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Hemifacial Spams
Definition
This is repetitive, irregular clonic twitching of the muscles innervated by the facial nerve on
one side of the face.

The first symptom is usually an intermittent twitching of the eyelid muscle that can lead
to forced closure of the eye.
The spasm may then gradually spread to involve the muscles of the lower face, which
may cause the mouth to be pulled to one side.
Eventually the spasms involve all of the muscles on one side of the face almost
continuously.
Epidemiology
It is a rare condition.[1]
All races are affected equally.
The disorder occurs in both men and women, although it is more frequent in middleaged or elderly women.
Aetiology

Vascular compression most commonly.[2] Most instances of hemifacial spasm (HFS)

previously thought to be idiopathic, were probably caused by aberrant blood vessels
compressing the facial nerve within the cerebellopontine angle.[3]
Idiopathic.
Facial nerve compression by mass or Paget's disease of bone.
Meningitis.
Brainstem lesion such as stroke or multiple sclerosisplaque.
Secondary to trauma or Bell's palsy. 16% of Bell's palsy sufferers are left with
hemifacial spasm.[4]
[ ]
Secondary to otitis media with effusion. 5
Presentation

The disorder presents in the fifth or sixth decade of life:

Usually unilaterally, although bilateral involvement may occur rarely in severe cases.[1]
Hemifacial spasm (HFS) generally begins with brief clonic movements of the orbicularis
oculi and spreads over years to other facial muscles. Closing of the eye and drawing up
of the corner of the mouth are typical.
Involuntary facial movement is the only symptom.

Fatigue, anxiety or reading may precipitate the movements. Stressful life events are also
associated with HFS.[6]
Mild facial weakness and contraction appear without frank facial palsy.
There is normal facial sensation.
There are no other physical signs.
Differential diagnosis
Facial myokymia appears as vermicular (worm-like) twitching under the skin:[1]
Often with a wavelike spread.
This is distinguished from other abnormal facial movements by characteristic
electromyogram.
Facial myokymia may occur with any pathological brainstem process.
Most cases are idiopathic and eventually resolve without treatment.
Myoclonic movements may arise from lesions at the brain or brainstem level.
They are distinguished from hemifacial spasm (HFS) by the distribution of
abnormal movements.
They tend to be more generalised, possibly bilateral.
Oromandibular dystonia (OMD) refers to dystonia affecting the muscles of the lower
face.
When OMD occurs with blepharospasm, it is called Meige's syndrome II.
Henry Meige first described it in 1910.
Craniofacial tremor may occur in association with essential tremor, Parkinson's disease,
thyroid dysfunction or electrolyte disturbance. It occurs rarely in isolation.
Focal motor seizures must occasionally be distinguished from other facial movement
disorders, particularly HFS. Postictal weakness and greater involvement of the lower
face are its distinguishing features.
Facial chorea usually occurs within a systemic movement disorder, eg Huntington's
chorea.
Spontaneous orofacial dyskinesia of the elderly, is observed primarily in the edentulous.
It usually responds to proper fitting of dentures.
Facial tics are brief, repetitive, co-ordinated, semi-purposeful movements of grouped
facial and neck muscles:
Tics may occur physiologically or in association with diffuse encephalopathy.
Tardive dyskinesia - some medications (dopamine agonists), eg neuroleptics,
anticonvulsants, caffeine, methylphenidate, anti-Parkinsonian agents, are associated
with producing tics.
Investigations

At initial evaluation, consider hemifacial spasm (HFS) as a symptom, not a diagnosis:

An abnormal neurological examination (except for the facial movements) should prompt
the search for an underlying cause, eg compressive lesion, tumour, stroke.
Needle electromyogram (EMG) shows irregular, brief, high-frequency bursts (150-400
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]
[
]
Operative success is improved with EMG use. 12

Postoperative complications have been noted in 35% of patients, eg facial palsy, hearing
deficit and low cranial nerve palsies. The more immediate and severe the facial palsy
was, the more permanent it remained.[13]
Myectomy is rarely ever required.
Prognosis
Hemifacial spasm (HFS) is a progressive, but nonfatal illness. It usually responds favourably
to treatment.
Provide feedback
Further reading & references
1. Gulevich S, Hemifacial Spasm, Medscape, Jun 2010
2. Illingworth RD, Porter DG, Jakubowski J; Hemifacial spasm: a prospective long-term
follow up of 83 cases treated by microvascular decompression at two neurosurgical
centres 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
spasm. Neurosurgery. 2009 Jul;65(1):130-7; discussion 137.
4. Peitersen E; Bell's palsy: the spontaneous course of 2,500 peripheral facial nerve palsies
of different etiologies. Acta Otolaryngol Suppl. 2002;(549):4-30.
5. Lavon H, Cohen-Kerem R, Uri N; Hemifacial spasm associated with otitis media with
effusion: a first reported case. Int J Pediatr Otorhinolaryngol. 2006 May;70(5):947-50.
Epub 2005 Nov 15.
6. Johnson LN, Lapour RW, Johnson GM, et al; Closely spaced stressful life events
precede the onset of benign essential blepharospasm and hemifacial spasm. J
Neuroophthalmol. 2007 Dec;27(4):275-80.
7. Alonso-Navarro H, Rubio L, Jimenez-Jimenez FJ; Topiramate as treatment for
hemifacial spasm. Clin Neuropharmacol. 2007 Sep-Oct;30(5):308-9.
8. Jost WH, Kohl A; Botulinum toxin: evidence-based medicine criteria in blepharospasm
and hemifacial spasm. J Neurol. 2001 Apr;248 Suppl 1:21-4.
9. Costa J, Espfrito-Santo C, Borges A, Ferreira JJ, Coelho M, Moore P, Sampaio C.
Botulinum toxin type A therapy for hemifacial spasm. Cochrane Database of Systematic
Reviews 2005, Issue 1. Art. No.: CD004899. DOI: 10.1002/14651858.CD004899.pub2.
10. Holds JB, White GL Jr, Thiese SM, et al; Facial dystonia, essential blepharospasm and
hemifacial spasm. Am Fam Physician. 1991 Jun;43(6):2113-20.
11. Moffat DA, Durvasula VS, Stevens King A, et al; Outcome following retrosigmoid
microvascular decompression of the facial nerve for hemifacial spasm. J Laryngol Otol.
2005 Oct;119(10):779-83.
12. Sekula RF Jr, Bhatia S, Frederickson AM, et al; Utility of intraoperative
electromyography in microvascular decompression for Neurosurg Focus. 2009
Oct;27(4):E10.
13. Huh R, Han IB, Moon JY, et al; Microvascular decompression for hemifacial spasm:
analyses of operative complications in 1582 consecutive patients. Surg Neurol. 2008
Feb;69(2):153-7.

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
thebrainstem 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 23% 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 middleaged 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 included
neurolysis (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 otheranticonvulsants.[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
compression results in nerve dysfunction.[9]

Causes

The Facial Nerve ( The Seventh Cranial Nerve)

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 orpredisposition in some cases. There appears to be an autosomal
dominant pattern of inheritance in these families with low penetrance, and except for a
younger 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
this disease.[14]

Signs & Symptoms

The two forms of Hemifacial Spasm

The first sign of hemifacial spasm is typically muscle movement in the patient's eyelid and
around the eye. It can vary in intensity.[9] The intermittent twitching of the eyelid, which can
result in forced closure of the eye which gradually spreads to the muscles of the lower part of
the face (Typical form- See Image). In atypical form the spasms start in the cheekbone area
and spreads to the eyelid.[2] Ultimately, all the muscles on that side are affected, nearly all the
time. This sometimes causes the mouth to be pulled to the side. Experts have linked
hemifacial spasm to facial nerve injury, Bell's palsy and tumors. Although the most frequent
cause is a blood vessel pressing on the facial nerve at the spot where it leaves the patient's
brain stem, sometimes there is no known cause. When the affected individual is younger than
40, doctors suspect an underlying cause such as multiple sclerosis.[15]
Diagnosis
There are several tests done to diagnose hemifacial spasm. Diagnosing a case of hemifacial
spasm begins with a complete neurological exam, including an Electromyography (EMG a
test that measures and records electrical activity generated in muscle at rest and in response to
muscle contraction), Magnetic resonance imaging (MRI a test that uses magnetic waves to
make pictures of structures inside the head), Computed tomography (CT scan a type of xray that uses a computer to make pictures of structures inside the head), and Angiography (an
x-ray exam of the blood vessels when they are filled with a contrast material).[16]
Studies have shown that the most effective method of hemifacial spasm screening is MRI. In
one study only 25% of the CT scans showed the abnormality in hemifacial spasm patients,
whilst more than half of the MRI imaging demonstrated a vascular anomaly. MRI imaging
should be the initial screening procedure in the assessment of patients with hemifacial
spasm.[17]
Treatments & Side Effects

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

and patients who had peripheral facial palsy or nerve lesion due
to tumors, demyelination, trauma, or infection as secondary hemifacial spasm. The study
found that 77% of hemifacial spasm is due to primary hemifacial spasm and 23% is due to
secondary hemifacial spasm. The study also found both sets of patients to share similar age at
onset, male to female ratios, and similar affected side.[28] Another study with 2050 patients
presented with hemifacial spasm between 1986 and 2009, only 9 cases were caused by
a cerebellopontine angle syndrome, an incidence of 0.44%.[13]
References
1. ^ a b c Kong, Doo-Sik; Kwan Park (2007). "Hemifacial Spasm: A Neurological
Perspective". Journal of Korean
362. doi:10.3340/jkns.2007.42.5.355.

Neurological

Society.

5 42 (5):

355

2. ^ a b c Jannetta, PJ (1998). "Typical or atypical hemifacial spasm". Journal of

Neurosurgery. 2 88: 3467.
3. ^ Ryu, H.; Yamamoto S., Miyamoto T. (1998). "Atypical hemifacial spasm". Acta
Neurochir 40 (11): 117376.doi:10.1007/s007010050233.
4. ^ a b c d Fukushima, T (1995). "Microvascular decompression for hemifacial spasm:
Result in 2890 cases". Neurovascular surgary (New York: McGraw Hill): 113345.
5. ^ "NINDS Hemifacial Spasm Information Page". National Institute for Neurological
Disorder and Strokes.
6. ^ Wilkins, RH (1991). "Hemifacial spasm: a review". Surgical Neurology 36 (4):
25177. doi:10.1016/0090-3019(91)90087-P. PMID 1948626.
7. ^ a b Gardner, J.W.; Sava, Gerard A. (1962). "Hemifacial Spasm - A reversible
pathophysiologic
state". Journal
of
Neurosurgery 27(3):
240
47. doi:10.3171/jns.1962.19.3.0240.
8. ^ Jannetta, PJ (1975). "Trigeminal neuralgia and hemifacial spasm--etiology and
definitive treatment". Trans Am Neurol Assoc 100: 8991. PMID 1226647.
9. ^ a b c Jannetta, PJ; Abbasy M, Maroon JC, Ramos FM, Albin MS. (1977). "Etiology
and definitive microsurgical treatment of hemifacial spasm. Operative techniques and
results
in
47
patients". Journal
of
Neurosurgery 47 (3):
321
8.doi:10.3171/jns.1977.47.3.0321. PMID 894338.
10. ^ a b c Eby, Joesph; Sung Tae Cha,Hrayr K. Shahinian (2002). "Fully endoscopic
vascular decompression of the glossopharyngeal nerve". The Journal of Craniofacial
Surgery 13(1): 9095. doi:10.1097/00001665-200201000-00021.PMID 11887002.
11. ^ Rahman, M.D. Ersalan; Jonathan D. Trobe, and Stephen S. Gebarski (June 2002).
"Hemifacial Spasm Caused by Vertebral Artery Dolichoectasia". American Journal
of Ophthalmology 133(6): 854855. doi:10.1016/S0002-9394(02)01387-9.

12. ^ Caces, F; Chays A, Locatelli P, Bruzzo M, Epron JP, Fiacre E, Magnan J. (1996).
"Neuro-vascular
decompression
in
hemifacial
spasm:
anatomical,
electrophysiological and therapeutic results apropos of 100 cases". Rev Laryngol Otol
Rhinol (Bord). 5 117: 34751.
13. ^ a b Lee SH, Rhee BA, Choi SK, Koh JS, Lim YJ; Jankovic J (2010).
"Cerebellopontine angle tumors causing hemifacial spasm: types, incidence, and
mechanism in nine reported cases and literature review". Acta Neurochir (Wien).
11 150 (11): 19018. doi:10.1007/s00701-010-0796-1.
14. ^ Toby C. Yaltho, MD 1 and Joseph Jankovic, MD (4/5/2011). "The Many Faces of
Hemifacial Spasm: Differential Diagnosis of Unilateral Facial Spasms". Movement
Disorders 29 (9).
15. ^ Office of Communications and Public Liaison. "NINDS Hemifacial Spasm
Information Page". National Institute of Neurological Disorders and Strokes.
Retrieved 11/03/2012.
16. ^ Zappia JJ; Wiet RJ; Chouhan A; Zhao JC, JJ; Wiet, RJ; Chouhan, A; Zhao, JC
(1997). "Pitfalls in the diagnosis of hemifacial spasm". The Laryngoscope. 4 107 (4):
461465.doi:10.1097/00005537-199704000-00007. PMID 9111374.
17. ^ a b c I. T. Lorentz (04/1995). "Treatment of hemifacial spasm with botulinum
toxin". Journal of Clinical Nueroscience. 2 2.
18. ^ Alexander GE, Moses H. (1982). Carbamazepine for hemifacial spasm
Neurology 32: 286287.
19. ^ PiattJH,J'r, Wilkins RH., JH; Wilkins, RH (1984). "Treatment of tic douloureux
and hemifacial spasm by posterior fossa exploration: therapeutic implications of
various
neurovascular
relationships". Neurosurgery 14 (4):
462
71.doi:10.1227/00006123-198404000-00012. PMID 6728149.
20. ^ HanakitaJ, Kondo A., J; Kondo, A (1988). "Serious complications of microvascular
decompression
operations
for
trigeminal
neuralgia
and
hemifacial
spasm". Neurosurgery 22(2):
34852. doi:10.1227/00006123-19880200000012.PMID 3352885.
21. ^ a b Tan, Eng-ing; Stephanie Fook-Chong, Sau-Ying Lum, Erle Lim (16).
"Botulinum toxin improves quality of life in hemifacial spasm: validation of a
questionnaire (HFS-30)". Journal of the Neurological Sciences 219 (12): 151
155.doi:10.1016/j.jns.2004.01.010. PMID 15050451.
22. ^ LoeserJD, ChenJ., John D.; Chen, James (1982). "Hemifacial spasm: treatment by
microsurgical
facial
nerve
decompression".Neurosurgery 13 (2):
141
46. doi:10.1227/00006123-198308000-00006.

23. ^ Toby C. Yaltho, and Joseph Jankovic, MD (04/04/2011). "The Many Faces of
Hemifacial Spasm: Differential Diagnosis of Unilateral Facial Spasms". Movement
Disorders 26 (9).
24. ^ Wang, A.; Jankovic J. (1998). "Hemifacial spasm: clinical findings and
treatment". Muscle and Nerve. 12 21 (12): 17407.doi:10.1002/(SICI)10974598(199812)21:12<1740::AID-MUS17>3.0.CO;2-V. PMID 9843077.
25. ^ Tan, N.C.; Chan, L.L, Tan E.K. (2002). "Hemifacial spasm and involuntary facial
movements". QJM. 8 95 (8): 493500.doi:10.1093/qjmed/95.8.493. PMID 12145388.
26. ^ Kemp, LW; Reich SG (6 2004). "Hemifacial spasm". Curr Treat Options
Neurol 3 (3): 1759. doi:10.1007/s11940-004-0009-4.
27. ^ Auger, RG; Whisnant JP (1990). "Hemifacial spasm in Rochester and Olmsted
County,
Minnesota,
1960
to
1984". Arch
Neurol 47 (11):
4.doi:10.1001/archneur.1990.00530110095023.PMID 2241620.

1233

28. ^ Colosimo, C; Bologna M, Lamberti S (2006). "A comparative study of primary and
secondary
hemifacial
spasm". Arch
Neurol.
6 63 (3):
441
4. doi:10.1001/archneur.63.3.441.PMID 16533973.