Case Report: Deep brain stimulation improves tremor in FGF-14 associated spinocerebellar ataxia.

ABSTRACT:

Objectives

Spinocerebellar ataxia 27 (SCA 27) is a rare heredodegenerative disorder caused by mutations in the fibroblast growth factor 14 (FGF14) and characterized by early-onset tremor and progressive ataxia later during the disease course. We investigated the effect of deep brain stimulation (DBS) of the ventralis intermedius nucleus of the thalamus (VIM) and subthalamic projections on tremor and ataxia.

Methods

At baseline, we studied the effects of high-frequency VIM stimulation and low-frequency stimulation of subthalamic projections on tremor and ataxia. The patient then adopted the best individual high-frequency stimulation programme at daytime and either 30 Hz-stimulation of the subthalamic contacts or StimOFF at night during two separate 5-weeks follo

SUBMITTER: Loeffler MA 

PROVIDER: S-EPMC9795845 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature