• 13 SECTIONDisorders Eyelid

Apraxia of Eyelid Opening

Key Facts

•Impaired or delayed ability to open eyes because of cerebral failure to recruit levator palpebrae superioris function

•Manifests as bilateral prolonged eyelid closure often provoked by bright light or touch on brow

•A sign of frontal lobe dysfunction (frontal release sign)

•Always accompanied by other signs of parkinsonism:

•Parkinson disease

•progressive supranuclear palsy

•multi-infarct state

•multiple system atrophy

•Alzheimer, Huntington, and Wilson diseases

•May be mistaken for ptosis, blepharochalasis, or essential blepharospasm

•Will not benefit from botulinum toxin injections unless there is also a blepharospasm component

Clinical Findings

•Prolonged bilateral eyelid closure occurring spontaneously or after exposure to bright light or touch of brow

•Eye opening is very slow to command

•Manual attempt to open eyes only worsens eyelid closure

•There are always other parkinsonian features:

•facial immobility

•bradykinesia

•hypophonia

•tremor

•stance and gait instability

•cognitive impairment

Ancillary Testing

• Not necessary if parkinsonian state already evaluated

Differential Diagnosis

• When parkinsonian features are present, there is no differential diagnosis

Treatment

•May benefit from orbicularis oculi botulinum toxin injections only if there is a component of blepharospasm

Prognosis

•Eyelid closure is rarely disabling—other features of the parkinsonian state are more problematic

A

0

B

0

30 seconds

Fig. 13.3 Apraxia of eyelid opening. (A) The patient’s eyelids are closed but not in spasm. (B) Thirty seconds after being instructed to open her eyes, the eyelids gradually open. The eyelids are now in normal position.

Opening Eyelid of Apraxia

• 13 SECTIONDisorders Eyelid

Benign Essential Blepharospasm

Key Facts

•Idiopathic involuntary bilateral eyelid closure

•May start as photophobia

•Often exacerbated by social encounters

•May be associated with contraction of other facial muscles

•Generally no associated neurologic or systemic illness

•Often misdiagnosed initially as ptosis, blepharochalasis, or dry eye

•Temporarily alleviated by subcutaneous botulinum toxin injections

•Anxiolytic medications may be adjuvants but are rarely effective alone

•Insight psychotherapy has been disappointing

Clinical Findings

•Intermittent or constant bilateral blepharospasm

•Tic-like mouthing movements sometimes present

•No other pertinent abnormalities

Ancillary Testing

• Not necessary

Differential Diagnosis

•Ptosis

•Blepharochalasis

•Dry eye syndrome or other superficial keratopathies

•Chronic uveitis

•Generalized dystonia

•Apraxia of eyelid opening

Treatment

•Botulinum toxin injections subcutaneously in a standard array on brow, eyelids, and cheek bilaterally

•Anxiolytic agents may have adjuvant benefit

Prognosis

•Botulinum toxin injections generally provide relief for 3–4 months

•repeated injections generally effective and side effects (ptosis, exposure keratopathy, diplopia) are temporary and tolerable

A B

C

Fig. 13.4 Blepharospasm. (A) The patient’s eyelids are in spasm bilaterally.

(B) Botulinum toxin injections are given subcutaneously in the brow and eyelids. (C) One day later, the eyelid spasm has disappeared.

Blepharospasm Essential Benign