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Original Investigation
November 2016

Use of Alleviating Maneuvers for Periocular Facial Dystonias

Author Affiliations
  • 1St Mary’s Hospital, London, England
  • 2Adnexal Department, Moorfields Eye Hospital, London, England
  • 3Instituto Clinic de Oftalmologia, Hospital Clinic, Barcelona, Spain
JAMA Ophthalmol. 2016;134(11):1247-1252. doi:10.1001/jamaophthalmol.2016.3277
Key Points

Question  Is the use of alleviating maneuvers by patients with benign essential blepharospasm (BEB) or hemifacial spasm (HFS) associated with disease severity or botulinum toxin treatment?

Finding  The use of alleviating maneuvers was associated with more severe disease in both patients with BEB and patients with HFS, but not with increased use of botulinum toxin.

Meaning  Although this association is not proof of cause and effect, patients with BEB or HFS with severe disease may benefit from a multimodal approach to their care, including advice on augmenting their maneuvers or tailoring devices to mimic their maneuvers.

Abstract

Importance  Patients with benign essential blepharospasm or hemifacial spasm are known to use botulinum toxin injections and alleviating maneuvers to help control their symptoms. The clinical correlates between the use of botulinum toxin injections and the use of alleviating maneuvers are not well established.

Objective  To determine whether the use of alleviating maneuvers for benign essential blepharospasm or hemifacial spasm correlates with disease severity or botulinum toxin treatment.

Design, Setting, and Participants  A prospective cross-sectional observational study (designed in September 2013) of 74 patients with benign essential blepharospasm and 56 patients with hemifacial spasm who were consecutively recruited from adnexal clinics at Moorfields Eye Hospital (January-June 2014) to complete a questionnaire and undergo a clinical review. Data analysis was performed in December 2015.

Main Outcomes and Measures  Prevalence and type of alleviating maneuvers used for blepharospasm and hemifacial spasm, dystonia severity, and dose and frequency of botulinum toxin injections.

Results  Of the 74 patients with blepharospasm, 39 (52.7%) used alleviating maneuvers (mean [SD] age, 70.4 [9.1] years); of the 56 patients with hemifacial spasm, 25 (44.6%) used alleviating maneuvers (mean [SD] age, 66.5 [12.7] years). The most commonly used maneuver was the touching of facial areas (35 of 64 patients [54.7%]); other maneuvers included covering the eyes (6 of 64 patients [9.4%]), singing (5 of 64 patients [7.8%]), and yawning (5 of 64 patients [7.8%]). Patients with blepharospasm who used alleviating maneuvers scored higher on the Jankovic Rating Scale (median score, 5 vs 4; Hodges-Lehmann median difference, 1 [95% CI, 0-2]; P = .01) and the Blepharospasm Disability Index severity score (median score, 11 vs 4; Hodges-Lehmann median difference, 4 [95% CI, 1-7]; P = .01) than patients with blepharospasm who did not use alleviating maneuvers. Patients with hemifacial spasm who used alleviating maneuvers scored higher on the 7-item Hemifacial Spasm Quality of Life scale (median score, 7 vs 3; Hodges-Lehmann median difference, 4 [95% CI, 1-7]; P = .01) and the SMC Severity Grading Scale (median score, 2 vs 2; Hodges-Lehmann median difference, 0 [95% CI, 0-1]; P = .03) than patients with hemifacial spasm who did not use alleviating maneuver. The severity of dystonia correlated with botulinum toxin treatment for patients with blepharospasm (r = 0.23; P = .049) and patients with hemifacial spasm (r = 0.45; P = .001). There was no difference found in botulinum toxin treatment between patients who used alleviating maneuvers and those who did not, in either the blepharospasm group (150 vs 125 units; Hodges-Lehmann median difference, 20 units [95% CI, −10 to 70 units]; P = .15) or the hemifacial spasm group (58 vs 60 units; Hodges-Lehmann median difference, 0 units [95% CI, −15 to 20 units]; P = .83).

Conclusions and Relevance  Half of the patients with periocular facial dystonias used alleviating maneuvers. Their use was associated with more severe disease but not with increased use of botulinum toxin. This may help to guide future therapies, such as advice on maneuver augmentation or tailored devices.

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