Table 1.
Patient characteristics and DBS treatment response
Age at onset | Symptoms | Age at DBS implant | DBS target | Stimulation response | Duration of response | |
---|---|---|---|---|---|---|
Hebb et al. [6], 2006 | 15 months | - Complex hyperkinetic syndrome | 17 years | VIM (unilateral) | - Contralateral chorea, myoclonus improved in few days to weeks | - Improvements stabilized after first year |
- Severe chorea | ||||||
- Myoclonus | - Eventually (with increased amplitude of stimulation) axial and then ipsilateral improvements noted | - System inadvertently deactivated 4 years postop, no deterioration in clinical symptoms | ||||
- Bilateral intention tremor | ||||||
- Dystonic features | - Intention tremor and dystonia persisted | |||||
Hamasaki et al. [5], 2010 | 30 years | - Cervical dystonia that progressed to secondary generalized | 52 years | GPi (bilateral) | - Dystonia and dystonic tremor improved over first weeks to months | - Continued improvements to 5 months with stabilization at last follow-up |
- Blepharospasm | ||||||
- Oromandibular grimacing | - Axial symptoms did not improve | |||||
- Truncal bending and torsion | ||||||
- Dystonic tremor | ||||||
- Postural instability | ||||||
Our patient - 1st implant | 12–15 months | - Frequent stumbling | 10 years | GPi (bilateral) | - Mild to moderate bilateral tremor reduction | - High impedance noted after 4 months prompting surgical exploration |
- Poor coordination with clumsy movements | ||||||
- Balance difficulty | - Opted to move intracranial leads to bilateral VIM in hopes of better response | |||||
- Tremor (started at age 5) | ||||||
Our patient - 2nd implant | Same | Same | 11 years | VIM (bilateral) | - Resting tremor resolved with significant improvement in intention tremor | - Continued refinement in programming with continued substantial improvement in tremor control 12 months postoperatively |
DBS, deep brain stimulation; VIM, ventral intermediate nucleus of the thalamus; GPi, globus pallidus interna.