Oyama Genko, Thompson Amanda, Foote Kelly D, Limotai Natlada, Abd-El-Barr Muhammad, Maling Nicholas, Malaty Irene A, Rodriguez Ramon L, Subramony Sankarasubramoney H, Ashizawa Tetsuo, Okun Michael S
Department of Neurology, University of Florida, Center for Movement Disorders and Neurorestoration, Gainesville, FL, USA.
Department of Neurosurgery, University of Florida, Center for Movement Disorders and Neurorestoration, Gainesville, FL, USA.
Tremor Other Hyperkinet Mov (N Y). 2014 Jul 9;4:228. doi: 10.7916/D8542KQ5. eCollection 2014.
Deep brain stimulation (DBS) has been utilized to treat various symptoms in patients suffering from movement disorders such as Parkinson's disease, dystonia, and essential tremor. Though ataxia syndromes have not been formally or frequently addressed with DBS, there are patients with ataxia and associated medication refractory tremor or dystonia who may potentially benefit from therapy.
A retrospective database review was performed, searching for cases of ataxia where tremor and/or dystonia were addressed by utilizing DBS at the University of Florida Center for Movement Disorders and Neurorestoration between 2008 and 2011. Five patients were found who had DBS implantation to address either medication refractory tremor or dystonia. The patient's underlying diagnoses included spinocerebellar ataxia type 2 (SCA2), fragile X associated tremor ataxia syndrome (FXTAS), a case of idiopathic ataxia (ataxia not otherwise specified [NOS]), spinocerebellar ataxia type 17 (SCA17), and a senataxin mutation (SETX).
DBS improved medication refractory tremor in the SCA2 and the ataxia NOS patients. The outcome for the FXTAS patient was poor. DBS improved dystonia in the SCA17 and SETX patients, although dystonia did not improve in the lower extremities of the SCA17 patient. All patients reported a transient gait dysfunction postoperatively, and there were no reports of improvement in ataxia-related symptoms.
DBS may be an option to treat tremor, inclusive of dystonic tremor in patients with underlying ataxia; however, gait and other symptoms may possibly be worsened.
