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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.

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Related in: MedlinePlus

Regression analysis between preoperative (PREOP) severity of motor impairment, as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS), and motor severity improvement at early and late post-operative time points (EPOP, panel A, r = 0.66, r2 = 0.44, p< 0.05, n=13; LOR, panel B, r = 0.67, r2 = 0.45, p< 0.05, n=11); and motor severity improvement versus functional improvement, as assessed by the UHDRS-Functional Capacity Score (UHDRS-FCS), at EPOP (panel C, r = 0.63, r2 = 0.40, p< 0.05, n=13) and LOR (panel D, r = 0.67, r2 = 0.45, p< 0.05, n=11) time points.For each patient, motor and functional improvements correspond to the difference between appropriate UHDRS scores at PREOP and EPOP or LOR time points.
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pone-0079241-g003: Regression analysis between preoperative (PREOP) severity of motor impairment, as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS), and motor severity improvement at early and late post-operative time points (EPOP, panel A, r = 0.66, r2 = 0.44, p< 0.05, n=13; LOR, panel B, r = 0.67, r2 = 0.45, p< 0.05, n=11); and motor severity improvement versus functional improvement, as assessed by the UHDRS-Functional Capacity Score (UHDRS-FCS), at EPOP (panel C, r = 0.63, r2 = 0.40, p< 0.05, n=13) and LOR (panel D, r = 0.67, r2 = 0.45, p< 0.05, n=11) time points.For each patient, motor and functional improvements correspond to the difference between appropriate UHDRS scores at PREOP and EPOP or LOR time points.

Mentions: No significant relationship was found between age at surgery or disease duration and postoperative improvement on either motor or functional scales. Preoperative motor severity in UHDRS-MS predicted improvement in motor function at both EPOP (Figure 3A) and LOR time points (Figure 3B). Motor improvement was associated with an improvement in functional status (UHDRS-FCS) at EPOP and at LOR (Figure 3C, D); but not in the dependency score at both time points (UHDRS-IS).


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)

Regression analysis between preoperative (PREOP) severity of motor impairment, as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS), and motor severity improvement at early and late post-operative time points (EPOP, panel A, r = 0.66, r2 = 0.44, p< 0.05, n=13; LOR, panel B, r = 0.67, r2 = 0.45, p< 0.05, n=11); and motor severity improvement versus functional improvement, as assessed by the UHDRS-Functional Capacity Score (UHDRS-FCS), at EPOP (panel C, r = 0.63, r2 = 0.40, p< 0.05, n=13) and LOR (panel D, r = 0.67, r2 = 0.45, p< 0.05, n=11) time points.For each patient, motor and functional improvements correspond to the difference between appropriate UHDRS scores at PREOP and EPOP or LOR time points.
© Copyright Policy
Related In: Results  -  Collection

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

pone-0079241-g003: Regression analysis between preoperative (PREOP) severity of motor impairment, as assessed by the Unified Huntington’s Disease Rating Scale-Motor Score (UHDRS-MS), and motor severity improvement at early and late post-operative time points (EPOP, panel A, r = 0.66, r2 = 0.44, p< 0.05, n=13; LOR, panel B, r = 0.67, r2 = 0.45, p< 0.05, n=11); and motor severity improvement versus functional improvement, as assessed by the UHDRS-Functional Capacity Score (UHDRS-FCS), at EPOP (panel C, r = 0.63, r2 = 0.40, p< 0.05, n=13) and LOR (panel D, r = 0.67, r2 = 0.45, p< 0.05, n=11) time points.For each patient, motor and functional improvements correspond to the difference between appropriate UHDRS scores at PREOP and EPOP or LOR time points.
Mentions: No significant relationship was found between age at surgery or disease duration and postoperative improvement on either motor or functional scales. Preoperative motor severity in UHDRS-MS predicted improvement in motor function at both EPOP (Figure 3A) and LOR time points (Figure 3B). Motor improvement was associated with an improvement in functional status (UHDRS-FCS) at EPOP and at LOR (Figure 3C, D); but not in the dependency score at both time points (UHDRS-IS).

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