Blepharospasm

Blepharospasm is involuntary closure of the eye(s) due to contraction of the orbicularis oculi and other muscles around the eyes. (1-3) It is most often idiopathic or “essential.” Benign essential blepharospasm is always bilateral and fairly symmetric, repetitive, transient and involuntary (video). Patients suffer attacks of involuntary eye closure multiple times daily that become more frequent and more sustained with the passage of time, and may interfere with vision. One patient was stopped by the police for driving with her eyes closed. The condition preferentially affects women and has a peak age at onset in the 6th decade. (3) Increased blinking may be part of the disorder. Some patients find a sensory trick to help alleviate the blinking, such as touching a certain area of the face, pulling on the upper eyelid or an eyebrow, wearing tinted lenses, talking or singing. (3)

Blepharospasm is often treated with botulinum toxin. The condition is classified as a focal dystonia. In Meige’s (Breugel’s) syndrome, blepharospasm is accompanied by oromandibular dystonia.

Similar movements sometimes occur as a tic, habit spasm or stereotypy, especially in adolescents. Blepharospasm may be confused with apraxia of eyelid opening and some patients with essential blepharospasm may have a component of apraxia of eyelid opening characterized by transient failure to voluntarily reopen the eyes without orbicularis spasm. (3) Rarely, the movements in hemifacial spasm are more prominent in the upper face and appear to cause unilateral blepharospasm, but subtle lower facial movements are always present in addition. Facial synkinesis (see http://neurosigns.org/wiki/Facial_synkinesis), as after Bell's palsy, may cause similar confusion (Marin Amat sign).

Video legend. Blepharospasm and blepharospasm with oromandibular dystonia (Meige’s (Breugel’s) syndrome). Video courtesy of Dr. Stephen G. Reich

References

1. Campbell WW. Barohn RJ. DeJong's the neurologic examination, 8th ed. Philadelphia: Wolters Kluwer, 2020.

2. Campbell WW. Clinical signs in neurology: a compendium. Philadelphia: Wolters Kluwer Health, 2016.

3. Defazio G, Hallett M, Jinnah HA, Conte A, Berardelli A. Blepharospasm 40 years later. Mov Disord. 2017;32:498-509