Management of Oculofacial Dystonias
Michael Ehrlich, MD
DISEASE DESCRIPTION
The most common spastic conditions of the eyelid and face include benign essential blepharospasm (BEB), hemifacial spasm (HFS), and aberrant regeneration with synkinesis after trauma or Bell’s palsy.
Benign Essential Blepharospasm (BEB)
Involuntary, bilateral spasms that tend to increase in strength over the course of the disease (Figure 18.1)
Spasm of orbicularis oculi, procerus, and corrugator. Consider Meige/Brueghel syndrome if oromandibular dystonia is present.
Rule out secondary blepharospasm from dry eye, trichiasis, and other ocular surface and intraocular pathology.
Occurs more in women than in men, onset commonly in those older than age 40
Can lead to functional blindness in otherwise well-seeing patients
 FIGURE 18.1. Benign essential blepharospasm is characterized by episodic contraction of the eyelid protractors (orbicularis oculi, corrugators, and procerus). |
 FIGURE 18.2. Left hemifacial spasm. Hemifacial spasm is characterized by involuntary tonic and clonic contractions of the mimetic muscles on one side of the face. |
Hemifacial Spasm (HFS)
Unilateral, synchronous contracture of the entire side of the face (Figure 18.2)
Present during sleep
Most common etiology is vascular compression of facial nerve root at the pontomedullary region of the brainstem
Order contrasted magnetic resonance imaging (MRI) of the skull base to rule out brainstem lesion.
Similar contractions may also be observed after aberrant regeneration following trauma or Bell’s palsy.
Many patients benefit from chemodenervation treatment of the affected muscles.
MANAGEMENT OPTIONS
Benign Essential Blepharospasm Research Foundation support group (www.blepharospasm.org) offers excellent patient resources.
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