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Published on: March 8, 2015
Deep Brain Stimulation of the VIM for Tremor in a Patient with POLR3A-Associated Cerebellar Syndrome Without
Ute Scheller1, Steffen Paschen2,3, Fabian Maass1
1University Medical Center Göttingen, Department of Neurology, Göttingen, Germany.
Background:
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.
Case Report:
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.
Discussion:
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.
