interval failed to show clinical benefit. It is possible that DN DBS effects, similar to those of Globus Pallidus Internus in dystonic patients, need more time to appear after turning DBS ON, and more time to disappear after DBS OFF, due to a network adaptive effect.

Table: Results

Figure: Study design

References: 1. Cury RG, França C, Duarte KP, Paraguay I, Diniz JM, Cunha P, et al. Safety and Outcomes of Dentate Nucleus Deep Brain Stimulation for Cerebellar Ataxia. Cerebellum. 2021 Sep 4;21(5):861–5

« Back to 2024 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/dentate-deep-brain-stimulation-for-cerebellar-ataxia-long-term-outcomes/