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Blepharospasme

Blepharospasme/ Benign Essential Blepharospasm adalah suatu distonia fokal bilateral yang ditandai
dengan spasme umum yang bermula sebagai kedutan ringan (mild twitches) atau pun mengedip yang
sering dan terjadi secara involunter. Hal ini terjadi karena kontraksi m.orbicularis oculi. Kondisi ini
lama kelamaan akan berlanjut menjadi kebutaan akibat ketidakmampuan eposidik membuka kelopak
mata.
Etiologi blepharospasme bersifat muti faktor, terdiri dari gangguan ekstra pyramidal dan batang otak
maupun faktor psikologis. Penelitian menggunakan MRI menunjukan adanya lesi fokal pada batang
otak bagian rostral, diencephalon dan ganglia bsalis. Pada kasus blepharospasme essensial
menunjukan adanya suatu hiperaktivitas sirkuit lortikal. Selain itu juga blepharospasme dapat
disebabkan oleh obat-obatan untuk mengobati parkinson, trauma pada mata, blepharitis, mata kering,
Tourette syndrome
Gejala klinis blepharospasme diawali dengan mengedip yang berlebihan saat terpapar dengan cahaya,
angin maupun stres, dengan progresivitas sesuai berjalannya waktu dapat terjadi spasme involunter
M.Orbicularis oculi secara bilateral, spasme dapat terjadi secara hebat dan menetap. Terdapat juga
droopy eyelids, fotofobia, pengelihatan kabur, kedutan pada kelopak mata yang tidak terkendali.
Penatalaksanaan kasus ini adalah penggunaan kacamata hitam untuk menghindari pemicu nya
terhadap cahaya, menjaga kebersihan kelopak mata untuk mencegah iritasi, dan penggunaan artificial
tears untuk mengurangi gejala. Selain itu ada pula injeksi berulang secara periodik Botulinum toxin
type A dapat menghasilkan paralisis otot terlokalisir. Injeksi daro Botox ini bersifat temporer. Onset
of action rata-rata adalah 2-3 hari, dan efek puncak terjadi sekitar 7-10 hari setelah injeksi. Efeknya
pun bervariasi sekitar 3-4 bulan. Untuk tindakan operatif dapat dilakukan myectomy













Blepharospasm is a neurological condition characterized by forcible closure of the
eyelids. The purpose of the Benign Essential Blepharospasm Research Foundation
(BEBRF) is to undertake, promote, develop and carry on the search for the cause and a
cure for benign essential blepharospasm and other related disorders and infirmities of
the facial musculature.
Blepharo means "eyelid". Spasm means "uncontrolled muscle contraction". The
term blepharospasm ['blef-a-ro-spaz-m] can be applied to any abnormal blinking or
eyelid tic or twitch resulting from any cause, ranging from dry eyes to Tourette's
syndrome to tardive dyskinesia. The blepharospasm referred to here is officially called
benign essential blepharospasm (BEB) to distinguish it from the less serious secondary
blinking disorders. "Benign" indicates the condition is not life threatening and "essential"
is a medical term meaning "of unknown cause". Patients with blepharospasm have
normal eyes. The visual disturbance is due solely to the forced closure of the eyelids.
Blepharospasm should not be confused with:
Ptosis - drooping of the eyelids caused by weakness or paralysis of a levator
muscle of the upper eyelid
Blepharitis - an inflammatory condition of the lids due to infection or allergies
Hemifacial spasm - a non-dystonic condition involving various muscles on one
side of the face, often including the eyelid, and caused by irritation of the facial
nerve. The muscle contractions are more rapid and transient than those of
blepharospasm, and the condition is always confined to one side
Sumber : http://www.blepharospasm.org/
Since the central control center for blepharospasm is unknown, drug therapy directed against this as
of yet unidentified center tends to follow a "shotgun approach." Historically, an extensive list of drugs
has been used to treat blepharospasm, in part because blepharospasm initially was considered a
manifestation of psychiatric illness, and because no one drug was demonstrably more efficacious
than another. Recently, these psychoactive medicines have been used not for their psychotropic
action but for their motor system action.
Most patients respond incompletely or not at all to pharmacotherapy. At best, pharmacotherapy
provides only partial, transient relief. Patients react differently to the various pharmacologic agents,
and there is no way to predict which patient may respond to any particular agent. Tricyclic
antidepressants do not directly help blepharospasm but are useful if there is depression exacerbating
the symptoms. Drugs with the highest percentages of favorable patient responses include lorazepam
(67% of patients), clonazepam (42%), and Artane (41%). The relief provided by these agents is
variable.
Although drugs from a variety of different classes have demonstrated some effectiveness in
blepharospasm, drug therapy for blepharospasm and facial dystonias usually are based upon the
following 3 unproven pharmacologic hypotheses: (1) cholinergic excess, (2) GABA hypofunction, and
(3) dopamine excess. Pharmacotherapy is generally less effective than BOTOX injections and, thus,
is reserved as second-line treatment for spasms that poorly respond to BOTOX, such as in mid-face
and lower-face spasm.
Botulinum toxin
Botulinum A toxin, or BOTOX, is regarded as the most effective treatment of choice for the rapid but
temporary treatment of orbicularis spasm. More than 95% of patients with blepharospasm report
significant improvement with use of the toxin. The toxin interferes with acetylcholine (ACh) release
from nerve terminals, causing temporary paralysis of the associated muscles. Botulinum A toxin is the
product of the bacteria, Clostridium botulinum(a large anaerobic, gram-positive, rod-shaped
organism).
Once injected, the toxin rapidly and firmly binds at receptor sites on cholinergic nerve terminals in a
saturable fashion. The toxin is internalized through the synaptic recycling process. Paralysis of
muscle is a result of the inhibition of the release of vesicular ACh from the nerve terminal. It is
assumed that the toxin attaches to the ACh-containing vesicles in the nerve terminal and prevents
calcium-dependent exocytosis.
The paralytic effect is dose related, with a peak of effect at 5-7 days after injection. Patients typically
note the onset of relief 2.5 days after injection, with a mean duration of relief from symptoms of 3
months. More than 5% of treated patients have sustained relief for more than 6 months, although
some patients require injections as often as monthly. It takes as much as 6-9 months for the injected
muscles to recover from the effects of the toxin, and, occasionally, muscles do not fully return to their
preinjection level of function. Some have suggested that the development of antitoxin antibodies or
the progressive atrophy of muscle may account for variations in the dose response curve, but no
studies have supported these findings.
Complications of botulinum toxin injections include ptosis (7-11%), corneal exposure/lagophthalmos
(5-12%), symptomatic dry eye (7.5%), entropion, ectropion, epiphora, photophobia (2.5%), diplopia (<
1%), ecchymosis, and lower facial weakness.

One of the more common adverse effects, ptosis, is due
to diffusion of toxin from the upper eyelid injection sites to the exquisitely sensitive levator muscle.
The incidence of ptosis has been reported as high as 50% of patients treated more than 4 times. In
the hands of experienced injectors, the rate of complications such as ptosis is presumably less.
Injection of botulinum toxin into the medial and lateral pretarsal orbicularis is usually sufficient to stop
spasms for the duration of effect; avoiding central injections to the preseptal and preorbital orbicularis
should help reduce the risk of ptosis.
Meticulous technique in the administration of BOTOX helps ensure reliable and consistent results.
BOTOX should be hydrated with 0.9% nonpreserved saline, which should be introduced slowly into
the vacuum-sealed vial to prevent frothing. Once reconstituted, the solution should be used within a
few hours or refrigerated.
At the first treatment, use of a total dose of no more than 25 units per eye, divided among 4-6
periocular injection sites is recommended to avoid adverse effects. Subsequent treatments should be
adjusted depending on patient response to the initial doses. At each site, inject 2.5-10 units of
BOTOX. Use of lower volumes (higher concentrations) is suggested to avoid the risk of spread to
adjacent areas. The solution should be injected subcutaneously over the orbicularis oculi and
intramuscularly over the thicker corrugator and procerus muscles. Patients may return home without
restrictions of activity. Most patients require repeated treatment every 3 months, but this ranges from
1-5 months.
Sumber : http://emedicine.medscape.com/article/1212176-treatment

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