Progressive Supranuclear Palsy with Apraxia of Eyelid Opening
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Apraxia of lid opening (ALO) is a poorly recognized and highly disabling disorder which causes inability to voluntarily open the eyes. It often occurs together with blepharospasm but may also occur alone. It occurs most commonly in patients with extrapyramidal disorders, the most common of which is progressive supranuclear palsy (PSP) (see Chap. 16). It has also been associated with multiple system atrophy, Parkinson’s disease, and frontal lobe injuries or stroke. Although often confused with upper eyelid ptosis, ALO occurs in the absence of lid levator weakness. The lids typically open more easily during reflex blinking and can be assisted to open by various maneuvers such as brushing the eyelids with the hand, opening the mouth, or prying open the lids with the fingers. ALO was initially attributed to involuntary levator palpebrae inhibition. However, electromyographic studies using microelectrode recordings later showed that most cases are due to persistent contraction of the pretarsal portion of the orbicularis oculi referred to as pretarsal motor persistence
KeywordsBotulinum Toxin Multiple System Atrophy Progressive Supranuclear Palsy Progressive Supranuclear Palsy Botulinum Toxin Injection
17 Progressive Supranuclear w. ALO.mp4 (MP4 15,609KB)
The patient exhibits slow voluntary elevation of upper eyelids, slow saccadic eye movements involving vertical more than horizontal gaze and, at the time of this video, only mild hesitation opening her eye due to recent treatment with botulinum toxin. Other features of PSP include hyperextension of the neck, facial masking, and monotonic voice.