JNS-13861; No of Pages 5

Journal of the Neurological Sciences xxx (2015) xxxxxx

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Journal of the Neurological Sciences

journal homepage: www.elsevier.com/locate/jns

Review article

Hemifacial spasm: The past, present and future

Neera Chaudhry, Abhilekh Srivastava , Laxmikant Joshi
Department of Neurology, GB Pant Institute of Postgraduate Medical Education and Research, India

a r t i c l e i n f o a b s t r a c t

Article history: Hemifacial spasm is characterised by unilateral contractions of the facial muscles. Though considered to be be-
Received 12 February 2015 nign by many people, it can lead to functional blindness and a poor quality of life due to social embarrassment
Received in revised form 14 June 2015 for the suffering individual. Botulinum toxin therapy is an excellent noninvasive tool to treat this condition. How-
Accepted 15 June 2015
ever, surgical decompression of the aberrant vessel is also an upcoming approach to therapy for this condition.
Available online xxxx
2015 Published by Elsevier B.V.
Keywords:
Hemifacial spasm
Botulinum toxin therapy
Movement disorder
Review
Differential diagnosis
Pathogenesis
Surgical treatment

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
2. Historical perspective . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
3. Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
4. Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
5. Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
6. Clinical features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
7. Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
8. Electrophysiology in hemifacial spasm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
9. Differential diagnoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
10. Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
10.1. Drugs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
10.2. Botulinum toxin therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
10.3. Role of botulinum toxin therapy in treatment of non-motor symptoms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
10.4. Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
11. Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 0

1. Introduction intermittent twitching of the muscles innervated by the facial nerve.

Hemifacial spasm (HFS) is a movement disorder of the seventh
cranial nerve which is characterised by either brief or persistent,
Corresponding author.
E-mail address: mrabhilekh@gmail.com (A. Srivastava).
The hallmark of the disease is involuntary clonic and/or tonic contrac-
tions of the muscles of facial expression, usually unilaterally, beginning
in the periorbital musculature, but later on progressing to involve the
perioral, platysma and other muscles of facial expression as well.
Although traditionally perceived as a benign illness, it can lead to
increasing embarrassment and social withdrawal for the individual

http://dx.doi.org/10.1016/j.jns.2015.06.032
0022-510X/ 2015 Published by Elsevier B.V.

Please cite this article as: N. Chaudhry, et al., Hemifacial spasm: The past, present and future, J Neurol Sci (2015), http://dx.doi.org/10.1016/
j.jns.2015.06.032
2 N. Chaudhry et al. / Journal of the Neurological Sciences xxx (2015) xxxxxx
and in severe cases even functional blindness due to involuntary eye
closure. Thus, prompt diagnosis and timely therapy for the patient are
needed in such cases.
2. Historical perspective
F Schultze, in 1875, probably reported the rst case of hemifacial
spasm in literature, when he described a 56-year-old man with involun-
tary movements involving the left side of his face [1]. The post-mortem
revealed a giant aneurysm of the left vertebral artery compressing the
left facial nerve.
In 1886, Gowers elaborated on this syndrome further and described
the classical features of this condition [2]. 6 years later, douard
Brissaud, made similar observations when he described a 35-year-old
lady with clonic contractions of the muscles of right half of her face.
He observed that these contractions even though present at rest, wors-
ened at times of stress [3].
The condition received its current terminology, when it was named
as hmispasme facial by Babinski in 1905 [4]. Babinski at the same time
also described another characteristic feature of this disease, thereafter
known as the other Babinski sign i.e. when the orbicularis oculi con-
tracts and the eye closes, the internal part of the frontalis contracts at
the same time, and the eyebrow rises during eye occlusion. This typical
feature distinguishes hemifacial spasm from blepharospasm in which
this sign is absent.
3. Epidemiology
The exact prevalence of the disease is difcult to estimate owing to
the large number of under and misdiagnosis of this condition. Broadly
two forms of hemifacial spasm exist i.e. primary and secondary. Epide-
miological studies usually focus on the primary form. A study by
Auger and Whisnant from the United States evaluated data of patients
from 1960 through 1984 [1]. They observed that the mean prevalence
of the disorder was 11 per 100,000 total population, with a 2:1 female
preponderance (prevalence rate among Women being 14.5 per
100,000 and men being 7.4 per 100,000 population) [5]. Another
study by Nilsen et al. in 2004 from Oslo, Norway gave a similar preva-
lence of 9.8 per 100,000 [6]. Few studies suggest a slightly higher prev-
alence of the disease among some Asian populations compared to
Caucasians [7,8] however the reason for this high prevalence in this geo-
graphical population is not clear.
Primary hemifacial spasm has a wide age range of presentations, but
it typically begins in the fth to sixth decades of life. 1 to 6% of patients
present before the age of 30 years [9], however presentation before the
age of 40 years should prompt search for an underlying secondary cause
of the condition.
Hemifacial spasm is usually sporadic, and familial cases though
reported, are rare [10,11]. Bilateral disease is rare (b 1%) and even in
bilateral cases, the disease starts unilaterally, and after several months
to years, begins to involve the other side. The contractions remain asym-
metrical in such patients with the side being involved later, typically
having less severe manifestations.
4. Pathophysiology
The root exit/entry zone of a nerve is the junction between the cen-
tral and peripheral nerve segments of a cranial nerve. In this area there
is a transition of the cells responsible for myelination of a cranial nerve
.i.e. from the central oligodendroglial cells to the peripheral Schwann
cells. Also, the cranial nerves in this zone lack an epineurium, being
protected by an arachnoid membrane only. This special segment of
the nerve thus is highly susceptible to injury [12].
The most common cause for hemifacial spasm reported in literature
is an ectatic or aberrant blood vessel, which compresses the facial nerve
at this root entry/exit zone leading to local demyelination [13]. Several
Please cite this article as: N. Chaudhry, et al., Hemifacial spasm: The past, present and future, J Neurol Sci (2015), http://dx.doi.org/10.1016/
j.jns.2015.06.032
theories have been put forward to explain how this compression of
the facial nerve at its root exit/entry zone leads to hemifacial spasm.
One of them the nerve origin hypothesis or the peripheral theory
postulates that there is ephaptic transmission of impulses between
neighbouring neurons (i.e. coupling of adjacent nerve bres due to
local exchange of ions or local electric elds) leading to excessive or
abnormal ring. Myelination is a natural inhibitor of ephaptic transmis-
sion and the demyelination due to local compression thus leads to
hemifacial spasm [11].
The other the nuclear origin hypothesis or the central theory
states that hemifacial spasm results from the hyperexcitability of the fa-
cial motor nucleus due to irritative feedback from peripheral lesions of
the nerve [14].
5. Etiology
As previously described, hemifacial spasm can either be primary or
secondary. Primary HFS results from compression of the seventh
nerve at the root exit zone in the posterior cranial fossa by an aberrant
or ectatic vessel, most commonly the superior cerebellar, anterior infe-
rior cerebellar or vertebral artery [15].
The list for causes of secondary hemifacial spasm is a long one and
includes [16]:
1. Cerebellopontine angle tumours acoustic neuroma, meningioma
2. Epidermoid, arachnoid cyst, lipoma
3. Arteriovenous malformations stulas, venous angiomas and arterial
aneurysms
4. Brainstem lesions stroke, trauma, demyelinating disorders
5. Infections otitis media, tubercular meningitis
6. Structural abnormalities of the posterior cranial fossa Paget's disease, Chiari
malformation
7. Parotid tumours
8. Bell's palsy
6. Clinical features
Hemifacial spasm classically begins unilaterally in the upper face
(around the eyes) most commonly in the orbicularis oculi muscle
(90%) [17], brief repetitive contractions of which lead to sudden, invol-
untary eye closure. This is typically associated with elevation of the
eyebrows the other Babinski sign. Patients usually complain of a
urry of twitches eventually leading to a sustained spasm.
This presentation is more commonly seen in the primary type of
hemifacial spasm, whereas simultaneous involvement of the upper
and lower face is more typical for secondary cases [18]. Naraghi et al. be-
lieve that this observation has a basis in the anatomy of the facial nerve
and nucleus [19]. In the facial nerve, the bres innervating the upper
part of the face are present dorsally, and the anterior inferior cerebellar
artery, which is the most common culprit vessel responsible for the
syndrome, is most commonly located in relation to the dorsal aspect
of the nerve in a majority of patients.
After beginning in the upper half of the face, the contractions gradu-
ally spread over time to involve the lower half of face as well i.e. the
perioral muscles and eventually the platysma leading to their irregu-
lar clonic or tonic contractions. Unlike most movement disorders, con-
tractions of hemifacial spasm persist during sleep which may add to
the morbidity of the condition by predisposing the affected individual
to disturbed sleep and insomnia. Other rare ndings in patients with
hemifacial spasm may include paroxysmal clicking sounds in the ear
due to involvement of the stapedius muscle, unilateral or bilateral
hearing loss and subtle facial nerve palsy [16]. As classically noted by
Brissaud, the symptoms increase during times of stress, reading, speak-
ing and sometimes eating while they abate with relaxation techniques
and occasionally touching some parts of the face (sensory tricks).
 N. Chaudhry et al. / Journal of the Neurological Sciences xxx (2015) xxxxxx
There are reports of association of hypertension with the hemifacial
spasm in as many as 40% of cases [20]. Hypertension has been explained
to be both as a cause and effect of the disease. Theoretically hyperten-
sion can contribute to vascular changes contributing to vessel ectasia
which is the underlying cause in the majority of patients. On the other
hand cases of left hemifacial spasm due to an aberrant vessel may
compress the root entry zone of cranial nerves 9 & 10, thus modulating
the cranial parasympathetic outow and contributing to hypertension.
The natural history of the disease is that of a chronic one with
gradual worsening of symptoms however spontaneous resolution has
been seen in less than 10% of cases [21].
7. Diagnosis
Diagnosis of hemifacial spasm is mainly a clinical one. All patients
must be subjected to a detailed history and clinical examination to
look for any subtle neurological decits which would point to an under-
lying secondary cause for the condition. Other investigations that may
be ordered in order to rule out secondary causes include an electromy-
ography (to rule out facial nerve lesions resulting in denervation), and
an MRI of the brain to rule out demyelination or space occupying lesions
near the brainstem. Patients in whom medical management has failed
and a surgical intervention is being planned need an MR Angiography
(with special high resolution sequences like CISS constructive
interference in steady state) as well [12].
8. Electrophysiology in hemifacial spasm
The electrophysiological hallmark of hemifacial spasm is spread of
the blink reex to muscles other than the orbicularis oculi. The proposed
explanation for the phenomenon is the lateral spread of the antidromic
impulse between neighbouring bres of the facial nerve via ephaptic
transmission. The most important clinical implication and use of
electromyography and recording of the lateral spread response lie in
helping the surgeons to determine whether adequate decompression
has been achieved. When the aberrant vessel is moved off the facial
nerve, the Lateral Spreading Response is known to disappear or become
markedly reduced.
It has also been seen that botulinum toxin is preferentially taken up
by hyperactive synapses as the ones involved in ephaptic transmission.
A study looking into the electrophysiological responses post-botulinum
toxin therapy showed an average 40% reduction of the orbicularis oculi
CMAP amplitudes in these patients, whereas the Lateral Spreading Re-
sponse could not be recorded in any injected patients [22]. However,
the role of routine use of electrophysiological studies prior to botulinum
toxin therapy still remains to be dened clearly.
9. Differential diagnoses
A long list of disorders which can mimic hemifacial spasm includes
1. Blepharospasm
2. Tardive dyskinesias
3. Motor tics
4. Psychogenic hemifacial spasm
5. Focal cortical seizures involving the facial muscles
6. Aberrant regeneration after facial nerve injury.
Blepharospasm in contrast to hemifacial spasm which is unilateral
in the majority of cases, involves bilateral synchronous contractions of
the orbicularis oculi. Even in the rare cases of bilateral hemifacial
spasm, the contractions on both sides of the face are asynchronous.
The other classical differentiation is the lowering of the eyebrow on clo-
sure of eyes (Charcot's sign), whereas there is raising of eyebrows with
each contraction in cases of hemifacial spasm (the other Babinski sign).
Tardive dyskinesia is seen in individuals with history of exposure to
neuroleptic agents or dopaminergic antagonists. Typical presentations
Please cite this article as: N. Chaudhry, et al., Hemifacial spasm: The past, present and future, J Neurol Sci (2015), http://dx.doi.org/10.1016/
j.jns.2015.06.032
3
include stereotypical movements of the face (perioral region), neck,
trunk, and limbs.
Motor tics are involuntary, brief, repetitive, stereotyped, movements
which can be willfully suppressed which most commonly manifest as
blinking or facial twitching [23].
Focal cortical seizures involving the facial muscles are continuous,
repetitive, stereotyped movements of the face and head may be seen
in focal motor seizures and may require an electroencephalogram to dif-
ferentiate them from hemifacial spasm [24].
Aberrant regeneration of the facial nerve after injury manifests with
synkinesias such closure of eyes on voluntary mouth opening but can be
easily differentiated from hemifacial spasm since these abnormal move-
ments are absent at rest.
Facial myokymia is characterised by involuntary undulating, rippling
movements of the facial muscles. This is a benign condition and most
cases resolve spontaneously in a few days or weeks. These movements
are worsened by fatigue, sleep deprivation, or excess caffeine
consumption.
Psychogenic hemifacial spasm is characterised by non-patterned fa-
cial movements that vary in intensity and frequency, and are easily dis-
tractible [25].
Another term commonly used while discussing hemifacial spasm is
peripheral myoclonus. Peripheral myoclonus is characterised by rhyth-
mic or semirhythmic jerks secondary to plexus, nerve, root lesion or
rarely anterior horn cell disease. Hemifacial spasm is considered to be
the most common example of peripheral myoclonus, while other causes
are relatively rare.
10. Treatment
Hemifacial spasm is a chronic condition with progressively increas-
ing spasms. However due to the low prevalence of the condition, not a
lot of controlled clinical trials have been done to determine the best
therapeutic modality for these patients. Although, botulinum toxin re-
mains the most popular choice for therapy, other options including
oral pharmacotherapy and microsurgical decompression surgeries are
also benecial in selected cases.
10.1. Drugs
A large number of drugs have been studied and found to be of some
efcacy in hemifacial spasm. These include anticonvulsants such as car-
bamazepine, clonazepam, gabapentin and other drugs like baclofen, an-
ticholinergics and haloperidol [16]. The biggest limitation of oral drugs
is their inconsistent efcacy and large number of side effects including
sedation, fatigue and exhaustion [26].
10.2. Botulinum toxin therapy
Botulinum toxin is a potent biological toxin derived from the organ-
ism Clostridium botulinum [27]. It acts on the presynaptic region of the
neuromuscular junction and prevents the calcium-mediated release of
acetylcholine at the nerve terminal preventing impulse generation
downstream resulting in functional reversible paralysis of the supplied
muscles. Based on the target site of action there are various serotypes
of botulinum toxin.
Currently, in the United States, there are four BoNT formulations
licenced for use: abobotulinumtoxinA, incobotulinumtoxinA,
onabotulinumtoxinA, and rimabotulinumtoxinB.
The most commonly used commercially available preparation is
onabotulinumtoxinA. A large number of trials have validated the
successful outcomes of this therapy with improvements in as many as
75100% of individuals with hemifacial spasm [2830].
The toxin is diluted to a minimal concentration to minimize diffusion
and injected subcutaneously. Injection in the upper part of the face (and
the platysma) is often sufcient by themselves and the perioral muscles
4 N. Chaudhry et al. / Journal of the Neurological Sciences xxx (2015) xxxxxx
Table 1
Commonly used doses for onabotulinumtoxinA in hemifacial spasm.
Muscle No of injections Usual dose of toxin
Orbicularis oculi 36 per eye 2.55 U/site 530 U/site
Procerus 1 per side 4 U/side
Mentalis 1 per side 4 U/side
Platysma 12 per strand up 10 U 520 U
to 6 per side
Orbicularis oris 46 2U
Depressor anguli oris 1 12 U
should be selected only in severe cases since toxin therapy at these
places can lead to drooping of the angle of mouth and weakness
(Table 1).
Compared to onabotulinumtoxinA doses for other preparations of
the toxin are presented in Table 2.
Total doses used for hemifacial spasm per therapy session range
from 10 to 34 U of onabotulinumtoxinA [32]. For abobotulinumtoxinA
total doses range from 53 to 160 U [33,34]. For rimabotulinumtoxinB
doses ranging from 1250 to 9000 U are used [35]. Therapeutic effect is
seen in as early as 36 days after the injections. The resulting muscular
weakness due to the injected toxin lasts for around 23 months. Lower
doses are used at the initiation of therapy and uptitrated depending on
response to therapy.
The usual doses of injected botulinum toxin have been seen to in-
crease during the course of therapy. Possible reasons that have been
put forward for this observation include use of low starting doses, and
requirement of higher doses as disease progresses (i.e. involvement of
other muscles like the platysma, not present at the outset). Ababneh
et al. in a long term follow-up study on botulinum toxin therapy in pa-
tients with blepharospasm and hemifacial spasm found higher mean in-
jection dose per visit during the last year compared to the rst year in
their patients. They also noticed an increase in duration of effect of
toxin therapy by almost two weeks when compared to duration of relief
in symptoms with the toxin on treatment initiation [36].
The sessions of botulinum toxin therapy are commonly scheduled at
three monthly intervals (based on the expected duration of effect of the
drug). However, in some patients the benecial effects wane quicker.
Sethi et al., in a survey of patients on botulinum toxin therapy noticed
this need in almost 45% of their patients who indicated a preferred treat-
ment interval of 10 weeks [37]. Thus a more exible dosing interval
based on patients' needs (between 6 and 20 weeks) may lead to overall
improvement in patient satisfaction with the treatment.
Botulinum therapy is not without its drawbacks. The major limiting
factor is the cost of therapy and need for repeat injections at an interval
of three to six months. Apart from these two major limitations other
adverse effects of botulinum toxin include mild facial paresis (23%),
diplopia (17%), and ptosis (15%) [16,38]. Trauma from subcutaneous in-
jections can lead to temporary bruising, however no serious systemic
side effects of the therapy have been reported till date. Concerns about
immunoresistance to botulinum toxin are not signicant in cases of
hemifacial spasm due to low doses involved. As a result in view of the
excellent efcacy, minimal side effects and the ease of therapy, it is
now widely considered as the therapeutic modality of choice for
management of hemifacial spasm.
Table 2
Various preparations of botulinum toxin in hemifacial spasm [31].
Botulinum toxin preparation Frontalis Corrugator
OnabotulinumtoxinA 10 1 1520
AbobotulinumtoxinA 30 3 4560
RimabotulinumtoxinB 500 50
Please cite this article as: N. Chaudhry, et al., Hemifacial spasm: The past, present and future, J Neurol Sci (2015), http://dx.doi.org/10.1016/
j.jns.2015.06.032
10.3. Role of botulinum toxin therapy in treatment of non-motor symptoms
Range Depression commonly accompanies hemifacial spasm. Rudziska
et al. [39] showed a high prevalence of depressive symptoms in patients
2.57 U/side with hemifacial spasm. There was also a correlation of severity of
2.55 U/side hemifacial spasm with higher scores on the depression scale. Tan et al.
also reported similar ndings [40]. It has also been seen that treatment
with botulinum toxin has led to improvement in the symptoms of de-
1.757.5 U
2.56 U pression in these patients. The mechanism postulated for the effect of
botulinum toxin is twofold. The rst is that there is an improvement
in symptoms of the disease per se, which leads to reduced social embar-
rassment for the individual. The other mechanism is what is known as
the facial feedback hypothesis. According to this theory facial muscles
provide feedback to the brain (amygdala). Expressions of frowning
may be associated with a negative feedback contributing to depression
in some individuals. Motor denervation of these muscles by botulinum
toxin reduces afferent sensory information from the trigeminal tract
to the brainstem and amygdala, which reduces hyperactivity in the
amygdala which has been linked to anxiety and depression. Thus
botulinum toxin therapy not only improves the symptoms of the dis-
ease but contributes to relief in non-motor symptoms like depression
and an overall improvement in health related quality of life.
10.4. Surgery
With the advent of botulinum toxin therapy, the need for operative
intervention has drastically gone down in cases of hemifacial spasm.
Nonetheless, surgery is the only modality which can offer complete
cure to the disease unlike toxin therapy which offers temporary symp-
tomatic relief.
The surgical procedure of choice is microvascular decompression of
the facial nerve which aims to remove the compression of the seventh
nerve at the root exit zone by the aberrant/ectatic vessel. The surgical
procedure has a success report of up to 90% in some case series [41],
however the major problem of the surgery is the risks of an invasive
procedure (including post-operative infections and general anaesthesia
complications) and a large number of procedure related complications
including recurrence of disease (20%) [42], hearing loss (726%) [43],
transient or permanent facial nerve paralysis, and CSF leakage (23%).
As a result the surgical option is mainly reserved for those patients
who either are not responding to botulinum toxin therapy or prefer to
opt for a permanent cure for the condition.
11. Conclusion
The benign appearing facial twitches of hemifacial spasm are actual-
ly very bothersome to the individuals who suffer from the condition
both in terms of functional blindness as well as social embarrassment.
Early recognition of the condition, ruling out secondary causes and insti-
tuting appropriate therapy are therefore a necessity. Botulinum toxin
therapy offers a simple, non-invasive therapy for this condition. Patients
who do not respond to the toxin injections or prefer a permanent cure
can be offered surgical options as well. Either treatment modality
when instituted gives good benets to the patient and leads to a signif-
icant improvement in their quality of life.
Orbicularis oculi Zygomaticus Buccinator Depressor anguli oris
1 2 1
3 6 3
1000 50 100 50
 N. Chaudhry et al. / Journal of the Neurological Sciences xxx (2015) xxxxxx 5

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Please cite this article as: N. Chaudhry, et al., Hemifacial spasm: The past, present and future, J Neurol Sci (2015), http://dx.doi.org/10.1016/
j.jns.2015.06.032