Severe tongue protrusion dystonia: clinical syndromes and possible treatment.
We describe intermittent or sustained severe involuntary tongue protrusion in patients with a dystonic syndrome. Speech, swallowing, and breathing difficulties can be severe enough to be life threatening. Causes include neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, Lesch-Nyhan syndrome, and postanoxic and tardive dystonia. The pathophysiology of intermittent severe tongue protrusion remains unknown. Tongue protrusion dystonia is often unresponsive to oral drugs but may benefit from botulinum toxin injections into the genioglossus muscle. Bilateral deep brain pallidal stimulation was beneficial in two cases.
Schneider
S A
SA
Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, London WC1N 3BG, UK.
Aggarwal
A
A
Bhatt
M
M
Dupont
E
E
Tisch
S
S
Limousin
P
P
Lee
P
P
Quinn
N
N
Bhatia
K P
KP
D000293:Adolescent; D000328:Adult; D018726:Anti-Dyskinesia Agents / Q000627:therapeutic use*; D001905:Botulinum Toxins / Q000627:therapeutic use*; D020821:Dystonic Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008538:Meige Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D008875:Middle Aged; D014059:Tongue
D018726:Anti-Dyskinesia Agents; D001905:Botulinum Toxins