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Eyelid Spasms

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					    In
                                                                                                               A QUARTERLY REPORT FOR

                                                                                                               HEALTH CARE PROFESSIONALS

                                                                                                               DELIVERING EYE CARE
                                                                                                               Volume 4, Issue 1      March 1998




    Eyelid Spasms
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    Part I – Essential Blepharospasm
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                                                          thr usting, tongue movements), the                   gum, talking continuously, rubbing the
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                                                          patients often have ver y unusual facial             eyelids or applying pressure to other areas
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                                                          expressions that are uncontrollable                  of the face, covering one eye, and solving
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                                                          (Figure 2). Dystonias may also                       puzzles or problems.
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                                                          occasionally occur elsewhere including
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                                                          the neck (torticollis, antecollis) or dystonic
                                                                                                                      What causes Essential
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                        by                                posturing of the limbs. Involuntar y
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                                                          vocalization, such as grunting, frequent                     Blepharospasm?
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                                                                                                                                                                ○
                        David R. Jordan
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                                                          throat clearing, and respiratory noises as           Blepharospasm is thought to be secondary
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                        M.D., F.A.C.S., F.R.C.S.(C)
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                                                          well as dysphagia and respir ator y                  to an abnormal functioning of the “basal
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                                                          difficulties may develop. In advanced
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                                                                                                               ganglia,” an area of the brain which plays
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               INTRODUCTION                               stages, patients with uncontrolled eyelid            an important role in the control of fine
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                                                          and facial spasms may become functionally            motor movements. The exact problem in
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    There are several different types of eyelid           blind, socially reclusive, and unable to
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    spasms. The three most common are eyelid                                                                   this area is unknown but a neurochemical
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                                                          work or care for themselves.                         imbalance is suspected. In some cases it may
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    t witch ing (myok ym ia), Essential
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                                                                                                               be a familial disease with more than one
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    Blepharospasm, and Hemifacial Spasm.
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                                                                                                               family member affected. In other cases,
    Part I of Eyelid Spasms will focus on                         How does Essential
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                                                                                                               blepharospasm may be secondary to drug
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    Essential Blepharospasm while Part II will
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                                                               Blepharospasm present?                          therapy for other diseases (ex. medication
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    look at Myokymia and Hemifacial Spasm.
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                                                          The average age of onset is 56 years with            for Parkinson’s disease, major tranquilizers).
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                                                          females being more commonly affected
              What is Essential
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                                                          than males by 3:1. Blepharospasm usually
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              Blepharospasm?                                                                                                   THERAPY
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                                                          begins with a gradual increase in the blink
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    “Blepharo” comes from the Greek word                  rate that eventually progresses to forceful
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                                                                                                               Blepharospasm can be treated with oral
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                                                          eyelid spasms frequently throughout the
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    for eyelids. Blepharospasm is a condition                                                                  medication, injections of Botulinum toxin
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    in which there is involuntary blinking of             day. A f luctuating course characterized by          or surgery.
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    the eyelids that may at times be forceful             remissions and exacerbations is usual. It                                                             ○
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                                                                                                               Drug therapy is effective in fewer than 10%
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                                                          may be precipitated or aggravated by
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    and sustained (several seconds). Blepharo-
                                                                                                               of patients and may only give partial relief.
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    spasm is a type of “Dystonia”, a term used            bright light, stress, fatigue, driving,
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                                                          reading, watching TV, and a variety of               The response is unpredictable and side
    to describe “abnor mal, involuntar y,
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                                                                                                               effects may outweigh the benefits. A
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                                                          other activities. Sleep, relaxation training,
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    sustained muscle contrac tions and
                                                                                                               variety of medications have been tried
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    spasms”. When the eyelid spasms occur,                walking and talking may improve the
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                                                          spasms. Some patients learn to avoid                 includi ng antipsychotics, affective
    a temporary inability to see may result due
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                                                                                                               disorder agents, anxiolytic agents,
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                                                          situations that aggravate the condition,
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    to the forceful involuntary eyelid closure
                                                                                                               sedatives, drugs for Parkinson’s disease,
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    (Figure 1a and 1b). Patients with                     while others develop certain behavioral
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                                                          techniques that involve using other facial           muscle relaxants, etc.
    Blepharospasm have normal eyes. The
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                                                          muscles or acts of mental concentration              Botulinum toxin is produced by the
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    visual disturbance is due solely to the
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    forced closure of the eyelids. In some                to decrease the frequency and intensity of           bacteria “Clostridium botulinum”.
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    individuals, other muscles of the face may            spasms. Examples include humming,                    Botuli num toxin i nterferes with
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                                                          singing, whistling, yawning, coughing,               acetylcholine release from peripheral
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    be involved. With other facial spasms (peri-
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    oral contractions, lip pursing, chin                  mouth opening, nose pinching, chewing                motor ner ve ter m inals, resulting in
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                                                                                1
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                                                         Figure 1A
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                                                         Pre-spasm,
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                                                         eyelids open.
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                                                                                        Figure 1B
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                                                                                  Profound spasm,




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                                                                                         of eyelids
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                                                                                 (Blepharospasm).
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    temporar y paralysis of the injected               Side effects are infrequent and transient.           Blepharospasm patient with apraxia of lid
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    muscles. Minute doses of botulinum toxin       ○
                                                       They include ptosis, blurred vision,                 opening will typically have lid spasms,




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    are injected around the eyelid and facial          double vision, ocular irritation and                 squeezing the eyelids shut and then for
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    area with a fine needle. The effect usually        tear i ng. These side effec ts resolve               several seconds to minutes af ter the
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    occurs within the first few days and lasts         spontaneously in a matter of days to                 spasms stop, the patient is unable to open
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    an average of 3 to 4 months, at which time         weeks.                                               the lids. The eyelids may then come open
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    another injection is given. Muscle relaxants                                                            almost normally for a period of time and
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    (orphenadrine, baclofen) have been useful                 SUPPORTIVE THERAPY                            then without warning, slowly drop shut
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    in several patients as the effects of                                                                   again, or be drawn shut by spasms. The
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                                                       The psychologic impact of Essential




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    botulinum toxin start wearing off.                                                                      apraxia of lid opening patient can be seen
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                                                       Blepharospasm can be tremendous.
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                                                                                                            raising their brows and trying to open their
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    Long term follow up studies have shown




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                                                       Patients often feel their physicians do not
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                                                                                                            eyelids as strongly as possible without any




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    botulinum toxin injection to be a very safe        believe their symptoms are uncontrolled
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    and effective treatment with as many as                                                                 elevation of the eyelids. Apraxia is thought
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                                                       and therefore may see several physicians
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                                                                                                            to be due to a problem in the neuro-
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    90% of patients obtaining significant relief




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                                                       before the proper diagnosis is made. It is           circuitry for opening the eyelids, much
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    of their spasms. Repeated treatment                not unusual for a patient to be initially seen
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                                                                                                            like blepharospasm is a problem in the




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    generally remains effective over a
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                                                       by a physician who is unaware of this
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    prolonged period in most patients.                                                                      neuro-circuitry causing squeezing of the
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                                                       disease and refers them for psychiatric              lids.
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    However, in some individuals, botulinum            evaluation. They are generally greatly
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    toxin injections may become less effective                                                              To make a diagnosis of apraxia of lid
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                                                       relieved when they are finally told they         ○
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    over time, possibly because of a re-                                                                    opening, all Blepharospasm or squeezing
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                                                       have a real disease and that some treatment
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    sprouting of motor endplate receptors or                                                                of the eyelids must be relieved. Botulinum




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                                                       is available. It is extremely important for
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    the development of an antitoxin.                   patients to understand their disease                 toxin, myectomy or a combination thereof
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                                                                                                            must completely relieve muscle squeezing
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                                                       process. Patients generally learn their own
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                                                                                                            before a diagnosis of apraxia of the
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                                                       coping techniques which can be shared
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                                                       with others at support group meetings. A             opening can be confirmed.
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                                                       monthly newsletter and information on                Blepharospasm patients with apraxia are
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                                                       support groups can be obtained through               treated by a li m ited myectomy in
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                                                       the Benign Essential Belpharospasm                   conjunction with tightening of the levator
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                                                                                                            tendon (aponeurotic ptosis repair). By
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                                                       Research Foundation, address:
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                                                           BEBRF, Inc.,                                     tightening the tendon of the muscle that
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                                                                                                            raises the eyelids, patients can more
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                                                           P.O. Box 12468,
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                                                                                                            effectively open their lids. By removing the
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                                                                                                                                                           ○
                                                           Beaumont, Texas – 7726-2468
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                                                                                                            squeezing muscles in the upper eyelids
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                                                       At present there is no cure for Essential            (li m ited myec tomy), any residual
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                                                       Blepharospasm and related dystonias.                 squeezing that is not completely relieved
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                                                       Systemic medications help some patients.             by botulinum toxin is improved. Most
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                                                       Debilatating spasms, however, continue in            patients with apraxia of lid opening can
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                                                       most cases, rendering patients functionally          be improved with a combination of limited
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                                                       blind. Botulinum A toxin injection is the            myectomy, ptosis repair and botulinum
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                                                       best temporary therapy and myectomy is               toxin. Unfortunately drugs have provided
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                                                       the best long term therapy.                          little or no improvement for this disorder.
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                                                              Apraxia of the Eyelids                                  Upcoming Issues
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                                                              and Blepharospasm?
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                                                       Apraxia of eyelid opening is a condition               5 Myokymia 5 Hemifacial Spasm
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                     Figure 2                          that affects about 7% of Blepharospasm
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       Profound eyelid spasms, facial spasms                                                                       5 Trichiasis    5 Dry Eyes
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                                                       patients and rarely occurs on its own. The
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            and neck spasm (torticollis).
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                                                                             2

                If you have any questions regarding the topics of this newsletter, or requests for future topics of “InSight”,
                         please contact Dr. David R. Jordan by telephone (613) 563-3800 or fax (613) 563-1576

				
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