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Hemifacial spasm consists of painless irregular involuntary

contractions on one side of the face. Symptoms may


develop as a sequela to Bells palsy but may also be due
to an irritative lesion of the facial nerve (e.g., an acoustic
neuroma, an aberrant artery that compresses the nerve,
or a basilar artery aneurysm).However, in the most common
form of hemifacial spasm, the cause and pathology
are unknown. Mild cases can be treated with carbamazepine,
gabapentin, or, if these drugs fail,with baclofen.
Local injections of botulinum toxin into affected muscles
can relieve spasms for 34 months, and the injections can
be repeated. Refractory cases due to vascular compression usually respond to
surgical decompression of the facial
nerve. Blepharospasm is an involuntary recurrent spasm of
both eyelids that usually occurs in elderly persons as an
isolated phenomenon or with varying degrees of spasm
of other facial muscles. Severe, persistent cases of blepharospasm
can be treated by local injection of botulinum
toxin into the orbicularis oculi. Facial myokymia
refers to a fine rippling activity of the facial muscles; it
may be caused by multiple sclerosis or follow GuillainBarr syndrome (Chap. 41).
Facial hemiatrophy occurs mainly in women and is
characterized by a disappearance of fat in the dermal
and subcutaneous tissues on one side of the face. It usually
begins in adolescence or early adult years and is
slowly progressive. In its advanced form, the affected side
of the face is gaunt, and the skin is thin, wrinkled, and
brown. The facial hair may turn white and fall out, and
the sebaceous glands become atrophic. Bilateral involvement
may occur. A limited form of systemic sclerosis
(scleroderma) may be the cause of some cases.Treatment
is cosmetic, consisting of transplantation of skin and subcutaneous
fat.
Harison

Hemifacial spasm has


many similarities to trigeminal neuralgia. Both are often
caused by minor anatomical variations of blood vessels
overlying the nerve. Hemifacial spasm consists of continuous
twitching movements usually maximal around the eye
and the mouth. The condition is often more annoying and
embarrassing than unpleasant. It may occur at any age but
is more commonly found in older age groups. Tumours at
the cerebellopontine angle, basilar artery aneurysms and
basal meningitis may all be responsible for this condition,

but in most cases no definite cause is found.


Clinical neuro anatomi

Hemifacial spasm is characterized by clonic spasms of the facial muscles, usually


starting around the eye and often spreading to other muscles of one side of the
face. It increases in intensity during stress and may occur in sleep. The characteristic
EMG findings include bursts of muscle action potentials that occur either
regularly or irregularly at 5 to 20 per second. Synkinetic motor responses in muscles
innervated by the facial nerve follow stimulation of the ipsilateral fifth nerve (blink
reflex). Hemifacial spasm does not have the ominous implications of myokymia, but the
cosmetic effects may be distressing. The cause is usually obscure, but it may
follow facial nerve trauma. Treatment with anticonvulsant medication, such as
carbamazepine, may be effective. Jannetta (1977) reported relief of the involuntary
movements by exposing the facial nerve in the posterior fossa and decompressing
vessels at the root entry zone. Botulinum toxin is also effective.

Pathology
After nerve damage, the pathologic changes depend on the nature of the injury, which
also affects the regenerative response and the prognosis for recovery.
According to Seddon (1954), mechanical nerve injuries are classified as follows: (1)
complete severing of a nerve ( neurotmesis), (2) axonal interruption with distal
degeneration but an intact endoneurium ( axonotmesis), or (3) conduction block at the
site of the lesion but normal distal conduction without degeneration of distal
fibers (neurapraxia).
Within the first 24 hours of injury, focal swelling occurs adjacent to the damaged site
with fragmentation of endoplasmic reticulum, neurotubules, and neurofilaments,
and accumulation of organelles. The axolemma becomes discontinuous; axons swell at
some sites and narrow at others to give a beaded appearance. This process
begins between the nodes of Ranvier and appears first in smaller fibers. Changes in
myelin sheaths lag behind those in axons but progress in a similar way along the
entire distal stump, again affecting small fibers first. The myelin surrounding the
fragmented axons breaks up to form rows of elliptoids. Finally, Schwann cells and
macrophages degrade the axon and myelin debris. In addition to these distal nerve
changes, a retrograde axon reaction or chromatolysis is seen, with retraction of
axons proximal to the lesion and alterations in the somata of neurons, such as cell body
swelling, disruption of Nissl substance, migration of the cell nucleus, and
increase in the size of the nucleolus. Presynaptic terminals gradually withdraw from the
soma and dendrites; synaptic transmission is reduced until dorsal root
stimulation fails to excite the motor neuron and evoke a reflex discharge in the ventral
root. The pathologic distal changes of degeneration and retrograde axon
reaction are similar in crush injury or complete nerve transection.

If a nerve has been completely severed, the orderly process just described is interfered
with in proportion to the length of the discontinuity between proximal and distal
ends. If this distance is great, regeneration is not possible, unless the ends are apposed
at operation. If the distance is small, the fine processes of the axon penetrate
the fibrin and connective tissue in the scar and enter the distal end of the nerve. Some
of these may be deflected from the proper path by the scar and become
entangled to form a neuroma.
Merit

H e m ifa c i a l S p a s m

The facial muscles o n one side may b e involved in


painless irregular clonic contractions of varying degree
(hemifacial spasm). This condition usually develops in the
fifth and sixth decades, affects women more than men,
and often proves to be caused by a compressive lesion
of the facial nerve, usually by a tortuous branch of the
basilar artery that lies on the ventral surface of the pons
and forms a loop under the proximal seventh nerve. Less
often the cause of compression is a fusiform basilar artery
aneurysm or a vestibular schwannoma or meningioma.
Multiple sclerosis is another rare cause.
The spasm usually begins in the orbicularis oculi
muscle and gradually spreads to other muscles on that
side of the face, including the platysma. Paroxysms may
be induced or aggravated by voluntary and reflexive
movements of the face.
The pathophysiology of the spasm is believed to be
focal demyelination at the site of nerve root compression
by the vessel. The demyelinated axon is presumed to
activate adjacent nerve fibers by ephaptic transmission
("artificial" synapse of Granit et al). Another possible
source of the spasm is spontaneous ectopic excitation
arising in injured fibers. Nielsen and Jannetta have
shown that ephaptic transmission disappears after the
nerve is decompressed.
Treatment Jannetta has attributed virtually all cases
to a compression of the root of the facial nerve by an aberrant
looped blood vessel. Microsurgical decompression of
the root with the interposition of a pledget between the
vessel and the facial nerve relieved the facial spasm in
most of his operative patients. These results were corroborated
by Barker and associates in a series of 705 patients
followed postoperatively for an average period of 8
years; 84 percent achieved an excellent result. An even
higher rate of benefit was obtained in a prospective series
by lllingworth and colleagues (cure of 81 of 83 patients) .
Surgical decompression o f the aforementioned vascular
loop, which involves exploration of the posterior
fossa, however, carries some risk. The facial muscles may
be weakened, sometimes permanently. Another complication
has been deafness as a result of injury of the adjacent
eighth nerve. Also, there is a risk of recurrence of the
spasms, usually within 2 years of the operation (Piatt and
Wilkins) . Tight dural closure is required to prevent CSF
leakage from the posterior fossa.
We suggest that patients with idiopathic hemifacial
spasm should first be treated medically. Alexander

and Moses noted that carbamazepine in doses of 600


to 1 ,200 mg/ d controls the spasm in two-thirds of the
patients. Baclofen or gabapentin can be tried if carbamazepine
fails. Some patients cannot tolerate these drugs,
have only brief remissions, or fail to respond; they may be
treated with botulinum toxin injected into the orbicularis
oculi and other facial muscles. The hemifacial spasms are
relieved in this way for 4 to 5 months and injections can
be repeated without danger. Some patients have been
injected repeatedly for more than 5 years without apparent
adverse effects. Failing these conservative measures,
surgery may be appropriate.
Adams

Hemifacial Spasm
Facial synkinesias may progress to a stage of HFS.
More often, HFS arises de novo, due to intermittent
compression by an ectatic arterial loop in the posterior circulation, most often a redundant
loop of
the AICA. The compression is usually near the anterior
aspect of the root exit zone. The pathophysiology
is similar to that in some cases of trigeminal neuralgia
(Chapter 15). The arterial pulsations are thought to
cause demyelination and focal nerve damage leading
to ephaptic transmission and ectopic excitation.
Combined studies using MRI and MRA may demonstrate
the neurovascular compression. An MRI
study using 3D reconstruction confi rmed the AICA
as the most common causative vessel, with the posterior
inferior cerebellar artery, vertebral artery, internal
auditory artery, and veins occasionally causing facial
nerve compression at the root entry zone. However,
radiographic studies using a 3T MRI has shown that
some contact between the facial nerve and nearby vessels,
even enough to cause mild nerve deviation, is the
rule rather than the exception.
Microvascular decompression is sometimes done
and may effectively halt the movements. The lateral
spread response is an electrophysiologic phenomenon
seen in HFS. Stimulation of the mandibular branch
of the facial nerve may cause a compound muscle
action potential to appear in the orbicularis oculi.
This response does not occur in normals. The lateral
spread response is objective evidence of ephaptic transmission from one facial nerve branch
to another.
During microvascular decompression, the lateral
spread response may disappear when the offending
vessel is lifted off the nerve, and the status of the
response may be used as an indicator of the effectiveness
of the decompression. HFS may also occur with
other extra-axial or intra-axial lesions, including aneurysm,
tumor, multiple sclerosis, or basilar meningitis.

HFS usually develops in older patients; the


prevalence for women is about twice that for men.
Twitching usually begins in the orbicularis oculi, less
often in the oris. Initially the twitching may be subtle
and diffi cult to distinguish from facial synkinesias.
HFS may involve the entire facial nerve distribution,
or only certain nerve branches; it may propagate from
one branch to another. Over months to years, HFS
usually spreads to involve all of the facial muscles on
one side, but it remains strictly limited to the muscles
supplied by the facial nerve. As HFS worsens,
it may involve the auricular muscles even when the
patient cannot deliberately wiggle the ears; the platysma
may also be affected. Fully developed HFS causes
repetitive, paroxysmal, involuntary, spasmodic, tonic
and clonic contractions of the muscles innervated
by the facial nerve on the involved side of the face.
The mouth twists to the affected side, the nasolabial
fold deepens, the eye closes, and there is contraction
of the frontalis muscle (see Figure 16.6). For a video
of HFS, see http://www.medclip.com/index.php?
page=videos&section=view&vid_id=101632. The
spasms may persist in sleep, and are often exacerbated
by chewing or speaking. Synkinesias following PFP
may cause movements resembling HFS. The essential
difference is that synkinesias are provoked by a
voluntary movement, whereas HFS is a spontaneous,
involuntary contraction. HFS is commonly associated
with some degree of facial weakness because of
underlying nerve damage. Rare patients may have
both HFS and trigeminal neuralgia, with lancinating
pain accompanying the facial spasms (tic convulsif ).
Brissaud-Sicard syndrome is HFS with contralateral
hemiparesis due to a lesion in the pons.
Blepharospasm (nictitating spasm) causes involuntary
twitching that primarily involves the orbicularis
oculi and frontalis muscles. Blepharospasm is most
often idiopathic or essential and is a form of focal
dystonia (Chapter 30). Blepharospasm is always bilateral
and fairly symmetric. For a video of blepharospasm
see http://www.medclip.com/index.php?page=
videos&section=view&vid_id=101616. Meiges syndrome
is the association of blepharospasm with oromandibular
dystonia. Patients with CNS Whipples
disease may have an oculofacial, more often an oculomasticatory,
myorhythmia.
Tic, or habit spasm, can cause a movement
resembling HFS or blepharospasm. Tic often causes
retraction of the angle of the mouth, contraction of
the orbicularis oculi or platysma, or eye blinking. The
movements are somewhat more bizarre and purposeful,
and other muscles not innervated by CN VII may
be brought into action. Bizarre grimacing movements
of the face are usually habit spasms. The movements
in HFS and essential blepharospasm are stereotyped.

The patient with tic can suppress the movements, at


least temporarily, while the movements of HFS and
blepharospasm are totally beyond volitional control
and cannot be suppressed or imitated.

De jong 266-268

Hemifacial Spasm
This condition is characterized by synchronous, irregular,
rapid, brief contractions of all of the muscles of facial
expression supplied by the facial n. on one side of the
face, particularly including the platysma. On close observation,
hemifacial spasm is readily distinguishable
from a facial tic (Fig. 11.19). It rarely arises in the aftermath
of a peripheral facial nerve palsy. The usual cause
is irritation of the facial nerve root by a looping blood vessel
just distal to its point of exit from the pons; this explains
why neurosurgical intervention (microvascular
decompression) is usually successful. It is very rare for
hemifacial spasm to be caused by a brainstem glioma.
The condition can be treated symptomatically with anticonvulsants
such as carbamazepine, or with injections
of botulinus toxin.

Fundamental of neurology 199

Hemifacial spasm is characterized by clonic spasms


of the facial muscles, usually starting around the eye
and often spreading to other muscles of one side of
the
face. It increases in intensity during stress and may
occur in sleep. The characteristic EMG findings
include bursts of muscle action potentials that occur
either
regularly or irregularly at 5 to 20 per second.
Synkinetic motor responses in muscles innervated by
the facial nerve follow stimulation of the ipsilateral
fifth nerve (blink
reflex). Hemifacial spasm does not have the ominous implications of myokymia, but the
cosmetic effects may be distressing. The cause is usually obscure, but it may
follow facial nerve trauma. Treatment with anticonvulsant medication, such as
carbamazepine, may be effective. Jannetta (1977) reported relief of the involuntary
movements by exposing the facial nerve in the posterior fossa and decompressing
vessels at the root entry zone. Botulinum toxin is also effective
lipincoot textbook of neurology 342

Treatment
Treatment with anticonvulsant medication, such as carbamazepine, may be effective.
Jannetta (1977) reported relief of the involuntary
movements by exposing the facial nerve in the posterior fossa and decompressing
vessels at the root entry zone. Botulinum toxin is also effective
lipincoot textbook of neurology 342
The condition can be treated symptomatically with anticonvulsants
such as carbamazepine, or with injections
of botulinus toxin.
Fundamental of neurology 199
Jannetta has attributed virtually all cases
to a compression of the root of the facial nerve by an aberrant
looped blood vessel. Microsurgical decompression of
the root with the interposition of a pledget between the
vessel and the facial nerve relieved the facial spasm in
most of his operative patients. These results were corroborated
by Barker and associates in a series of 705 patients
followed postoperatively for an average period of 8
years; 84 percent achieved an excellent result. An even
higher rate of benefit was obtained in a prospective series

by lllingworth and colleagues (cure of 81 of 83 patients) .


Surgical decompression o f the aforementioned vascular
loop, which involves exploration of the posterior
fossa, however, carries some risk. The facial muscles may
be weakened, sometimes permanently. Another complication
has been deafness as a result of injury of the adjacent
eighth nerve. Also, there is a risk of recurrence of the
spasms, usually within 2 years of the operation (Piatt and
Wilkins) . Tight dural closure is required to prevent CSF
leakage from the posterior fossa.
We suggest that patients with idiopathic hemifacial
spasm should first be treated medically. Alexander
and Moses noted that carbamazepine in doses of 600
to 1 ,200 mg/ d controls the spasm in two-thirds of the
patients. Baclofen or gabapentin can be tried if carbamazepine
fails. Some patients cannot tolerate these drugs,
have only brief remissions, or fail to respond; they may be
treated with botulinum toxin injected into the orbicularis
oculi and other facial muscles. The hemifacial spasms are
relieved in this way for 4 to 5 months and injections can
be repeated without danger. Some patients have been
injected repeatedly for more than 5 years without apparent
adverse effects. Failing these conservative measures,
surgery may be appropriate.
Adams

Treatment with anticonvulsant medication, such as carbamazepine, may be effective.


Jannetta (1977) reported relief of the involuntary
movements by exposing the facial nerve in the posterior fossa and decompressing
vessels at the root entry zone. Botulinum toxin is also effective
Merrit

. Mild cases can be treated with carbamazepine,


gabapentin, or, if these drugs fail,with baclofen.
Local injections of botulinum toxin into affected muscles
can relieve spasms for 34 months, and the injections can
be repeated. Refractory cases due to vascular compression usually respond to
surgical decompression of the facial
nerve.
Harrison

Tatalaksana
Dalam penelitian yang telah dilakukan oleh janneta , telah dikaitkan dari seluruh kasus pada
pasien dengan kompresi akar n. facialis yang diakibatkan penekanan oleh letak pembuluh darah
yang menyimpang. Tindakan operasi yang dilakukan pada akar saraf dengan penempatan sebuah
tampon diantara pembuluh darah dan saraf memberikan penyembuhan yang baik pada hampr
seluruh pasien pasien dengan hemifacial spasme yang dilakukan operasi. Penelitian ini juga
diperkuat oleh penelitian yang juga dilakukan oleh Barker dkk pada 705 pasien yang dilakukan
operasi dekompresi yang sama dengan periode rata-rata pasca operasi 8 tahun, 84 % pasien
mengalami penyembuhan yang sangat baik. Barker juga menjelaskan walaupun bedah
dekompresi yang dilakukan memberikan hasil yang baik, beberapa resiko tetap dapat terjadi
diantaranya otot-otot wajah mungkin melemah bahkan lumpuh permanen , juga terdapat rsiko
kekambuhan dari spasme yang terjadi, biasanya dalam waktu 2 tahun pasca operasi.
Beberapa peneliti menyarankan pada pasien dengan hemifacial spasme serangan awal sebaiknya
dilakukan pengobatan secara medis tanpa dilakukan tindakan operasi. Alexander dan musa
mencatat bahwa carbamazepine dalam dosis 600-1200 mg/d dapat mengontrol spasme yang
terjadi pada dua per tiga dari pasien dengan hemifacial spasme, jika carbamazepine gagal ,

sebagai lini kedua dapat digunakan baclofen atau gabapentin , namun pada beberapa pasien
terdapat efek samping pada obat ini , sebagai penggantinya dapat dilakukan penyuntikan toksin
botulinum pada otot orbicularis oculi yang mengalami spasme. Beberapa pasien telah melakukan
penyuntikan ini selama lebih dari 5 tahun tanpa efek samping yang bermakna. Jika seluruh terapi
pengobatan yang diberikan tidak mencapai hasil yang baik , tindakan operasi mungkin dapat
dipilih sebagai terapi lanjutan. (adams, merit Harrison lipincoot)

Patofisiologi

Patogenesis hemifacial spasm karena kompresi saraf wajah dijelaskan dengan


menggunakan beberapa teori. Menurut hipotesis "perifer", eksitasi ephaptik dan ektopik terjadi di
zona akar-keluar. Konduksi impuls ephaptik ditandai dengan transfer patologis impuls antara
serabut saraf tetangga. Konduksi impuls ektopik menggambarkan perkembangan spontan dari
impul ssaraf di daerah kompresi. Sebaliknya, hipotesis "pusat" mengasumsikan hipereksitabilitas
motor nucleus wajah di batang otak. Penjelasan untuk usia yang relative tua pada pasien saat
onset penyakit adalah karena adanya perubahan progresif yang ektatik dan perpanjangan yang
dapat mempengaruhi pembuluh darah di cerebello pontine angle. Hal ini umum pada pasien
dengan hipertensi arteri. Seiring waktu, hal ini menyebabkan kontak antara pembuluh darah dan
saraf, yang menyebabkan kompresi. Diasumsikan bahwa ini mengarah pada gilirannya untuk
demielinasi fokal, yang mengarah ke proses elektrofisiologi yang disebutkan di atas.6

Pemeriksaan radiologis dengan CT-scan atau radiografi polos dapat dilakukan untuk
menyingkirkan fraktur, metastasis tulang, dan keterlibatan sistem saraf pusat (SSP).2,4,5
Pemeriksaan MRI dilakukan pada pasien yang dicurigai neoplasma di tulang temporal, otak,
glandula parotis, atau untuk mengevaluasi sklerosis multipel. Selain itu, MRI dapat
memvisualisasi perjalanan dan penyengatan kontras saraf fasialis. Pemeriksaan neurofisiologi
sudah dikenal sejak tahun 1970- sebagai prediktor kesembuhan, bahkan dahulu sebagai acuan
pada penentuan kandidat tindakan dekompresi intrakanikular. 10,11 Grosheva et al10

melaporkan pemeriksaan elektromiografi (EMG) mempunyai nilai prognostik yang lebih baik
dibandingkan elektroneurografi (ENG). Pemeriksaan serial EMG pada penelitian tersebut setelah
hari ke-15 mempunyai positive-predictivevalue (PPV) 100% dan negative-predictive-value
(NPV) 96%. 6Pemeriksaan EMG pada hemifacial spasm secara karakteristik ditandai timbulnya
irama gelombang frekuensi tinggi ( 150-400 Hz ), dengan sinkronisasi. 1Pemeriksaan blink reflex
ini sangat bermanfaat karena 96% kasus didapatkan abnormalitas hingga minggu kelima, meski
demikian sensitivitas pemeriksaan ini rendah.