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Update on blepharospasm

Report from the BEBRF International Workshop

From the Human Motor Control Section (M.H.), NINDS, NIH, Bethesda, MD; Department of Neurobiology & Behavior (C.E.), SUNY Stony Brook, NY; Parkinson’s Disease Center and Movement Disorders Clinic (J.J.), Department of Neurology, Baylor College of Medicine, Houston, TX; and Neurology Department (M.S.), Duke University Medical Center, Durham, NC.

Address correspondence and reprint requests to Dr. Mark Hallett, Human Motor Control Section, NINDS, NIH, Building 10, Room 5N226, 10 Center Drive MSC 1428, Bethesda, MD 20892-1428 hallettm{at}ninds.nih.gov

This review updates understanding and research on blepharospasm, a subtype of focal dystonia. Topics covered include clinical aspects, pathology, pathophysiology, animal models, dry eye, photophobia, epidemiology, genetics, and treatment. Blepharospasm should be differentiated from apraxia of eyelid opening. New insights into pathology and pathophysiology are derived from different types of imaging, including magnetic resonance studies. Physiologic studies indicate increased plasticity and trigeminal sensitization. While botulinum neurotoxin injections are the mainstay of therapy, other therapies are on the horizon.

Abbreviations: BFMDRS = Burke-Fahn-Marsden dystonia rating scale; BoNT = botulinum neurotoxin; DBS = deep brain stimulation; DTI = diffusion tensor imaging; FDG = ¹⁸fluorodeoxyglucose; VBM = voxel-based morphometry.

Supported by the NIH Intramural Program (M.H.) and NIH grant EY07391 (Control of eyelids in normal and pathological states) (C.E.). Dr. Jankovic has received research grants from the following: Advanced Neuromodulation Systems; Allergan, Inc.; Boehringer-Ingelheim; Ceregene, Inc.; EMD; Eisai; Ipsen Limited; Huntington’s Disease Society of America; Kyowa Pharmaceuticals; Medtronic; Merz Pharmaceuticals; National Institutes of Health; National Parkinson Foundation; Novartis; Ortho-McNeil; Parkinson Study Group; Prestwick Pharmaceuticals; Schering; Schwarz Pharma/UCB; Teva.

Disclosure: Author disclosures are provided at the end of the article.

Received January 14, 2008. Accepted in final form June 3, 2008.