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


CONCLUSIONS
This study shows that bilateral DBS of the GPi effectively reduces the severity of drug-resistant hyperkinetic movement disorders such as present in ChAc.
Service de Neurologie, CHU Bordeaux, Bordeaux, France ; Service de Neurologie, CH François Mitterrand, Pau, France.
Marie
M
Miquel
Umberto
U
Spampinato
Chrystelle
C
Latxague
Iciar
I
Aviles-Olmos
Benedikt
B
Bader
Kelly
K
Bertram
Kailash
K
Bhatia
Pierre
P
Burbaud
Lothar
L
Burghaus
Jin Whan
JW
Cho
Emmanuel
E
Cuny
Adrian
A
Danek
Thomas
T
Foltynie
Pedro J
PJ
Garcia Ruiz
Santiago
S
Giménez-Roldán
Dominique
D
Guehl
Jorge
J
Guridi
Marwan
M
Hariz
Paul
P
Jarman
Zinovia Maria
ZM
Kefalopoulou
Patricia
P
Limousin
Nir
N
Lipsman
Andres M
AM
Lozano
Elena
E
Moro
Dhita
D
Ngy
Maria Cruz
MC
Rodriguez-Oroz
Huifang
H
Shang
Hyeeun
H
Shin
Ruth H
RH
Walker
Fusako
F
Yokochi
Ludvic
L
Zrinzo
François
F
Tison
D000328:Adult; D001922:Brain Abscess / Q000209:etiology; D003430:Cross-Sectional Studies; D046690:Deep Brain Stimulation / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods*; D004567:Electrodes, Implanted; D005260:Female; D005917:Globus Pallidus / Q000503:physiopathology* / Q000601:surgery; D006801:Humans; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D054546:Neuroacanthocytosis / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy*; D016459:Prosthesis-Related Infections / Q000209:etiology; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult