Animal model explains the origins of the cranial dystonia benign essential blepharospasm.
The current study demonstrates that combining two mild alterations to the rat trigeminal reflex blink system reproduces the symptoms of benign essential blepharospasm, a cranial dystonia characterized by uncontrollable spasms of blinking. The first modification, a small striatal dopamine depletion, reduces the tonic inhibition of trigeminal reflex blink circuits. The second alteration, a slight weakening of the lid-closing orbicularis oculi muscle, begins an adaptive increase in the drive on trigeminal sensory-motor blink circuits that initiates blepharospasm. By themselves, neither of these modifications causes spasms of lid closure, but combined, they induce bilateral forceful blinking and spasms of lid closure. A two-factor model based on these rodent experiments may explain the development of benign essential blepharospasm in humans. The first factor, a subclinical loss of striatal dopamine, creates a permissive environment within the trigeminal blink circuits. The second factor, an external ophthalmic insult, precipitates benign essential blepharospasm. This two-factor model may also be applicable to the genesis of other cranial dystonias.
Schicatano
E J
EJ
Department of Neurobiology and Behavior, State University of New York at Stony Brook, 11794-5230, USA.
Basso
M A
MA
Evinger
C
C
D000818:Animals; D001479:Basal Ganglia / Q000502:physiology; D001764:Blepharospasm / Q000503:physiopathology*; D001767:Blinking / Q000502:physiology*; D004195:Disease Models, Animal; D004292:Dominance, Cerebral / Q000502:physiology; D004298:Dopamine / Q000502:physiology; D004421:Dystonia / Q000503:physiopathology; D004576:Electromyography; D008297:Male; D009415:Nerve Net / Q000503:physiopathology; D009433:Neural Inhibition / Q000502:physiology; D051381:Rats; D017207:Rats, Sprague-Dawley; D012815:Signal Processing, Computer-Assisted; D014276:Trigeminal Nerve / Q000503:physiopathology*
D004298:Dopamine