Apraxia of Lid Opening Clinical Presentation

Updated: Sep 20, 2018
  • Author: Marta Ugarte, MBBS, PhD, DPhil, FRCOphth; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

Apraxia of lid opening (ALO) can present spontaneously in otherwise healthy individuals. [11, 37] In patients with Parkinson disease, progressive supranuclear palsy, and Shy-Drager syndrome, Lepore and Duvoisin reported that extrapyramidal symptoms preceded the inability to open the eyelids by a mean of 9.7 years. [15]

ALO is typically a chronic disorder, but spontaneous remission has been reported in patients within 1 month of strokes involving the nondominant hemisphere. [38, 17, 39] In instances of ALO that may be drug induced, symptoms have been reported to remit within 2 weeks of withdrawal of the agent [21] ; however, in one individual, the symptoms persisted for 7 months. [22]

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Physical Examination

The inability to reopen the lids is not evident during spontaneous or reflex blinking. The premotor pathways that regulate and control the state of voluntary activity of the levator palpebrae superioris (LPS) and make it parallel with OO inhibition differ, at least in part, from the pathways of spontaneous and reflex blinking.

A man with apraxia of lid opening is unable to open his lids at will. Eye movements were full. Attempted eye opening resulted in frontalis muscle contraction, backward thrusting of the head, and pretarsal orbicularis oculi activity. Spontaneous reflex blinking was normal. The lids remained open following manual elevation.

Once the lids have been opened, however, the patient has no difficulty in keeping them open. LPS tonic activity is normal while the lids are open.

Intermittent involuntary closure of the eyes may occur in some patients, both in the presence and in the absence of spasmodic contractions of the OO in blepharospasm. ILPI not accompanied by blepharospasm may cause the eyelid to drop and to be kept closed as long as inhibition of the LPS activity persists.

Attempted eye opening can result in forceful contraction of the frontalis, backward thrusting of the head, and delay in lid closure.

Different tricks may be used to help open the eyes, such as manually lifting the lids, opening the mouth, lightly touching the temporal region, and massaging the lids. The physiology of these maneuvers is unknown.

Some patients have been reported to have associated supranuclear limitation of eye movements. [15] In general, the coordination of eyelid movement is preserved.

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