BLEPHAROSPASM
Philip A. Hanna, M.D.
Parkinson's Disease and Movement Disorders Center
New Jersey Neuroscience Institute
Edison, New Jersey
Blepharospasm is a form of focal dystonia (dystonia refers to
involuntary sustained, patterned, twisting, pulling or squeezing
movements) characterized by involuntary contractions of the
orbicularis oculi (facial muscles surrounding the eyes) leading to
forceful closure of eyelids. Blepharospasm is often associated with
dystonia of other facial and neck muscles, named
"cranial-cervical dystonia" which is preferred over the
older term, Meige syndrome. Before sustained closure of the eyelids
develops, nearly a third of patients report increased blinking, often
with a sense of irritation of the eyes. Thus, many patients are
initially diagnosed with "dry eyes".
Blepharospasm may interfere with daily activities such as reading,
driving and watching television. Up to two thirds of patients are
functionally blind due to their blepharospasm. Blepharospasm is
usually worsened by bright light, thus some patients wear sunglasses
constantly. The spasms may be transiently relieved by "sensory
tricks" such as pulling on an eyelid or eyebrow, singing,
sleeping, relaxation, reading, concentration, looking down and other
maneuvers. Most cases of blepharospasm are believed to be genetic in
nature. A number of gene loci have been identified for certain types
of dystonia, and a gene mutation has been discovered (Ozelius et al,
1997].
Botulinum toxin (BTX) injections into the eyelids and eyebrows is
considered by many to be the treatment of choice, providing moderate
or marked functional improvement in over 90% of patients. The average
time following an injection to the onset of improvement is 2 to 5 days
and the average duration of benefit is 3 to 4 months. Although nearly
10% of all treatment sessions have some side effects such as ptosis
(drooping of an eyelid), blurring of vision or double vision, tearing,
and local bruising, the complications are usually mild, rarely affect
patients' functioning and typically resolve in less than 2 weeks.
Though BTX is seemingly the most effective treatment, some patients
derive relief from medications such as clonazepam, trihexyphenidyl,
lorazepam, baclofen and tetrabenazine.
Contact: Benign Essential Blepharospasm Research Foundation, Inc.
P.O. Box 12468
Beaumont, Texas 77726-2468
(409) 832-0788, Fax: (409) 832-0890, E-mail: bebrf@ih2000.net
Hanna PA, Cardoso F, Jankovic J. Basal Ganglia and Movement Disorders.
In: Rolak LA, ed. Neurology Secrets, 2nd ed. New York: Hanley and
Belfus, 1998:137-169.
Ozelius LJ, Hewett JW, Page CE, et al. The early onset torsion
dystonia gene [DYT1] encodes an ATP-binding protein. Nature Genet
1997;17:40-48.
Cardoso F, Jankovic J. Blepharospasm. In: Tsui J, Calne DB, eds.
Handbook of Dystonia, Marcel Dekker, New York, NY, 1995:129-141.
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