Inability to Close Eyelids as a Feature of Palpebral Dystonia
We report the case of a patient with a severe Meige syndrome with paradoxical difficulties keeping his eyes closed.
Case Report
A 54-year-old man with a 4-year history of severe idiopathic Meige syndrome complained of difficulties keeping his eyes closed, which led to problems in getting to sleep. When trying to sleep, he had to force the lids closed with his fingers. When sitting or lying relaxed, no dystonic activity of his face was visible. Upon closing his lids, they would involuntarily reopen after a few seconds. There was n o visible Bell's phenomenon, but on forceful lid closure with contraction of the orbital orbicularis oculi, Bell's phenomenon was preserved. His difficulty in closing his eyes occurred both sitting and lying. Blink frequency was markedly increased. Blinking to a visual threat, to glabellar tap, or to corneal stimulus was increased, and these stimuli generally provoked blepharospasm. Forceful eye closure provoked blepharospasm, followed by difficulties in reopening the lids, with voluntary frontalis contraction, as in focal eyelid dystonia.
The patient was functionally blind when trying to read, using a phone, or crossing a street. In order to prevent blepharospasm, he voluntarily looked down when walking.
On needle E M G recording from the levator palpebrae and orbicularis oculi muscles, voluntary lid closure led to dystonic activity of the levator palpebrae with typical 100to 500-ms bursts of E M G activity and irregular pauses of activity. Co-contraction of the orbicularis oculi and levator palpebrae muscles was recorded. Whereas activity of the levator palpebrae was provoked by voluntary lid closure and blepharospasm by actively looking, perioral dystonia with closure of the orbicularis oris muscle and jaw-opening dystonia were induced by talking and by eating. The blepharospasm was moderately improved after a first injection of botulinum toxin into the orbital orbicularis oculi and markedly improved (subjective improvement 80%) after a subsequent pretarsal injection, whereas the inability to keep the eyes closed was unchanged.
Discussion
We report the case of a patient with involuntary eyelid opening as one of the presenting complaints of his idio-