# Deep Brain Stimulation of the VIM for Tremor in a Patient with POLR3A-Associated Cerebellar Syndrome Without Long-Term Benefit

## TL;DR

A patient with a rare genetic disorder did not benefit from a brain stimulation treatment typically used for tremors.

## Contribution

This case suggests VIM DBS may not be effective for POLR3A-related cerebellar tremors.

## Key findings

- VIM DBS did not provide long-term relief for the patient's drug-resistant tremor.
- Tremor from POLR3A mutations may involve different brain networks than typical tremor disorders.

## Abstract

Deep brain stimulation is an approved therapy for essential tremor and Parkinson’s disease. In addition, VIM-DBS is used off-label for the treatment of tremor syndromes with a different etiology.

We present the case of a woman with a drug-refractory action tremor due to rare compound heterozygous POLR3A mutations. Her treatment with VIM DBS did not lead to a sustained improvement of symptoms.

Tremor due to POLR3A-related cerebellar syndromes may not be responsive to VIM DBS. The networks contributing to cerebellar tremor should be better investigated in terms of where neuromodulatory therapy might be more effective.