Apraxia of Eyelid Opening

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Article initiated by:

Neel Vaidya, MD

All contributors:

Michael T Yen, MD, Neel Vaidya, MD, Cat Nguyen Burkat, MD FACS

Assigned editor:

Michael T Yen, MD

Review:

Assigned status Up to Date

 by Michael T Yen, MD on August 2, 2024.

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Contributing Editors:	add

Apraxia of Eyelid Opening

OMIM

603119

Disease Entity

Disease

Apraxia of eyelid opening is defined as “nonparalytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi muscle.”Abnormalities of eyelid function have been described in the literature as far back as 1907, where Lewandowsky described apraxia of eyelid closure. While Schilder and Riese both described apraxias of eyelid closure and opening in 1927 and 1930 in neurology literature, Goldstein et al were the first to describe apraxia of eyelid opening in ophthalmologic literature in 1965.

Mechanism

Elevation of the eyelid involves simultaneous activation of the levator palpebrae superioris muscle and inhibition of the orbiculares oculi muscle. Apraxia of eyelid opening can result from involuntary inhibition of levator function, prolonged contraction of the orbicularis, or both. The classic finding of inability to open the lids after closure is postulated to be caused by persistent contraction of the activated orbicularis oculi muscle.

Tozlovanu et al studied the role of orbicularis contraction in apraxia of eyelid opening by using electromyography to measure orbicularis activity and latency of lid opening. This study demonstrated a strong correlation between time to orbicularis oculi inhibition and the completion of lid opening in both the control group and the study group (patients with ALO), with an abnormal persistence of orbicularis oculi function present in 10 of the 11 patients with ALO.

Causes/Associated Syndromes

Though ALO has been described in isolation, it is also associated with other conditions such as benign essential blepharospasm, progressive supranuclear palsy, dystonic parkinsonian syndrome/parkinsonism, motor neuron disease, and Shy-Drager syndrome. ALO has also been associated with CNS lesions in various locations, including the non-dominant hemisphere, medial frontal lobe, basal ganglia, and rostral brain stem. Additionally, isolated case reports of medication-induced ALO (including lithium, sulpiride, and MPTP), as well as sleep-induced ALO, exist in the literature.

Diagnosis

Clinical Presentation

ALO typically presents with the inability to open the lids following either voluntary or involuntary lid closure. Eye opening is, however, typically normal after reflex blinking. Additionally, forceful contraction of the frontalis muscle upon attempted opening has been reported as a feature of ALO, though it is not present in all cases. ALO is often accompanied by blepharospasm, though case reports do exist of isolated ALO without the presence of blepharospasm. In patients with essential blepharospasm, the combination of spasmodic closure of the eyelids and levator inhibition cause difficulty with opening the lids after forced closure.

Diagnostic Approach

The diagnosis of ALO is generally made clinically by identification of the hallmark physical exam findings described above. Brain imagining in the form of CT or MRI may be ordered in order to isolate a causal lesion. Reports of the use of positron emission tomography (PET scanning) to identify areas of neuronal glucose hypometabolism in the basal ganglia, frontal lobe, or primary visual cortex do exist in the literature. Electromyography (EMG) may also be helpful in diagnosis and to distinguish the presence or absence of concurrent blepharospasm. Thorough evaluation for associated systemic/neurologic diseases should also be pursued.

Management

Treatment and Prognosis

Historically, the mainstay of treatment for ALO (with or without concurrent blepharospasm) has been injection of botulinum toxin into the pre-tarsal orbicularis oculi muscle. Other treatment options including levodopa, trihexyphenidyl, and valproic acid have been described in the literature, although results are limited.

Several reports exist detailing the successful use of frontalis suspension and upper eyelid myectomy as surgical alternatives for some patients with ALO. These procedures have also been described in combination with medical therapy.