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REVIEW

The Many Faces of Hemifacial Spasm: Differential Diagnosis of


Unilateral Facial Spasms
Toby C. Yaltho, MD1 and Joseph Jankovic, MD*

Parkinsons Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas, USA

A B S T R A C T : Hemifacial spasm is dened as uni- pathic hemifacial spasm (presumably caused by vas-
lateral, involuntary, irregular clonic or tonic movement cular compression of the ipsilateral facial nerve), and 4
of muscles innervated by the seventh cranial nerve. (2%) had hereditary hemifacial spasm. Secondary
Most frequently attributed to vascular loop compres- causes were found in 40 patients (19%) and included
sion at the root exit zone of the facial nerve, there are Bells palsy (n 5 23, 11%), facial nerve injury (n 5 13,
many other etiologies of unilateral facial movements 6%), demyelination (n 5 2), and brain vascular insults
that must be considered in the differential diagnosis of (n 5 2). There were an additional 38 patients (18%)
hemifacial spasm. The primary purpose of this review with hemifacial spasm mimickers classied as psycho-
is to draw attention to the marked heterogeneity of genic, tics, dystonia, myoclonus, and hemimasticatory
unilateral facial spasms and to focus on clinical char- spasm. We concluded that although most cases of
acteristics of mimickers of hemifacial spasm and on hemifacial spasm are idiopathic and probably caused
atypical presentations of nonvascular cases. In addi- by vascular compression of the facial nerve, other eti-
tion to a comprehensive review of the literature on ologies should be considered in the differential diagno-
hemifacial spasm, medical records and videos of con- sis, particularly if there are atypical features. V
C 2011

secutive patients referred to the Movement Disorders Movement Disorder Society


Clinic at Baylor College of Medicine for hemifacial
spasm between 2000 and 2010 were reviewed,
and videos of illustrative cases were edited. Among Key Words: hemifacial spasm; tics; dystonia;
215 patients referred for evaluation of hemifacial myoclonus; psychogenic; hemimasticatory spasm;
spasm, 133 (62%) were classied as primary or idio- blepharospasm

Hemifacial spasm (HFS) is dened as involuntary, cial, perioral, and platysma muscles, often leading to
irregular clonic or tonic movement of muscles inner- social embarrassment and interference with vision
vated by the ipsilateral seventh cranial nerve. The from involuntary eye closure.1 In 1893, Edouard Bris-
spasms usually start as twitching of the lower eyelid, saud, a French neuropsychiatrist, provided one of the
followed by involvement of the other periorbital, fa- rst pictures and descriptions of unilateral facial
spasms in a 35-year-old woman with apparent HFS.2
In 1905, Babinski described paradoxical synkinesis in
------------------------------------------------------------ HFS when orbicularis oculi contracts and the eye
Additional Supporting Information may be found in the online version of
this article. closes, the internal part of the frontalis contracts at the
*Correspondence to: Dr. Joseph Jankovic, Professor of Neurology, same time, the eyebrow rises during eye occlusion.3
Director of Parkinsons Disease Center and Movement Disorders Clinic, This other Babinskis sign is typically present in
Department of Neurology, Baylor College of Medicine, 6550 Fannin,
Suite 1801, Houston, TX 77030, USA; josephj@bcm.tmc.edu
HFS, but not in essential blepharospasm.4,5 Although
Relevant conicts of interest/nancial disclosures: Nothing to report. nearly always unilateral, rare cases of bilateral HFS
Full nancial disclosures and author roles may be found in the online have been reported.615 In 1 movement disorders
version of this article.
clinic, 10 of 382 consecutive patients with HFS (2.6%)
Received: 18 August 2010; 29 January 2011; Accepted: 3 February had bilateral involvement.14 Bilateral HFS usually
2011
Published online 5 April 2011 in Wiley Online Library starts as a unilateral HFS, and after a latency of several
(wileyonlinelibrary.com). DOI: 10.1002/mds.23692 months or years, the contralateral side becomes

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involved. The bilateral contractions are asynchronous were also identied through searches of the authors
and asymmetrical, with the most recent side usually own les. The characteristics of patients referred
being less severely involved than the original side. to Baylor College of Medicine for evaluation of HFS
Although HFS is often attributed to a vascular com- are summarized in Table 1, and cases reported in
pression of the ipsilateral facial nerve, there are other the literature, organized according to etiology, are
etiologies that should be considered in the differential summarized in Table 2.
diagnosis. Indeed, HFS is often confused with other
facial movement disorders, such as blepharospasm, Classication
oromandibular dystonia, facial myorhythmia, facial
tic, facial myokymia, and hemimasticatory spasm.1618 We have organized the discussion according to etiol-
Prior medical history, phenomenology of involuntary ogic classication and whenever possible include an
facial movements, accompanying symptoms, and coex- illustrative video of a case selected from the series of
istent disorders, as well as abnormal ndings on patients evaluated at the Movement Disorders Clinic,
neurological examination and neuroimaging usually Baylor College of Medicine.
provide clues to the etiology.
The primary goal of this article is to review the lit- Primary or Idiopathic HFS (Case 1)
erature on HFS and present illustrative cases to The largest group in our series, consisting of 133
draw attention to the differential diagnosis of HFS, of 215 patients (62%), had HFS without an identia-
focusing on secondary and atypical cases. During the ble etiology (Table 1). The mean age was 55.4 6
period from January 2000 to February 2010, 215 15.0 years, and mean duration was 10.9 6 7.6 years,
patients with possible HFS were referred to the the male:female ratio was 1:1.8, and 54% presented
Movement Disorders Clinic at Baylor College of with left HFS. Although in 6 cases imaging studies
Medicine. All patients underwent a detailed neurolog- suggested the possibility of an enlarged ectatic
ical examination and were evaluated by a movement artery and vascular compression of the facial nerve
disorders specialist. The medical records and videos of was thought to be the most likely mechanism of
these patients were retrospectively reviewed. Informa- the HFS, we classied these cases as idiopathic
tion about demographics, clinical features, and etiol- because a vascular etiology could not be conrmed.
ogy was entered into a database and analyzed. Based The presence of an ectatic artery on magnetic reso-
on presumed etiology, the cases were grouped into the nance imaging (MRI) may not be specic for HFS, as
following categories: (1) primary or idiopathic (attrib- it may be found in otherwise normal individuals,
uted to vascular compression of the ipsilateral facial and not all cases had the appropriate imaging
nerve), (2) hereditary (with strong family history of studies, such as MRA or arteriograms to further
HFS in rst-degree relatives),(3) secondary (due to an delineate the posterior and middle fossa vasculature.
identiable cause), and (4) HFS mimickers (psycho- Ninety patients were white, 24 Hispanic, 10 Asian, 7
genic, tics, dystonia, myoclonus, myokymia, myor- African American, and 5 Indian. Seven patients had
thythmia, and hemimasticatory spasm). We did not coexisting Parkinsons disease, 6 patients had associ-
include patients with orbicularis oculi myokymia, as ated migraine, 4 patients had essential tremor, 2 had
this is virtually always a benign condition. spasmodic dysphonia, and 2 patients had restless
References for this review were identied by legs syndrome. Only 1 subject had coexistent tri-
searches of PubMed from 1980 to January 2011, with geminal neuralgia, compared with 8 of 158 (5.1%)
the terms hemifacial spasm, tics, dystonia, myoclonus, in a previous study of patients with HFS.1 Ectopic
psychogenic, and hemimasticatory spasm. Articles excitation of the trigeminal nerve has been

TABLE 1. Characteristics of patients referred to Baylor College of Medicine for HFS


Classication n Sex (M/F) Age at onset (y) Duration (y)

1. Idiopathic 133 (62%) 48/85 55.4 6 15.0 10.9 6 7.6


2. Familial 4 (1.9%) 2/2 49.5 6 32.0 30.2 6 27.4
3. Bells palsy 23 (11%) 5/18 53.7 6 17.7 9.1 6 10.6 (after injury)
4. Facial nerve injury 13 (6%) 2/11 46.8 6 16.0 5.1 6 8.0 (after injury)
5. Brain vascular insult 2 (1%) 1/1 72 and 72 7 years and 4 months
6. Demyelinating 2 (1%) 1/1 55 and 64 2 months and 1.5 years
7. Psychogenic 16 (7.4%) 1/15 37.4 6 19.5 1.7 6 2.2
8. Facial tics 14 (6.5%) 10/4 33.9 6 22.2 13.8 6 10.1
9. Facial dystonia 5 (2.3%) 2/3 52.8 6 8.1 4 6 6.2
10. Facial myoclonus 2 (1%) 0/2 20 and 5 6 and 7
11. Hemimasticatory spasm 1 F 59 4

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TABLE 2. HFS categorized by etiology reported in the


Stroke Towgh et al141
literature
Arunabh et al142
Vascular abnormality Reference Kawakami et al143
Vermersch et al144
1. AV malformation Nagata et al91 Ambrosetto and Forlani145
Altinors et al92 Schiess et al146
2. AV stula Deshmukh et al93 Vascular headache
3. Fusiform aneurysm Choi et al94 1. Migraine Cuadrado et al147
Uchino et al95 Husid68
Tsuchiya et al96 2. Cluster headache Nakazato et al66
Moriuchi et al97
Infection
4. Venous angioma Chen et al98
5. External carotid artery compression Rakover et al99 1. Otitis media Razdan et al148
6. Vertebrobasilar dolichoectasia Papapetropoulos et al100 2. Neurocysticercosis Lavon et al149
Arantes et al101 3. Tuberculosis meningitis Sandyk150
Titlic et al102 Peripheral facial nerve injury
Deleu and Mesraoua103 Bells palsy Valls-Sole and Montero49
Rahman et al104 Valls-Sole151
7. Compression of contralateral Grigoryam et al105
vertebral artery
Mass lesion proposed as a mechanism of the combination of HFS
and trigeminal neuralgia, also referred to as tic
1. Parotid tumor Behbehani et al106
convulsive.19
Destee et al107
Nussbaum108
2. CP angle lipoma Barajas et al109 Hereditary (Case 2)
Sade et al110
Inoue et al111 Four patients (2%) had a positive family history of
Levin and Lee112 HFS based on historical information. One of these
3. Acoustic neuroma Nishi et al113 patients, a 74-year-old man, rst reported unilateral
4. Arachnoid cyst Mastronardi et al114 facial twitching at 5 years of age, although this could
Bonde et al115
Altinors et al92 not be conrmed. He also had orthostatic tremor and
5. Meningioma Han et al116 several rst-degree relatives with HFS. The clinical
Park et al117 presentation of HFS in these familial cases, except 1
Ferroli and Broggi118 with bilateral HFS, did not differ from patients with
Cancelli et al119 idiopathic HFS.
Iwai et al120
Bhayani and Goel121
Rhee et al122 Secondary HFS
Nagata et al91
6. Schwannoma Han et al116 Bells Palsy (Case 3)
Kudo et al123 Twenty-three patients (12%) were diagnosed with
7. Hemangioma Asaoka et al124
8. Glomus jugulare tumor Kiley et al125
HFS following Bells palsy. Mean age at onset was
9. Astrocytoma Mezer et al126 53.5 6 17.7 years, and mean interval from the onset
Sandberg and Souweidane127 of Bells palsy to development of facial spasm was
10. Ependymal cyst Han et al116 reported by patients as 9.1 6 10.6 years. This interval
Harada et al128 is not likely accurate, as patients often had difculty
11. Pontine glioma Elgamal and Coakham129
12. Epidermoid cyst Choid et al130
recalling the onset of the facial twitching in relation-
13. Cerebellar gangliocytoma Minkin et al131 ship to the onset of facial weakness. Of the 23
patients with Bells palsy, 7 were found to have synki-
Bony lesion or structural abnormality
netic movement associated with aberrant regeneration
1. Pagets disease Fu and Ko132 of the facial nerve.
Linazasoro et al133
Fernandez et al134
2. Marfans syndrome Braca et al1305 Facial Nerve Injury
Nagatani et al136
3. Chiari malformation Felicio et al137 Thirteen patients (6%) presented with HFS after
Felicio et al138 facial nerve injury. The injuries were caused by air-
Colpan and Sekerci139 bag deployment in 4 patients, physical assault in 3
Braca et al135 patients, and 1 each following a dental procedure,
4. Focal bone hyperostosis Mitsos et al140
surgical removal of left ear glomus tumor, rhino-
Demyelinating Nociti et al53
Telischi et al52 plasty, wisdom tooth extraction, resection of ear
squamous cell carcinoma, mastoid surgery, and left

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parotidectomy. Mean interval between facial nerve Facial Myoclonus (Case 7)


injury and onset of HFS was 5.1 6 8.0 years. Two patients (1%) were diagnosed with facial myo-
clonus secondary to encephalitis. One patient, previ-
Demyelinating Lesions ously reported,20 was a 26-year-old woman with
Two patients were diagnosed with HFS associated Rasmussen encephalitis who presented with facial
with demyelinating lesions. One patient was diagnosed myoclonus. The presentation in these 2 patients was
with HFS secondary to multiple sclerosis. The other different from the other HFS patients in that the facial
patient was a 55-year-old man with HIV who had twitching was more rhythmic and continuous.
demyelinating lesions in the brain stem on MRI
imaging. Hemimasticatory Spasm (Case 8)
One patient was diagnosed with idiopathic hemi-
Brain. Vascular Insults
masticatory spasm, dened as paroxysmal contractions
Two patients, both 72 years old, were diagnosed of 1 or more of the jaw-closing muscles (masseter,
with HFS presumed to be a result of brain stem vascu- temporalis, and medial and lateral pterygoids) on 1
lar insults. Both had a history of hypertension and side of the face.21 The patients symptoms were
diabetes. One patients symptoms started 1 month limited to involvement of the left masseter muscle,
after a transient ischemic attack, and the other patient and there was no associated facial hemiatrophy.
was found to have multiple lacunar infarcts in the
pons on MRI.
Discussion
HFS Mimickers
Although HFS is a common involuntary facial
Psychogenic (Case 4) movement disorder, the diagnosis of HFS can be dif-
Psychogenic causes of facial movements were con- cult. In 1 study, the positive diagnostic rate of HFS
sidered if at least 4 of the following characteristics after referral to a movement disorder clinic was only
were present: (1) acute onset; (2) inconsistent and 25.7%.22 HFS is frequently confused with other facial
incongruous features; (3) associated somatizations; movement disorders such as blepharospasm, oroman-
(4) reduction or abolishment of facial spasm with dibular dystonia, facial tic, hemimasticatory spasm,
distraction; (5) response to placebo, suggestion, or myokymia, and facial myorhythmia.16,17
psychotherapy; (6) spontaneous remission; and (7) We did not include myokymia in our series, as this
normal neuroimaging. Sixteen patients (7.5%) were transient twitching, typically involving the upper or
diagnosed with psychogenic HFS. Mean age at onset lower eyelid, is usually a benign condition, and most
was 37.4 6 19.5 years, and mean disease duration people with this involuntary movement do not seek
of symptoms was 1.7 6 2.2 years. Almost all medical attention. Myokymia refers to involuntary
patients were women (15 of 16, 93.8%). Seven contractions of small muscles or muscle fascicles that
patients had bilateral involvement. Most patients appears as undulating, rippling movements beneath
responded modestly to antidepressant medications or the skin.23 This differs from the tonic or tonicclonic
stress management techniques. muscle contractions seen with HFS. Furthermore, elec-
trophysiological studies typically show brief bursts of
Facial Tics (Case 5) single motor unit potentials ring at rates of 5150
Hz. The bursts may appear as doublets, triplets, or
Fourteen patients (7%) referred to our clinic for
multiplets on EMG, followed by short periods of elec-
HFS were diagnosed with facial tics. Mean age at
trical silence. Myokymia may last for hours to years
onset was 33.9 6 22.2 years, and mean duration was
before it evolves into some other neurological disorder
13.8 6 10.1. Twelve of the 14 patients had bilateral
or spontaneously resolves. Deafferentiation and hyper-
facial involvement, and the facial tics in 10 patients
excitability of the facial nerve nucleus or a lesion in
were markedly asymmetrical, resembling HFS. The
the intramedullary nerve bers are implicated as the
tics were conned to the face in 4 patients, whereas
cause of facial myokymia.24 Facial myokymia is often
the others had additional motor or phonic tics.
associated with multiple sclerosis, GuillainBarre syn-
drome, episodic ataxia type 1, spinocerebellar atrophy
Facial Dystonia (Case 6) type 3, and brain stem lesions, particularly pontine
Five patients (2%) had facial dystonia that mim- gliomas, but in most instances, it is a benign condi-
icked HFS. Etiologies of the focal dystonia included tion, possibly associated with excessive fatigue, sleep
peripherally induced facial dystonia after parotid sur- deprivation, or caffeine consumption.
gery, facial injury after a motor vehicle collision, corti- Although we did not encounter patients with facial
cal gliosis, and idiopathic. myorhythmia in our series collected over a decade, we

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have seen patients in the past with this movement study, NVC was demonstrated on the contralateral
disorder, all with Whipples disease and all initially mis- side in 15% of HFS patients.38 The mean age and du-
diagnosed as HFS. Myorhythmia is a coarse, synchronous ration of symptoms were similar in HFS patients with
or asynchronous, relatively slow, 13 Hz, rhythmical, and without contralateral NVC. Various causes for
tremor-like involuntary movement that usually occurs in facial nerve compression are listed in Table 2.
the face but may also involve other body parts.25 The largest group in our series, consisting of 133
Although oculomasticatory myorhythmia is highly char- patients (62%), had idiopathic HFS without an identi-
acteristic of Whipples disease, isolated facial myorhyth- able etiology. Although in 6 cases imaging studies
mia may rarely occur with other conditions such as a- suggested the possibility of an enlarged ectatic artery,
interferon 2a therapy used in the treatment of leukemia26; most patients did not show compression of the seventh
it may also be seen in brain vascular infarcts.27 cranial nerve on the MRI images, and therefore a
HFS usually occurs without an identiable etiology, vascular etiology could not be conrmed.
but it has been most frequently attributed to compres-
sion of the facial nerve at the root exit zone by an Genetic
ectopic anatomical or pathological structure resulting Several families with HFS have been reported, sug-
in ephaptic transmission.28 Three-dimensional volu- gesting a genetic etiology or predisposition in some
metric analysis has shown that patients with HFS have cases.3946 There appears to be an autosomal dominant
a lower posterior fossa volume when compared with pattern of inheritance in these families with low pene-
matched controls, which may contribute to the higher trance, and except for a younger age at onset, the clini-
rate of vascular compression of the facial nerve.29 cal features overlap with the idiopathic cases.41
This study, however, was performed in an Asian pop- Evaluation of single-nucleotide polymorphisms in genes
ulation, which has been found to have a higher fre- related to vascular change causing vascular compression
quency of HFS than a white population.15 In another did not show an association with HFS.47 Clarifying the
study of 132 patients with HFS, we found that 7 (8%) role of genetic susceptibility in HFS may help to better
were of Asian origin, in contrast with only 3 of 204 understand the pathogenesis of this disease.
patients (1.5%) with oromandibular dystonia eval-
uated in our clinic over the same period (2000
HFS Following Bells Palsy and
2010).30 The frequency of Asians with HFS was 3.1
Facial Nerve Injury
times higher than among those with cranial-cervical
dystonia. This nding further supports previous In our series, 11% had HFS after Bells palsy, and
reports that HFS is more frequent in the Asian popula- 6% of all patients had HFS secondary to facial nerve
tion,15,31 although a well-designed epidemiological injury (other than NVC). These patients did not differ
study is warranted to conrm this observation. from idiopathic HFS patients in age at onset and
Here we further characterize HFS according the clinical presentation (initially involved the orbicularis
underlying etiology and discuss HFS mimickers. oculi and then spread to involve the zygomaticus or
orbicularis oris muscles).
Synkinetic movement, often misdiagnosed as HFS,
Idiopathic HFS with or Without Compression associated with aberrant regeneration following facial
of Seventh Cranial Nerve nerve palsy is an example of a peripherally induced
The most commonly accepted theory of HFS is a movement disorder.48 Unintended muscle contractions
vascular abnormality in the posterior fossa compress- involving the adjacent ipsilateral or even contralateral
ing the facial nerve at the root exit zone. This is sup- muscles can occur as a result of synkinesis in patients
ported by reports of posterior fossa surgical with peripheral facial palsy, often triggered by auto-
explorations in HFS patients.32,33 In a study of 115 matic or emotional facial movements, mimicking HFS.
patients undergoing microvascular decompression sur- EMG may be used to detect synkinetic muscle
gery, the anterior inferior cerebellar artery was most contractions that may not be appreciated by simple ex-
commonly involved (43%), followed by the posterior amination.49 Synkinesia, however, is not unique in
inferior cerebellar artery (31%), and vertebral artery patients with HFS after Bells palsy or other facial nerve
(23%).34 Multiple compressing vessels were found in injury, as it can be also found in patients with otherwise
38% of cases. Using magnetic resonance angiography typical HFS. In 1 report, 43% patients with primary
(MRA) with MRI increases the recognition of the HFS and 58% patients with secondary HFS had evi-
neurovascular compression (NVC) seen in HFS.3538 dence of synkinesis.50 Another study compared blink
Although MRI and MRA have been used to investi- reexes and lateral spread in patients with synkinesis af-
gate patients with HFS, the identied NVC may not ter Bells palsy, and in HFS there was no difference in
always be pathogenic, and such a nding has also latencies of early and late responses.51 The most obvious
been also detected on the asymptomatic side and in difference between patients with typical HFS and those
control subjects.37 In a clinicoradiologic correlation with HFS after Bells palsy was the presence of irregular,

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often very frequent complex and prolonged spontaneous manifestations of HFS with or without NVC. In
discharges in the HFS patients. Synkinesis in HFS patients with HFS, the elicitation of a larger R2
patients was often accompanied or followed by episodes response on the affected side may represent enhanced
of automatic facial movements, whereas synkinesis in motoneuronal excitability.63 Misawa et al showed that
Bells palsy patients occurred predominantly following the abnormal muscle response of the blink reexes fre-
voluntary contraction of the facial muscles. quently present in patients with HFS are likely to be
mediated by trigeminal afferent inputs rather than an-
Demyelination tidromic activation of the facial nerve.64 Wilkinson et
HFS has been well documented in patients with al monitored the facial muscle motor evoked poten-
multiple sclerosis. In 2 patients the plaques were tials during microvascular decompression for HFS and
found in the area of the facial nucleus on the involved showed shorter latency and decreased amplitude of
side, supporting a central (brain stem) origin of the motor evoked potentials following the microvascu-
HFS.52 In another series, 5 of 733 patients had HFS lar decompression and the abolition of the lateral
associated with multiple sclerosis.53 Although no spread response.65
demyelinating lesions were noted in the brain stem, Hyperactive facial motor nucleus has been suggested
the authors concluded that HFS was caused by as a possible mechanism of HFS associated with vas-
multiple sclerosis, as these patients improved with cular migraine.66 Cluster headache is postulated to be
high-dose intravenous methylprednisolone treatment. associated with an abnormal interaction between
trigeminal afferents and cranial parasympathetic effer-
ents. Trigeminal afferent input alters the excitability
Brain Stem Tumors
of facial motoneurons in HFS.6769 In our series, 6
Although typical HFS is manifested by clonic, patients had associated migraine, and 1 patient had
twitching movements involving 1 side of the face, trigeminal neuralgia, possibly indicative of increased
rarely patients present with more sustained contrac- excitability of brain stem nuclei.
tions. The presence of these persistent tonic facial
spasms usually indicates underlying brain stem pathol-
ogy, such as tumors in the cerebellopontine angle or HFS Mimickers
pontine gliomas.54
Psychogenic
Other Mechanisms of Hemifacial Spasm Psychogenic HFS is one of the most common pre-
sentations of psychogenic movement disorders, and
Hypertension and Ventrolateral Medulla
such facial movements may lead to unnecessary treat-
Compression
ment, including decompressive surgery.70 Psychogenic
An association between arterial hypertension, HFS is characterized by nonpatterned facial move-
benign intracranial hypertension, and HFS has been ments that frequently vary in intensity and are dis-
reported.55 Arterial hypertension was present in all tractible. In a previous report from our Movement
symptomatic subjects with late-onset HFS in a family Disorders Clinic, psychogenic facial spasm was
with HFS spanning 4 generations.46 In a casecontrol diagnosed in 2.4% of patients evaluated for HFS, with
study, Defazio et al found that arterial hypertension a mean age of 34.6 years, which was considerably
occurred more frequently among patients with HFS.56 younger than that of patients with organic HFS, most
Oliveira et al also reported that arterial hypertension of whom usually present after age 40.71 In the present
was signicantly more common in HFS than in study, 7.4% of our patients referred for evaluation for
blepharospasm.57 However, other studies did not HFS were diagnosed with psychogenic facial spasm.
nd this association between hypertension and HFS.5860 The mean age at onset was 37.4 6 19.5 years, and
Neuroimaging studies have demonstrated that ventro- similar to previous studies, women accounted for most
lateral medullary compression is more common in hyper- of those cases (15 of 16).
tensive HFS patients and hypertensive controls
compared with normal controls.61 Jannetta et al
described patients with HFS and hypertension who had Facial Tics
additional neurovascular compression of the left ventro- Facial tics are abrupt-onset, brief, unsustained facial
lateral medulla (VLM) at the root-entry zone of cranial movements that often occur in response to a premoni-
nerves IX and X.62 Microvascular decompression of the
tory sensation or an urge to perform the movement.
left VLM normalized or improved their blood pressure. Some patients perceive motor tics as a voluntary move-
ment in response to an involuntary sensation.72 Blinking
Hyperactive Facial Motor Nucleus and facial twitching were the most common clonic tics
Hyperexcitability of facial motor neurons has been seen in 1 series.73 Facial tics can be confused with HFS if
suggested to contribute to clinico-electrophysiological they occur in isolation, but many patients describe a

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premonitory sensation prior to the tics, and in contrast spasm and a prolonged postexcitatory inhibition before
to HFS, tics are often suppressible.74 returning to the normal value.81 A lesion with abnormal
In our series, facial tics were found to be part of EEG ndings in the left frontotemporal region indicated
multiple motor tics (64%) or the only tic present a frontal origin. Right inferior frontal epileptiform dis-
when a patient rst sought medical evaluation (36%). charges were noted in a patient with HFS and epilepsia
Facial tics are easier to distinguish from HFS when partialis continua.82
they occur in combination with other motor or phonic
tics. A detailed history of motor and phonic tics or Oromandibular Dystonia
other comorbid disorders such as obsessive-compulsive
disorder (OCD) and attention decit disorder (ADD) Oromandibular dystonia (OMD) consists of sus-
should be obtained. tained involuntary, repetitive, patterned muscle con-
tractions affecting the lower part of the face, mouth,
jaw, tongue, and pharynx.1,83,84 It can be associated
Facial Myoclonus with difculties in speaking, chewing, swallowing,
Facial myoclonus such as that manifested by clonic and even breathing. OMD is a focal dystonia that can
facial contractions during focal motor seizures can be classied as idiopathic, primary, or secondary. Sec-
mimic HFS.75 The cases presented here share some ondary causes include tardive dystonia, trauma, oro-
common features with HFS with constant contractions mandibular surgery, and dental procedures.85 OMD
of the orbicularis oculi and zygomaticus muscle on the may be associated with temporomandibular joint dis-
left side of face, exacerbated by speech or smile. orders or bruxism.86 Occasionally, OMD may be
A case of isolated unilateral facial myoclonus induced asymmetrical or even unilateral, thus mimicking HFS.
exclusively and specically by speaking and writing,
indicating a possible origin in the left Rolandic oper- Hemimasticatory Spasm
cular cortex, has been described.76 Back-averaging of
right facial EMG bursts failed to show a reliable elec- Hemimasticatory spasm (HMS) is a rare condition
troencephalogram (EEG)electromyogram (EMG) cor- rst described in 1897 by Gowers.87 It is characterized
relation. Bonanni and Guerrini reported a case of by involuntary contractions of muscles innervated by
Moebius syndrome presenting with rhythmic rising of the trigeminal nerve. HMS is usually painless and may
the upper lip and eyebrow.77 Burst-locked EEG aver- be associated with hemifacial atrophy. Usually idio-
aging again failed to show any consistent EEG activity pathic, HMS has been associated with biopercular
preceding the EMG bursts. However, blink reex and syndrome and pontine infarctions.88,89 HMS may
somatosensory and motor evoked potentials showed share a common pathophysiology with HFS.19 It has
ndings consistent with pontine pathology. Canevini been attributed in 1 case to compression of the distal
et al reported a case of facial myoclonic jerks triggered mandibular nerve branch.90 EMG recordings demon-
by speaking and listening to spoken language, attrib- strate irregular motor unit activity lasting seconds to
uted to a left frontal lesion.78 EEG recording showed minutes with loss of the silent periods during episodes
paroxysms of spikes and polyspikes followed by a of involuntary spasm.
slow wave, more evident in the left frontotemporal
region. Lou et al79 studied 4 patients with familial oli- Conclusions
vopontocerebellar atrophy who had abnormal twitch-
ing of cheeks and perioral muscles induced by facial HFS or a phenomenologically similar movement dis-
movements.79 EMG recording showed synchronous order can be associated not only with microvascular
discharges in the orbicularis oris and risorius muscles compression along the course of the facial nerve, but
associated with visible twitching. Munoz et al reported also with lesions involving the brain stem, subcortical
a case of jerking movements of the head and clonic areas, and even cortex. Unilateral facial contractions
involuntary twitches involving the eyelid and cheek.80 can also be the manifestation of other movement disor-
MRI showed a dolichoectatic left vertebral artery dis- ders usually considered to be of central origin and pos-
placing the medulla. Direct compression the eleventh sibly involving the basal ganglia. Clinicians should be
and seventh cranial nerves by the dolichoextatic left familiar with the phenomenology of typical and atypi-
vertebral artery was postulated as the mechanism cal HFS and search for clues suggestive of other facial
responsible for the myoclonic movements. movement disorders such as tics, dystonia, myoclonus,
Cortical myoclonus mimicking HFS, as seen in our hemimasticatory spasm, psychogenic HFS, and other
case 7 with underlying encephalitis and in another case causes of facial movements. Most cases of HFS res-
of Rasmussen encephalitis,20 has been investigated in a pond well to repeated botulinum toxin injections.
few cases. Kotterba et al demonstrated increasingly Early diagnosis facilitates appropriate medical therapy
shortened postexcitatory inhibition after transcranial and prevents unnecessary treatment or invasive
magnetic stimulation in 8 patients with HFS during procedures.

1588 Movement Disorders, Vol. 26, No. 9, 2011


D I F F E R E N T I A L D I A G N O S I S O F H E M I F A C I A L S P A S M

Legends to the Video contraction of the left masseter muscle was noted
on surface EMG recording. Segment 2: This video
VideoCase 1. This video shows a 32-year-old man shows the patient with complete resolution of hemi-
with a right hemifacial spasm with involvement of the masticatory spasm after chemodenervation with
right orbicularis oculi, orbicularis oris, and zygomati- onabotulinumtoxinA.
cus muscles. He also demonstrates a classic other
Babinski sign on the right.
VideoCase 2. This video shows an 81-year-old
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