Clinical and electromyographic features of levator palpebrae superioris muscle dysfunction in involuntary eyelid...

We report on five patients with involuntary eyelid closure, diagnosed as blepharospasm and referred to use for treatment with botulinum A toxin. Synchronous electromyographic (EMG) recording was performed from the levator palpebrae superioris (LP) and the orbicularis oculi (OO) muscles. In the first two cases, EMG registration showed alternating, semirhythmic dystonic activities in both the LP and OO, clinically perceptible as "flickering" of the eyelids. While the eyelids were lowered, one of them also showed involuntary upper eyelid tractions due to dystonic activities of LP. In the third patient, EMG patterns were characterized by a gradual decrease in the level of LP activity, followed by the contraction of OO, which facilitated the return of LP to its tonic activity, termed "postinhibition potentiation" of LP. In the fourth patient, EMG recording showed involuntary inhibition of LP in combination with blepharospasm. Involuntary closure of the eyelids in the fifth patient was caused by short or prolonged periods of involuntary LP inhibition, whereas OO activity remained normal. Our results provide further evidence that LP muscle activities are regulated by burst-tonic motoneurons, and we suggest that these motoneurons, and/or the input signals regulating their activities, can be involved independently in a pathological process. Clinical symptoms are discussed that may be helpful to recognize those cases with LP motor dysfunction, whether or not accompanied by OO activity disorders. Because the term blepharospasm indicates an abnormal motor function of OO, we propose "blepharospasm-plus" to designate those cases with a combined motor dysfunction of LP and OO muscles.