Short and Long Term Outcome of Bilateral Pallidal Stimulation in Chorea-Acanthocytosis

PLoS One

Fusako Yokochi¹  Jorge Guridi²  Ruth H. Walker³  Thomas Foltynie⁴  Adrian Danek⁵  Ludvic Zrinzo⁶  François Tison⁷  Maria Cruz Rodriguez-Oroz⁸  Dhita Ngy⁸  Huifang Shang⁹  Hyeeun Shin^1,10  Nir Lipsman^1,11  Kailash Bhatia^1,12  Santiago Giménez-Roldán^1,12  Zinovia Maria Kefalopoulou^1,13  Pierre Burbaud^1,14  Pedro J. Garcia Ruiz^1,15  Umberto Spampinato^1,16  Marie Miquel^1,17  Benedikt Bader^1,17  Chrystelle Latxague^1,17  Emmanuel Cuny^1,18  Paul Jarman^1,18  Marwan Hariz^1,19  Jin Whan Cho^2,20  Patricia Limousin^2,21  Dominique Guehl^2,21  Elena Moro^2,21  Andres M. Lozano^2,21  Kelly Bertram^2,21  Iciar Aviles-Olmos^2,22  Lothar Burghaus^2,23 

[1] Avicenna Medical Center, New York, New York, United States of America;Department of Neurology, Fundacion Jimenez Diaz, Madrid, Spain;Department of Neurology, Mount Sinai School of Medicine, New York, New York, United States of America;Department of Neurology, Samsung Medical Centre, Sungkyunkwan University, School of Medicine, Seoul, Korea;Department of Neurology, University Hospital Cologne, Cologne, Germany;Department of Neurology, University Hospital Donostia, Neuroscience Unit, BioDonostia Research Institute, San Sebastian, Spain;Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, China;Division of Neurosurgery, Toronto Western Hospital, University of Toronto, University Health Network, Toronto, Ontario, Canada;Morton and Gloria Shulman Movement Disorders Centre, Toronto Western Hospital, University of Toronto, University Health Network, Toronto, Ontario, Canada;Movement Disorders Center, Department of Psychiatry and Neurology, University Hospital Centre of Grenoble, Grenoble, France;National Hospital of Neurology and Neurosurgery, Queen Square, London, United Kingdom;Neurologische Klinik und Poliklinik, Ludwig-Maximilians-Universität München, Munich, Germany;Neurology and Neurosurgical Department, Clinica Universidad de Navarra, Pamplona, Spain;Neurosciences, Alfred Hospital, Commercial Road, Melbourne, Victoria, Australia;Service de Neurochirurgie, CHU Bordeaux, Bordeaux, France;Service de Neurologie, CH François Mitterrand, Pau, France;Service de Neurologie, CHU Bordeaux, Bordeaux, France;Service de Neurophysiologie Clinique, CHU Bordeaux, Bordeaux, France;Servicio de Neurología, Hospital General Universitario Gregorio Marañón, Madrid, España;Sobell Department of Motor Neuroscience and Movement Disorders, University College London, Institute of Neurology, Queen Square, London, United Kingdom;Unit of Functional Neurosurgery, Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, United Kingdom;Univ.Bordeaux-INSERM U862, Neurocentre Magendie, Bordeaux, France;Van Cleef Roet Centre for Nervous Diseases, Monash University, Melbourne, Victoria, Australia

关键词: Surgical and invasive medical procedures;    Dystonia;    Movement disorders;    Deep-brain stimulation;    Functional electrical stimulation;    Adverse events;    Huntington disease;    Parkinson disease;   

DOI  :  10.1371/journal.pone.0079241

学科分类：医学（综合）

来源: Public Library of Science

【 摘 要 】

BackgroundChorea-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. MethodsData 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). ResultsMotor 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. ConclusionThis study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc.

【 授权许可】

CC BY