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Symptoms ⬇

Uncontrolled blinking, twitching, or closure of the eyelids. Always bilateral, but may briefly be unilateral at the first onset. Occasionally may have mid to lower face and/or neck spasms. Can also be associated with oromandibular dystonia that results in spasms in the jaw and tongue, as well as laryngeal and cervical dystonia, a condition referred to as Meige syndrome.

Signs ⬆ ⬇

Critical

Bilateral, episodic, and involuntary contractions of the orbicularis oculi muscles.

Other

Disappears during sleep.

Differential Diagnosis ⬆ ⬇

  • Hemifacial spasm: Unilateral contractures of the entire side of the face that do not disappear during sleep. Usually idiopathic but may be related to prior CN VII palsy, injury at the level of the brainstem, or compression of CN VII by a blood vessel or tumor. MRI of the cerebellopontine angle should be obtained in all patients to rule out tumors. Treatment options include observation, botulinum toxin injections, or neurosurgical decompression of CN VII.
  • Ocular irritation induced blepharospasm (e.g., corneal or conjunctival foreign body, trichiasis, blepharitis, iritis, and dry eye).
  • Eyelid myokymia: Subtle eyelid twitch felt by the patient but difficult to observe, commonly brought on by stress, caffeine, alcohol, or ocular irritation. Usually unilateral lower eyelid involvement. Typically self-limited. Treat by avoiding precipitating factors and/or administering small doses of botulinum toxin.
  • Tourette syndrome: Multiple compulsive muscle spasms associated with utterances of bizarre sounds or obscenities.
  • Tic douloureux (trigeminal neuralgia): Acute episodes of pain in the CN V distribution, often causing a wince or tic.
  • Tardive dyskinesia: Orofacial dyskinesia, often with dystonic movements of the trunk and limbs, typically from long-term use of antipsychotic medications.
  • Apraxia of eyelid opening. Usually associated with Parkinson disease. Unlike blepharospasm, apraxia of eyelid opening does not feature orbicularis spasm. Instead, apraxic patients simply cannot open their eyelids voluntarily.

Etiology ⬆ ⬇

  • Idiopathic and likely multifactorial, possibly involving dopaminergic pathways within the basal ganglia.

Work Up ⬆ ⬇

Workup
  1. History: Unilateral or bilateral? Does the episode involve the eyelids alone or is the lower face also involved? Are limb muscles involved? Medications?
  2. Slit lamp examination: Examination for dry eye, blepharitis, or foreign body.
  3. Neuroophthalmic examination to rule out other accompanying abnormalities.
  4. Typical blepharospasm does not require central nervous system imaging as part of the workup. MRI of the brain with attention to the posterior fossa and path of CN VII is reserved for atypical cases or other diagnoses (e.g., hemifacial spasm).

Treatment ⬆ ⬇

  1. Treat any underlying eye disorder causing ocular irritation. See 4.3, DRY EYE SYNDROME and 5.8, BLEPHARITIS/MEIBOMITIS.
  2. Consider botulinum toxin (onabotulinumtoxinA, incobotulinumtoxinA, and abobotulinumtoxinA) injections into the orbicularis muscles around the eyelids if the blepharospasm is severe. Can also be used to treat orofacial dyskinesia.
  3. If the spasm is not relieved with botulinum toxin injections, consider surgical excision of the orbicularis muscle (myectomy) from the upper and lower eyelids and brow.
  4. Muscle relaxants and sedatives are rarely of value but can be helpful in some patients. Oral medications such as lorazepam can help, but their use is often limited by their sedative qualities. Oral methylphenidate may be helpful in patients with severe spasm.

Follow Up ⬆

Not an urgent condition, but patients with severe blepharospasm can be functionally blind.