Miquel, Marie, Spampinato, Umberto, Latxague, Chrystelle, Aviles-Olmos, Iciar ORCID: 0000-0002-1645-7406, Bader, Benedikt, Bertram, Kelly ORCID: 0000-0002-4283-1182, Bhatia, Kailash ORCID: 0000-0001-8185-286X, Burbaud, Pierre, Burghaus, Lothar, Cho, Jin Whan, Cuny, Emmanuel, Danek, Adrian ORCID: 0000-0001-8857-5383, Foltynie, Thomas, Garcia Ruiz, Pedro J., Gimenez-Roldan, Santiago, Guehl, Dominique, Guridi, Jorge ORCID: 0000-0001-5714-5145, Hariz, Marwan, Jarman, Paul, Kefalopoulou, Zinovia Maria, Limousin, Patricia, Lipsman, Nir, Lozano, Andres M., Moro, Elena, Ngy, Dhita, Cruz Rodriguez-Oroz, Maria, Shang, Huifang, Shin, Hyeeun, Walker, Ruth H., Yokochi, Fusako, Zrinzo, Ludvic and Tison, Francois (2013). Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis. PLoS One, 8 (11). SAN FRANCISCO: PUBLIC LIBRARY SCIENCE. ISSN 1932-6203

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Miquel, MarieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Spampinato, UmbertoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Latxague, ChrystelleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Aviles-Olmos, IciarUNSPECIFIEDorcid.org/0000-0002-1645-7406UNSPECIFIED
Bader, BenediktUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bertram, KellyUNSPECIFIEDorcid.org/0000-0002-4283-1182UNSPECIFIED
Bhatia, KailashUNSPECIFIEDorcid.org/0000-0001-8185-286XUNSPECIFIED
Burbaud, PierreUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Burghaus, LotharUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cho, Jin WhanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cuny, EmmanuelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Danek, AdrianUNSPECIFIEDorcid.org/0000-0001-8857-5383UNSPECIFIED
Foltynie, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Garcia Ruiz, Pedro J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gimenez-Roldan, SantiagoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Guehl, DominiqueUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Guridi, JorgeUNSPECIFIEDorcid.org/0000-0001-5714-5145UNSPECIFIED
Hariz, MarwanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jarman, PaulUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kefalopoulou, Zinovia MariaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Limousin, PatriciaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lipsman, NirUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lozano, Andres M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Moro, ElenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ngy, DhitaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cruz Rodriguez-Oroz, MariaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shang, HuifangUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shin, HyeeunUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Walker, Ruth H.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yokochi, FusakoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zrinzo, LudvicUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tison, FrancoisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-472140
DOI: 10.1371/journal.pone.0079241
Journal or Publication Title: PLoS One
Volume: 8
Number: 11
Date: 2013
Publisher: PUBLIC LIBRARY SCIENCE
Place of Publication: SAN FRANCISCO
ISSN: 1932-6203
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
DEEP-BRAIN-STIMULATION; PRIMARY GENERALIZED DYSTONIA; PARKINSONS-DISEASE; GLOBUS-PALLIDUS; FOLLOW-UP; NEUROACANTHOCYTOSIS; NEURODEGENERATION; DISORDERS; PATIENTMultiple languages
Multidisciplinary SciencesMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/47214

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