JURNAL READING Pembimbing : dr. H. Dikdik Suparman, Sp. S dr. Fuad Hanif, Sp. S, M. Kes Disusun oleh : Latifa 2008730019 Sita Pradjnadewi 20087300 Tubagus Budi P 2008730128 KEPANITERAAN KLINIK STASE SARAF FAKULTAS KEDOKTERAN DAN KESEHATAN UNIVERSITAS MUHAMMADIYAH JAKARTA 2013
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Hz) of motor unit potentials, which correlate with clinically observed facial movements.
Magnetic resonance imaging (MRI) is the first-line imaging study.
Angiography and/or magnetic resonance angiography may be necessary before a
vascular surgical procedure.
Management
Pharmacological treatment
Medications may be used in early hemifacial spasm (HFS):
This is when spasms are mild and infrequent or in patients who decline botulinum
toxintype A injection. Response to medication varies but can be satisfactory in early or
mild cases.
The most helpful agents are carbamazepine and benzodiazepines, eg baclofen,
clonazepam. Topiramate has also been used successfully.[7]
Often, medication effects attenuate over time, necessitating more aggressive treatment.
In most patients, the treatment of choice is injection of botulinum toxin type A (Botox®)
under EMG guidance:[8][9]
This is a chemical denervation that safely treats most patients, especially those with
sustained contractions.
It works by binding to receptor sites on the motor nerve terminals and, after uptake,inhibits release of acetylcholine, blocking transmission of impulses in neuromuscular
tissue.
The spasms usually relax 3-5 days after injection and the effect lasts approximately 6
months.
Potential side-effects of botulinum injection are facial asymmetry, ptosis and facial
weakness. These are usually transient.
Most patients are very satisfied with their response to treatment.
Patients should be warned that although botulinum toxin stops the spasm, the sensation
of spasm often persists.
It is now far more commonly used than surgical intervention.[10]
Surgical treatment
This usually consists of exploration of the posterior fossa to separate blood vessels from
the VIIth cranial nerve.
Microvascular decompression of the facial nerve can provide long-term symptom
control in approximately 90% of patients with HFS.[11]
follow up of 83 cases treated by microvascular decompression at two neurosurgicalcentres in the United Kingdom. J Neurol Neurosurg Psychiatry. 1996 Jan;60(1):72-7.
3. Han IB, Chang JH, Chang JW, et al; Unusual causes and presentations of hemifacial
Hemifacial spasm (HFS) is a rare neuromuscular disease characterized by irregular,
involuntary muscle contractions (spasms) on one side (hemi-) of the face (-facial).[1] The
facial muscles are controlled by the facial nerve (seventh cranial nerve), which originates at
the brainstem and exits the skull below the ear where it separates into five main branches.
This disease takes two forms: typical and atypical. In typical form, the twitching usually
starts in the lower eye lid in orbicularis oculi muscle. As time progresses, it spreads to the
whole lid, then to the orbicularis oris muscle around the lips, and buccinator muscle in
thecheekbone area.[2] The reverse process of twitching occurs in atypical hemifacial
spasm; twitching starts in orbicularis oris muscle around the lips, and buccinator muscle in
the cheekbone area in the lower face, then progresses up to the orbicularis oculi muscle in the
eyelid as time progresses.[2] The most common form is the typical form, and atypical form is
only seen in about 2 – 3% of patients with hemifacial spasm.[3] The incidence of hemifacial
spasm is approximately 0.8 per 100,000 persons.[4]
This disorder occurs in both men and women, although it affects middle-
aged or elderly women more frequently.[5] Hemifacial spasm is much more common in
some Asian population.[1] It may be caused by a facial nerve injury, a tumor , or it may have
no apparent cause. Individuals with spasm on both sides of the face are very rare.
History
The earliest descriptions about hemifacia spasm is by Shultze in 1875 and Gowers in 1899.
The etiology of hemifacial spasm and location of the abnormality have been debated for more
than a century.[4]
Surgical treatment for hemifacial spasm in the early 20th century includedneurolysis (destruction of nerve tissue), stretching the facial nerve (seventh cranial nerve),
and high-pressure irrigation of the nerve with lactate ringer's solution. The medical regimens
of that time involved injection of the nerve with ethanol, electrical stimulation, application of
toxic compounds (nitrate of silver , zinc, arsenic, bromides) as well as medications such
as Dilantin or other anticonvulsants.[4][6][7]
Additional advances in understanding the etiology and improving treatments for hemifacial
spasm did not occur until the mid-seventies. In 1977, 47 cases of hemifacial spasm
underwent microvascular decompression of the facial nerve using the operating microscope.
The results illustrated nerve-vessel conflicts (or cholesteatoma) to be located at the root exit
zone of the facial nerve in all cases.[8][9] The root exit zone is where the
central glial axonal insulation of the nerve ends and the peripheral nerve axonal
myelination begins. Biopsies of the root exit zone demonstrated degeneration of axons,
denuded axis cylinder and interrupted myelin. The results of the experiment strengthened the
theory that vascular compression of the facial nerve was the primary cause of hemifacial
spasm, and proposed a specific region of the facial nerve where the effects of longstanding
Three theories exist to explain the facial nerve dysfunction found in hemifacial spasm. The
first proposed theory is ephaptic transmission, which is electrical activity crossing from one
demyelinated neuron to another resulting in a false synapse.[7] The second theory involves
abnormal activity of axons at the facial nerve root end zone secondary to compressive
damage/demyelination.[10]
The third theory or "Kindling theory" involves increased excitability of the facial
nerve nucleus due to feedback from a damaged facial nerve.[10]
It is generally accepted as compression of the facial nerve by vessels of the posterior
circulation.[10] In detail compression of the seventh cranial nerve by a dolichoectatic (a
distorted, dilated, and elongated) vertebral artery[11] is accepted to be the general cause of
hemifacial spasm. Less than 1% of cases are cause by tumor .[12][13] Hemifacial spasm is much
more common in some Asian population.[1]
Several families with hemifacial spasm have been reported, suggesting a
genetic etiology or predisposition in some cases. There appears to be an autosomal
dominant pattern of inheritance in these families with low penetrance, and except for ayounger age at onset, the clinical features overlap with the idiopathic cases.Evaluation
of single-nucleotide polymorphisms in genes related to vascular change causing compression
of blood vessles did not show an association with hemifacial spasm. Clarifying the role of
genetic susceptibility in hemifacial spasm may help to better understand the pathogenesis of
Mild cases of hemifacial spasm may be managed with sedation or carbamazepine (an
anticonvulsant drug).[18] Microsurgical decompression and botulinum toxin injections are the
current main treatments used for hemifacial spasm.[17]
Microvascular Decompression
Microvascular decompression appears to be the most popular surgical treatment at present.
Microvascular compression relieves pressure on the facial nerve, which is the cause of most
hemifacial spasm cases. Excellent to good results are reported in 80% or more cases with a
10% recurrence rate.[19] In the present series approximately 10% had previously failed
surgery. Serious complications can follow microsurgical decompressive operations, even
when performed by experienced surgeons. These include cerebellar haematoma or
swelling, brain stem infarction (blood vessel of the brain stem blocked), cerebral
infarction (ischemic stroke resulting from a disturbance in the blood vessels supplying blood
to the brain), subdural haematoma and intracerebral infarction (blockage of blood flow to the brain). Death or permanent disability (hearing loss) can occur in 2% of patients of hemifacial
spasm.[20]
Botulinum Toxin
Botulinum toxin is highly effective in the treatment of hemifacial spasm.[21] It has a success
rate equal to that of surgery, but repeated injections may be required every 3 to 6 months. The
injections are administered as an outpatient or office procedure. Whilst side effects occur,
these are never permanent. Repeated injections over the years remain highly
effective.[22] Whilst the toxin is expensive, the cost of even prolonged courses of injections
compares favourably with the cost of surgery.[21] Patients with HFS should be offered a
number of treatment options. Very mild cases or those who are reluctant to have surgery
or Botulinum toxin injections can be offered medical treatment, sometimes as a temporary
measure.In young and fit patients microsurgical decompression and Botulinum injections
should be discussed as alternative procedures. In the majority of cases, and especially in the
elderly and the unfit, Botulinum toxin injection is the treatment of first choice. Imaging
procedures should be done in all unusual cases of hemifacial spasm and when surgery is
contemplated.[17]
Prevention
There is no known way to prevent hemifacial spasm.[23]
Epidemiology
The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons.[4] Hemifacial
spasm is more prevalent among females over 40 years of age.[24][25] The estimated prevalence
for women is 14.5 per 100,000 and 7.4 per 100,000 in men.[26] Prevalence for hemifacial
spasm increase with age, reaching 39.7 per 100,000 for those aged 70 years and older .[27] One
study divided 214 hemifacial patients based on the cause of the disease. The patients who had
a compression in the facial nerve at the end of the brain stem as the primary hemifacial spasm