Limits...
Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis.

Miquel M, Spampinato U, Latxague C, Aviles-Olmos I, Bader B, Bertram K, Bhatia K, Burbaud P, Burghaus L, Cho JW, Cuny E, Danek A, Foltynie T, Garcia Ruiz PJ, Giménez-Roldán S, Guehl D, Guridi J, Hariz M, Jarman P, Kefalopoulou ZM, Limousin P, Lipsman N, Lozano AM, Moro E, Ngy D, Rodriguez-Oroz MC, Shang H, Shin H, Walker RH, Yokochi F, Zrinzo L, Tison F - PLoS ONE (2013)

Bottom Line: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively).Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable.Parkinsonism did not improve.

View Article: PubMed Central - PubMed

Affiliation: Service de Neurologie, CHU Bordeaux, Bordeaux, France ; Service de Neurologie, CH François Mitterrand, Pau, France.

ABSTRACT

Background: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS.

Methods: Data were collected in a standardized way 2-6 months preoperatively, 1-5 months (early) and 6 months or more (late) after surgery at the last follow-up visit (mean follow-up: 29.5 months).

Results: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Functional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independence Score) improvement reached significance at early post-surgery only (mean 37.3%). Long term significant improvement of motor symptom severity (≥ 20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable. Parkinsonism did not improve. Linear regression analysis showed that preoperative motor severity predicted motor improvement at both post-surgery time points. The most serious adverse event was device infection and cerebral abscess, and one patient died suddenly of unclear cause, 4 years after surgery.

Conclusion: This study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc.

Show MeSH

Related in: MedlinePlus

Effect of deep brain stimulation on motor impairment as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS).Bar histograms (panel A) represent means ± SEM (n= 11 patients). Line plots (panels B, C) show individual values per patient plotted at each of three time points: 2-6 months preoperatively (PREOP), 1-5 months postoperatively (early post-operative: EPOP), and 6 months or more after surgery (last outcome reporting: LOR). Individual data curves are shown in panel B, whereas panel C depicts percentage changes at EPOP and LOR with PREOP values set to 100% to make improvements and deteriorations easier to distinguish. Scores of patient 7 correspond to the Burke-Fahn-Marsden Dystonia Rating Scale-Motor part (BFMDRS-M) [16]. **p<0.01, ***p<0.001 versus PREOP time point (Bonferroni test after ANOVA: F(2, 20)= 15.11, p< 0.001, n= 11).
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC3818425&req=5

pone-0079241-g001: Effect of deep brain stimulation on motor impairment as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS).Bar histograms (panel A) represent means ± SEM (n= 11 patients). Line plots (panels B, C) show individual values per patient plotted at each of three time points: 2-6 months preoperatively (PREOP), 1-5 months postoperatively (early post-operative: EPOP), and 6 months or more after surgery (last outcome reporting: LOR). Individual data curves are shown in panel B, whereas panel C depicts percentage changes at EPOP and LOR with PREOP values set to 100% to make improvements and deteriorations easier to distinguish. Scores of patient 7 correspond to the Burke-Fahn-Marsden Dystonia Rating Scale-Motor part (BFMDRS-M) [16]. **p<0.01, ***p<0.001 versus PREOP time point (Bonferroni test after ANOVA: F(2, 20)= 15.11, p< 0.001, n= 11).

Mentions: Figure 1 displays the effect of DBS on UHDRS motor scores at EPOP and LOR time points compared to baseline. Mean UHDRS-MS (Figure 1A) was significantly reduced at both time points after surgery. Mean proportional improvement of UHDRS-MS compared to baseline was +54.3% and +44.1% for EPOP and LOR, respectively. Figures 1B and 1C show individual outcomes on UHDRS-MS scores and changes compared to PREOP values. All 13 patients showed an improvement of 20% or more at EPOP, whereas at LOR, only 8 of these 13 patients (61.5 %), for whom UHDRS-MS was available, showed a significant improvement.


Short and long term outcome of bilateral pallidal stimulation in chorea-acanthocytosis.

Miquel M, Spampinato U, Latxague C, Aviles-Olmos I, Bader B, Bertram K, Bhatia K, Burbaud P, Burghaus L, Cho JW, Cuny E, Danek A, Foltynie T, Garcia Ruiz PJ, Giménez-Roldán S, Guehl D, Guridi J, Hariz M, Jarman P, Kefalopoulou ZM, Limousin P, Lipsman N, Lozano AM, Moro E, Ngy D, Rodriguez-Oroz MC, Shang H, Shin H, Walker RH, Yokochi F, Zrinzo L, Tison F - PLoS ONE (2013)

Effect of deep brain stimulation on motor impairment as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS).Bar histograms (panel A) represent means ± SEM (n= 11 patients). Line plots (panels B, C) show individual values per patient plotted at each of three time points: 2-6 months preoperatively (PREOP), 1-5 months postoperatively (early post-operative: EPOP), and 6 months or more after surgery (last outcome reporting: LOR). Individual data curves are shown in panel B, whereas panel C depicts percentage changes at EPOP and LOR with PREOP values set to 100% to make improvements and deteriorations easier to distinguish. Scores of patient 7 correspond to the Burke-Fahn-Marsden Dystonia Rating Scale-Motor part (BFMDRS-M) [16]. **p<0.01, ***p<0.001 versus PREOP time point (Bonferroni test after ANOVA: F(2, 20)= 15.11, p< 0.001, n= 11).
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3818425&req=5

pone-0079241-g001: Effect of deep brain stimulation on motor impairment as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS).Bar histograms (panel A) represent means ± SEM (n= 11 patients). Line plots (panels B, C) show individual values per patient plotted at each of three time points: 2-6 months preoperatively (PREOP), 1-5 months postoperatively (early post-operative: EPOP), and 6 months or more after surgery (last outcome reporting: LOR). Individual data curves are shown in panel B, whereas panel C depicts percentage changes at EPOP and LOR with PREOP values set to 100% to make improvements and deteriorations easier to distinguish. Scores of patient 7 correspond to the Burke-Fahn-Marsden Dystonia Rating Scale-Motor part (BFMDRS-M) [16]. **p<0.01, ***p<0.001 versus PREOP time point (Bonferroni test after ANOVA: F(2, 20)= 15.11, p< 0.001, n= 11).
Mentions: Figure 1 displays the effect of DBS on UHDRS motor scores at EPOP and LOR time points compared to baseline. Mean UHDRS-MS (Figure 1A) was significantly reduced at both time points after surgery. Mean proportional improvement of UHDRS-MS compared to baseline was +54.3% and +44.1% for EPOP and LOR, respectively. Figures 1B and 1C show individual outcomes on UHDRS-MS scores and changes compared to PREOP values. All 13 patients showed an improvement of 20% or more at EPOP, whereas at LOR, only 8 of these 13 patients (61.5 %), for whom UHDRS-MS was available, showed a significant improvement.

Bottom Line: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively).Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable.Parkinsonism did not improve.

View Article: PubMed Central - PubMed

Affiliation: Service de Neurologie, CHU Bordeaux, Bordeaux, France ; Service de Neurologie, CH François Mitterrand, Pau, France.

ABSTRACT

Background: Chorea-acanthocytosis (ChAc) is a neuroacanthocytosis syndrome presenting with severe movement disorders poorly responsive to drug therapy. Case reports suggest that bilateral deep brain stimulation (DBS) of the ventro-postero-lateral internal globus pallidus (GPi) may benefit these patients. To explore this issue, the present multicentre (n=12) retrospective study collected the short and long term outcome of 15 patients who underwent DBS.

Methods: Data were collected in a standardized way 2-6 months preoperatively, 1-5 months (early) and 6 months or more (late) after surgery at the last follow-up visit (mean follow-up: 29.5 months).

Results: Motor severity, assessed by the Unified Huntington's Disease Rating Scale-Motor Score, UHDRS-MS), was significantly reduced at both early and late post-surgery time points (mean improvement 54.3% and 44.1%, respectively). Functional capacity (UHDRS-Functional Capacity Score) was also significantly improved at both post-surgery time points (mean 75.5% and 73.3%, respectively), whereas incapacity (UHDRS-Independence Score) improvement reached significance at early post-surgery only (mean 37.3%). Long term significant improvement of motor symptom severity (≥ 20 % from baseline) was observed in 61.5 % of the patients. Chorea and dystonia improved, whereas effects on dysarthria and swallowing were variable. Parkinsonism did not improve. Linear regression analysis showed that preoperative motor severity predicted motor improvement at both post-surgery time points. The most serious adverse event was device infection and cerebral abscess, and one patient died suddenly of unclear cause, 4 years after surgery.

Conclusion: This study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc.

Show MeSH
Related in: MedlinePlus