The “Drama Mask” Phenomenon in Craniofacial Dystonia
Matthew Remz1, Jason Chan1, Alfonso Enrique Martinez Nunez1, Irene Malaty1
1University of Florida
Objective:
To highlight an interesting case of craniofacial dystonia, which caused the patient to have facial expressions incongruent with her emotions, and to demonstrate its response to botulinum toxin injections.
Background:
Dystonia involves sustained or intermittent muscle contractions that are often exacerbated by voluntary actions. Craniofacial dystonia can interfere with facial expression and social interaction, although this may be underappreciated and underreported in the literature. 
Design/Methods:
Case report.
Results:

The patient is a 52-year-old woman with a 4-year history of involuntary eyelid closure. Based on her complaint of “heavy” eyelids that wouldn’t stay open, she was initially diagnosed with seronegative myasthenia gravis and treated with medications and thymectomy. The lack of improvement led to subspecialty consult and a diagnosis of blepharospasm.  Her symptoms progressed to involve involuntary contractions of other facial muscles, consistent with craniofacial dystonia. When she chewed food, she had stereotyped eyelid clenching, nose elevation, and lip puckering. In pleasant social settings, people would consistently ask her if she was disgusted with her food. Conversely, when angrily scolding her children, she noticed paradoxical smiling, which diminished the emotion she was trying to convey. These “drama masks” resulted in a frustrating mismatch between her emotional state and outward expression. Clonazepam had minimal impact. Botulinum toxin injections were progressively tailored, and ultimately alleviated her craniofacial dystonia and “drama mask” phenomenon. Injections included the bilateral orbicularis oculi, corrugator supercilii, procerus, levator labii superioris aleque nasi, risorius, mentalis, orbicularis oris, masseters, temporalis, and sternocleidomastoid. Video will demonstrate facial muscle activation during eating, before and after botulinum toxin injections.

Conclusions:

This case report shows that craniofacial dystonia can impact outward expression of emotions, even producing emotionally incongruent facial expressions. This “drama mask” phenomenon can be socially disabling, but is treatable with targeted botulinum toxin injections.

10.1212/WNL.0000000000206462