Last modified: 2014-04-03
Abstract
Introduction: Deep brain stimulation (DBS) of the thalamic ventral intermediate nucleus (VIM) has been used to treat tremor in Parkinson Disease and Essential Tremor, but its use has not been described in the treatment of tremor related to cerebral palsy. We describe the use of DBS for treatment of medically refractory “rubral” tremor in a patient with ataxic cerebral palsy.
Case Description: A 16 year-old female with ataxic cerebral palsy and “rubral” tremor was treated with bilateral VIM DBS after modest benefit from propranolol, clonazepam, and primidone. Prior to DBS, she was unable to draw a spiral with her right hand. Tremor severity was assessed with the Fahn-Tolosa-Marin Tremor Rating Scale (TRS) with DBS off and on.
Results: Her “off” TRS score tremor score was 35/116 with moderate-severe handwriting and spiral drawing impairment. With DBS “on”, TRS score was 27 and handwriting and spiral drawing improved slightly. Despite the numerical improvement, the TRS did not capture the full magnitude of her clinical benefit. After DBS, for the first time in her life, she was able to apply her own nail polish and make-up, carry a cup of hot coffee safely, and write more legibly in school. It also had a tremendous benefit for her confidence and hopefulness regarding her personal and career goals.
Conclusion: This case report illustrates the potential therapeutic benefit of VIM DBS to treat tremor associated with cerebral palsy. The TRS may be insensitive to meaningful functional improvement.