Abstract
Objective: Complex regional pain syndrome type is a poorly understood chronic neuropathic pain condition that is associated with movement disorders in 9−49% of patients and studies suggest that 14−25% of these involve dystonia. Here we present the cases of two female patients who were referred to us with generalised fixed dystonia that presented on a background of CRPS1. At the point of referral they had been seen by over 5 different specialists and their condition was rapidly progressive and refractory to optimum medical treatment.
Methods: Both patients underwent deep brain stimulation surgery under general anaesthesia whereby DBS electrodes were implanted into the Globus Pallidus interna bilaterally with the aid of an MRI-directed stereotactic technique. At one year, Dystonia was assessed using the Burke Fahn Marsden dystonia rating scale in addition to video recordings. Subjective pain scores were obtained using the Visual Analog Scale (VAS) and the Neuropathic Pain Scale (NPS); QOL was measured using the SF-36 health survey.
Results: Patient 1 had a 58.9% improvement in the severity of her dystonia; her disability score improved by 58.6% and she
experienced a 48% overall improvement in her SF-36 score. Patient 2 had a 77.7% reduction in her dystonia severity; a
53.3% reduction in her disability and a 20% improvement in her SF-36 score. Interestingly, this patient’s DBS battery ran
out at one year and this proved to be a useful blinded tool for assessment, it resulted in her dystonia reverting to presurgery severity.
Conclusions: Fixed dystonia alone and in association with CRPS is an extremely challenging condition to treat and
current therapeutic modalities are sub-optimal. Although DBS has been used widely in primary mobile dystonia, we
have demonstrated for the first time the successful use of DBS in complex regional pain syndrome associated with fixed
dystonia.
Methods: Both patients underwent deep brain stimulation surgery under general anaesthesia whereby DBS electrodes were implanted into the Globus Pallidus interna bilaterally with the aid of an MRI-directed stereotactic technique. At one year, Dystonia was assessed using the Burke Fahn Marsden dystonia rating scale in addition to video recordings. Subjective pain scores were obtained using the Visual Analog Scale (VAS) and the Neuropathic Pain Scale (NPS); QOL was measured using the SF-36 health survey.
Results: Patient 1 had a 58.9% improvement in the severity of her dystonia; her disability score improved by 58.6% and she
experienced a 48% overall improvement in her SF-36 score. Patient 2 had a 77.7% reduction in her dystonia severity; a
53.3% reduction in her disability and a 20% improvement in her SF-36 score. Interestingly, this patient’s DBS battery ran
out at one year and this proved to be a useful blinded tool for assessment, it resulted in her dystonia reverting to presurgery severity.
Conclusions: Fixed dystonia alone and in association with CRPS is an extremely challenging condition to treat and
current therapeutic modalities are sub-optimal. Although DBS has been used widely in primary mobile dystonia, we
have demonstrated for the first time the successful use of DBS in complex regional pain syndrome associated with fixed
dystonia.
| Original language | English |
|---|---|
| Pages | S6-S7 |
| Number of pages | 2 |
| DOIs | |
| Publication status | Published - 1 Oct 2009 |
| Event | 8th International Congress of the European Paediatric Neurology Society - Harrogate, United Kingdom Duration: 30 Sept 2009 → 3 Oct 2009 |
Conference
| Conference | 8th International Congress of the European Paediatric Neurology Society |
|---|---|
| Abbreviated title | EPNS 2009 |
| Country/Territory | United Kingdom |
| City | Harrogate |
| Period | 30/09/09 → 3/10/09 |