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Inhibition of Blepharospasm in a CPEO Patient by Unilateral Enhanced Levator Innervation
1Bau V., 2Neudecker S., 1Weidlich R., 1Duncker G. I. W., 2Zierz S.,
1Martin-Luther-Universität, Klinik und Poliklinik für Augenheilkunde (Halle/Saale)
2Martin-Luther-Universität, Neurologische Universitätsklinik (Halle/Saale)
Purpose: Various forms of movement disorders has been discribed in association with mitochondrial defects. So far, blepharospasm in mitochondrial chronic progressive external ophthalmoplegia, however, has only rarely been discribed.
Case report: A 39 year old patient with CPEO developed bilateral blepharospasm involving facial muscles following 3 years after unilateral frontalis suspension on the left. At that time ptosis was more severe on the left side. Examination: Visual acuity R 0,8, L 0,6 (retina degeneration). Motility of both eyes almost completely lost. Persistent preferential fixation with left eye. Severe ptosis right covering pupil, mild divergence strabism right. Persistant blepharospasmus. Covering the left eye and subsequent fixation by the right eye resulted in improvement of ptosis and inhibition of blepharospasm. (Video.)
Discussion: Origin of blepharospasm in the present patient might either be due to a functional dysbalance following unilateral ptosis surgery or might be a rare symptom of the mitochondrial disorder. It is peculiar that the blepharospasm can be inhibited. This could be due to increased activity of the nervus oculomotorius when fixating with the more ptotic eye. This inhibition phenomenon might be analogous to geste antagonistige in spasmodic torticollis.
Conclusions: Therapeutical options are ptosis surgery and botulinum toxin. Due to the inhibition phenomenon, ptosis and blepharospasm might improved by stabil fixation by the right eye following artificial impairment of visual acuity of the left eye (partial optical occlusion).