Improvement of severe trunk spasms by bilateral high-frequency stimulation of the motor thalamus in a patient with chorea-acanthocytosis.
We report on a patient with a severe form of chorea-acanthocytosis, intractable to medical treatment, who benefited from bilateral high-frequency stimulation (HFS) of the posterior ventral oral nucleus of the thalamus. The frequency of trunk spasms dramatically decreased after surgery and the clinical benefit remained stable 1 year later. However, no clear effect was observed on dysarthria nor on hypotonia, which always impaired gait. We propose that HFS of the motor thalamus is a potential treatment for choreic or truncal dystonic symptoms whenever hypotonia is not the main feature of the syndrome.
Burbaud
Pierre
P
Service de Neurophysiologie Clinique, Centre Hospitalier Universitaire, Bordeaux, France. Pierre.Burbaud@umr5543.u-bordeaux2.fr
Rougier
Alain
A
Ferrer
Xavier
X
Guehl
Dominique
D
Cuny
E
E
Arne
Pierre
P
Gross
Ch
Ch
Bioulac
B
B
D000328:Adult; D002819:Chorea / Q000150:complications*; D004599:Electric Stimulation Therapy / Q000379:methods*; D004567:Electrodes, Implanted; D004576:Electromyography; D006801:Humans; D008297:Male; D018482:Muscle, Skeletal / Q000503:physiopathology*; D012720:Severity of Illness Index; D013035:Spasm / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D013788:Thalamus / Q000601:surgery*; D014743:Videotape Recording