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Hemifacial Spasm

Lawrence W. Kemp, MD
Stephen G. Reich, MD
Address
*Department of Neurology, University of Maryland School of Medicine,
22 South Greene Street, Baltimore, MD 21201, USA.
E-mail: sreich@som.umaryland.edu
Current Treatment Options in Neurology 2004, 6:175179
Current Science Inc. ISSN 1092-8480
Copyright 2004 by Current Science Inc.

Opinion statement
Hemifacial spasm (HFS) is a peripheral movement disorder caused by direct or indirect
compression or distortion of the root exit zone of the seventh cranial nerve, which is
most commonly compressed by an arterial loop, but also may be compressed by a
tumor, cyst, or aneurysm. All patients with HFS should undergo magnetic resonance
imaging, with particular attention to the seventh cranial nerve. For patients with
HFS who want treatment, there are three options. Oral medications, particularly
anticonvulsants, may be useful, but the response rate is low and evidence is almost
exclusively anecdotal. Local injection of botulinum toxin into the overactive muscles
has a very high rate of success and virtually no serious side effects. Backed by
controlled clinical trials, the authors consider it the treatment of choice. Microvascular
surgical decompression has the advantage of being potentially curative, and obviates
the need for chronic injections with botulinum toxin. However, surgery carries much
greater risk than botulinum toxin and the spasm may recur. It is important that
surgery is carried out by an experienced neurosurgeon to reduce the risk.

Introduction
Hemifacial spasm (HFS) aptly names a movement Even if HFS begins in the orbicularis oculi or another
disorder characterized by tonic or clonic spasms involv- muscle innervated by the facial nerve, it usually spreads
ing one side of the face [1, Class III; 2,3]. Rarely is HFS to involve most divisions with time. Although some
bilateral [4, Class III]. HFS is almost always sporadic, patients are not bothered by the spasms, others complain
but rare familial cases have been described [5, Class III]. that it interferes with vision or speaking and it often
In his classic 1952 monograph, Wartenberg [6] causes embarrassment [1]. Continuously winking at
described 13 distinctive clinical characteristics of HFS, others also can lead to problems. There is virtually never
including its adult onset and that HFS often originates pain, unless there is simultaneous involvement of the
in the orbicularis oculi. Irregularity in site, rhythm, and trigeminal nerve, but patients may experience mild dis-
degree is the salient feature [6]. The muscular contrac- comfort. In long-standing cases, even when not preceded
tions occur in intermittent, irregular attacks and show by Bells palsy, one can observe mild lower-motor neuron
great variability, from quivering, flickering, fibrillary, facial weakness and a motor synkinesia. HFS must be
myokymia-like twitchings of parts of the muscle or of differentiated from other facial dyskinesias, including
the whole muscle, to violent clonic spasms involving blepharospasm-facial dystonia, motor tics, tardive dyski-
the entire facial musculature of one side [6]. Warten- nesia, myoclonus, and myokymia [7].
berg [6] further observed that illogical contractions of The annual incidence of HFS in residents of Olm-
muscles, such as the orbicularis oculi and frontalis sted County, MN, between 1960 and 1984 was 0.74 per
occur simultaneously. Other Wartenbergs characteris- 100,000 in men and 0.81 per 100,000 in women. The
tics, which have stood the test of time, include persis- average prevalence rate was 7.4 per 100,000 in men and
tence of HFS during sleep, inability of the patient to 14.5 per 100,000 in women. Incidence and prevalence
voluntarily suppress the spasms, and the lack of a rates were highest in those ages 40 to 79 [8, Class III].
compulsion to perform the movement, thus differenti- Wartenberg [6] was emphatic that this distinct clin-
ating HFS from a motor tic [6]. ical entityis certainly not a psychogenic affection. He
176 Movement Disorders

proposed that the disorder arose from uninhibited reported, almost exclusively in noncontrolled studies,
activity emanating from the facial nucleus. Today, it is to be effective, but most patients do not benefit. Wang
generally well accepted that HFS is caused by extrinsic and Jankovic [1, Class III] found that only 8% of their
compression of the root exit zone of the facial nerve, patients treated with oral medications experienced
resulting in ephaptic transmission or cross talk [9]. meaningful benefit. Nevertheless, there are several
Moller and Jannetta [10] suggested that HFS was caused medications available that provide an option for the
by a process similar to kindling. In almost all cases, exceptional patient who has a suboptimal response to
the compression is caused by an arterythe anterior botulinum toxin and who is not a surgical candidate.
inferior cerebellar artery, the posterior inferior cerebel- During the past two decades, local injection of botuli-
lar artery, the vertebral artery, or the basilar artery [11, num toxin has emerged as the treatment of choice for HFS
Class III]. Vascular compression also can be demon- based on its high success rate and low complication rate.
strated radiographically. Adler et al. [12, Class II], using Botulinum toxin works by preventing the release of acetyl-
magne tic resonance tomograp hic angi ography, choline from the presynaptic nerve terminal thereby caus-
observed compression of the facial nerve or pons in two ing reversible, local weakness where injected [15]. The
thirds of patients with HFS and only 6% of control sub- limited duration of improvement from botulinum toxin
jects. HFS also can be a sign of a mass lesion directly or and the need for ongoing injections, as well as the occa-
indirectly compressing or distorting the facial nerve. sional patient who does not benefit from botulinum
Case reports include a variety of lesions, including neo- toxin, leads some patients to undergo microvascular
plasm, cyst, arteriovenous malformation, bony abnor- decompression of the facial nerve, a procedure refined
mality, many of which have been reviewed by Galvez- and popularized by Jannetta et al. [11].
Jimenez et al. [13]. Although Wartenbergs [6] statement No matter the therapy, if any, that is chosen, all
that HFS is certainly not psychogenic is almost always patients with HFS should be referred to the Benign Essen-
correct, there are rare exceptions [14]. tial Blepharospasm Research Foundation (Beaumont, TX;
There are four main treatment options for HFS. The http://www.blepharospasm.org/). In addition to
first is no treatment at all. Patients who are not particu- blepharospasm, the Benign Essential Blepharospasm
larly bothered by HFS, and who do not have a mass on Research Foundation also is concerned with HFS. This
magnetic resonance imaging, can be reassured that they organization and its literature serve as valuable sources of
have a benign condition. With time, they may opt for information for patients, as well providing mutual
therapy. Second, a variety of oral medications have been understanding, support, advocacy, and funding research.

Treatment
Pharmacologic treatment
Carbamazepine
In 1982, just before the introduction of botulinum toxin for HFS, Alexander
and Moses [16, Class III] reported improvement in three patients. Including
their patients, a literature review demonstrated that approximately 50% of 46
patients treated with carbamazepine improved or experienced complete control.
None of these studies was controlled.
Standard dosage 400 to 1200 mg per day.
Contraindications Known hypersensitivity to drugs in this class, including tricyclic antidepressants.
Use with caution in the presence of blood dyscrasia; liver, cardiac, or renal
disease; porphyria or lupus; pregnancy.
Main drug interactions Carbamazepine can alter the blood level or effectiveness (positive or negative)
of many commonly used medications, including other anticonvulsants, oral
contraceptives, antibiotics, antidepressants, and anticoagulants.
Main side effects Rash (Stevens-Johnson syndrome), cytopenia, hyponatremia, dizziness, nausea,
and ataxia.
Special points Generic preparation is available. Blood levels can be monitored for compliance
and toxicity.
Cost/cost effectiveness Cost is approximately $13.99 for 60 200-mg generic tablets.
Hemifacial Spasm Kemp and Reich 177

Gabapentin
Gabapentin has been reported to be beneficial for HFS in three noncontrolled
case series including one, five, and 23 patients, respectively [1719, Class III].
Standard dosage 600 to 1200 mg per day.
Contraindications Caution in the elderly and in patients with renal insufficiency.
Main drug interactions Gabapentin is largely free of serious drug interactions.
Main side effects Leukopenia (rare), somnolence, and dizziness.
Special points Gabapentin is generally well tolerated, especially in the elderly, in whom it should
be started at the lowest dose possible (100 mg per day) and escalated gradually.
Gabapentin is expensive, especially compared with carbamazepine.
Cost/cost effectiveness Cost is approximately $189.00 for 90 600-mg tablets.

Clonazepam
Clonazepam is a popular drug for a variety of hyperkinetic movement disorders.
Its use in HFS, like other oral medications, is anecdotal [20, Class III].
Standard dosage 0.5 to 4 mg per day.
Contraindications Liver disease.
Main drug interactions Clonazepam interacts with multiple medications.
Main side effects Drowsiness, weakness, dizziness, lightheadedness, headache, clumsiness,
or unsteadiness.
Special points Start with 0.25 mg at night and escalate gradually.
Cost/cost effectiveness Cost is approximately $11.00 for 30 0.5-mg tablets.

Baclofen
Noncontrolled case reports document the potential effectiveness of baclofen
for HFS [21, Class III].
Standard dosage 15 to 80 mg per day.
Contraindications Renal insufficiency; seizure disorder.
Main drug interactions Baclofen interacts with multiple medications.
Main side effects Seizures, somnolence, dizziness, and confusion.
Special points Start low and escalate gradually.
Cost/cost effectiveness Cost is approximately $10.00 for 30 10-mg tablets.

Botulinum toxin
The use of botulinum toxin type A (BTX-A) for treatment of HFS in patients over
12 years of age has been approved by the US Food and Drug Administration since
1989. BTX-A has proven to be an effective, well-tolerated, long-term treatment with
minimal side effects. As of 2001, open-label studies reported the experience in
treating over 2200 patients [2230]. In contrast, less than 100 patients were treated
in double-blind, placebo-controlled studies [31], but results have been similar.
Yoshimura et al. [32, Class I] treated 11 patients. Patients reported improve-
ment after 79% of BTX-A injections, whereas only one patient reported improve-
ment after placebo. Objective improvement was observed after 84% of botulinum
injections compared with none of the placebo injections. In the study by Park
et al. [33, Class III], which combined the results of placebo-controlled and
open-label treatment, almost 100% of patients had an excellent result. The
reported efficacy of BTX-A in HFS ranges from 86% to 100%, with a duration
of effect from 12 to 20 weeks.
Development of tolerance to BTX-A has not been a problem, and the response
has been stable over at least 10 years. In a study of 70 patients with HFS who
underwent a total of 630 treatment cycles with BTX-A, Hsiung et al. [29, Class III]
observed 96% (67 of 70) of patients still had a good response after 2 years,
and 88% (35 of 40) of patients continued to benefit after 5 years of treatment.
Similarly, Defazio et al. [30, Class III], in a 10-year multicenter study, showed that
the effectiveness of BTX-A for HFS remained unchanged (95% response rate) at 10
178 Movement Disorders

years compared with 1 year of treatment. Hsiung et al. [29] found that only 7%
(five of 70) of patients discontinued BTX-A because of resistance. The primary
reason for stopping BTX-A was a decision to undergo microvascular decompression
surgery. In contrast, Wang and Jankovic [1, Class III] observed no cases of immu-
noresistance to BTX-A therapy in 158 HFS patients after 15 years of follow-up.
Botulinum toxin has become the mainstay of therapy for most patients with
HFS. The safety and efficacy is operator-dependent, and patients should be treated
by a physician experienced in diagnosing HFS and administering BTX-A injections.
Standard procedure Once reconstituted, botulinum toxin is injected into the overactive muscles.
Initially, 20 to 30 U are injected, divided among four to six sites in the orbicularis
oculi, with additional injections of 2.5 to 5.0 U injected into the lower face.
The dosage must be individualized.
Contraindications Infection or inflammation at injection site. Myasthenia gravis.
Complications Transient side effects of BTX-A injections are usually not severe and include ptosis,
excessive facial weakness (mouth droop), diplopia, dry eyes, eyelid edema, tearing,
corneal exposure, and difficulty focusing (caused by ciliary muscle weakness).
The most common side effect is ptosis, with an incidence ranging from 0% to 20%.
Systemic side effects such as nausea, generalized weakness, or malaise have been
reported, but these are uncommon and transient.
Special points Patients should be counseled that BTX-A is not a cure for HFS. It may take several
injection sessions to find the optimal dosage and injection sites.
Cost/cost effectiveness This is variable and includes the cost of the toxin plus a procedural fee, typically
totaling $300 to $500.

Surgery
Microvascular decompression
Standard procedure This procedure is based on the finding of a crossing artery (rarely a vein) at the root
exit zone in most cases of HFS. It was initially performed by Gardner and Sava [34],
and refined and popularized by Jannetta et al. [11] who wrote, The principle of
treatment is to decompress the facial nerve root exit zone by changing the axis of the
arterial loop through interposition of a small prosthesis of non-resorbable spongy
material between the proximal and distal limbs of the loop and the brain stem.
There are numerous case series (Class III) documenting the efficacy of
microvascular decompression and its complications. Two of the largest and most
detailed will be briefly reviewed here. Barker et al. [35] reported their experience
in 782 operations on 703 patients over a 20-year period, most of which were
performed by Jannetta (Unpublished data). In the group of patients who had their
first operation by the authors, 84% were described as having an excellent result at
10 years, 7% had a partial success, and there was a 9% failure rate. There was a
recurrence rate of 9%. The most common complications included transient facial
weakness (3.2%), followed by unilateral deafness (2.7%) and cerebrospinal fluid
leak (2.4%). There was only one operative death. In a series of 310 patients,
Huang et al. [36, Class III] reported comparable outcomes.
Contraindications Medically unstable to undergo craniotomy. Age does not appear to be a contraindi-
cation. Barker et al. [35] found that women and those with atypical symptoms
had a lower success rate.
Complications Permanent facial weakness (>4%), hearing loss (>3%), stroke or hemorrhage
(0.8%), cerebrospinal fluid leak (2.4%), wound infection or meningitis (1.7%),
and death (0.1%) [35]. Recurrence rates vary. In the series by Wang and Jankovic
[1], recurrence was 20%, but much lower in surgical series.
Special points The high success and low complication rates reported in large series reflect
extensive operative experience. Patients contemplating surgery should seek
out an experienced neurosurgeon.
Cost/cost effectiveness Variable; approximately $10,000 to $20,000.
Hemifacial Spasm Kemp and Reich 179

References and Recommended Reading


Papers of particular interest, published recently, have been highlighted as:
Of importance
Of major importance
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This is nice review of the differential diagnosis of HFS. treatment of hemifacial spasm: a report of 175 cases.
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