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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
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.
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.
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
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
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.