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Hemifacial Spasm
Updated: Sep 16, 2019
Author: Steven Gulevich, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE

Overview

Background
First described by Gowers in 1884, hemifacial spasm represents a segmental myoclonus of muscles innervated by the
facial nerve. Hemifacial spasm presents in the fifth or sixth decade of life, almost always unilaterally, although bilateral
involvement may occur rarely in severe cases. Hemifacial spasm generally begins with brief clonic movements of the
orbicularis oculi and spreads over years to other facial muscles (corrugator, frontalis, orbicularis oris, platysma,
zygomaticus).[1, 2]

Clonic movements progress to sustained tonic contractions of involved musculature. Chronic irritation of the facial
nerve or nucleus, the near-universal cause of hemifacial spasm, may arise from numerous underlying conditions.

Facial musculature is subject to the same movement disorders as muscles of the limbs or trunk. Myoclonus, dystonia,
and other movement disorders present with specific syndromes in the facial musculature. An understanding of the
underlying mechanism leads to appropriate diagnostic evaluation and potential treatment.

The causes of hemifacial spasm include vascular compression, facial nerve compression by a mass, brainstem lesions
such as stroke or multiple sclerosis plaques, and secondary causes such as trauma or Bell's palsy.[3]

Although specific treatments are available for many craniofacial movement disorders, botulinum toxin (BTX)
chemodenervation has proven useful in many of these disorders, supplanting surgery and medical therapy.

For patient education information, see the Procedures Center, as well as BOTOX® Injections.

Pathophysiology
Irritation of the facial nerve nucleus is believed to lead to hyperexcitability of the facial nerve nucleus, while irritation of
the proximal nerve segment may cause ephaptic transmission within the facial nerve. Either mechanism explains the
rhythmic involuntary myoclonic contractions observed in hemifacial spasm.[4]

Compressive lesions (eg, tumor, arteriovenous malformation, Paget disease) and noncompressive lesions (eg, stroke,
multiple sclerosis plaque, basilar meningitis) may present as hemifacial spasm. Most instances of hemifacial spasm
previously thought to be idiopathic were probably caused by aberrant blood vessels (eg, distal branches of the anterior
inferior cerebellar artery or vertebral artery) compressing the facial nerve within the cerebellopontine angle.

Epidemiology
Hemifacial spasm affects all races equally. There is a slight female preponderance. Idiopathic hemifacial spasm
typically begins in the fifth or sixth decade of life. Onset of hemifacial spasm in patients younger than 40 years is
unusual and often heralds an underlying neurologic illness (eg, multiple sclerosis).
Prognosis
Hemifacial spasm rarely remits spontaneously. Current treatments, fortunately, prove highly effective. Patients should
be counseled, however, that treatments are likely to continue indefinitely.

Presentation

Physical Examination
Involuntary facial movement is the only symptom of hemifacial spasm. Fatigue, anxiety, or reading may precipitate the
movements. Spontaneous hemifacial spasm manifests with facial spasms that represent myoclonic jerks and are
analogous to segmental myoclonus, which may affect other body regions. Postparalytic hemifacial spasm, following
facial nerve trauma such as Bell's palsy, manifests as facial synkinesis and contracture.

Physical findings are restricted to the involved facial muscles. The examiner will observe these contracting
synkinetically (all at the same time), usually with an irregular frequency. Other neurological findings signal another
process elsewhere, or an underlying process such as cerebrovascular disease or multiple sclerosis.

Complications
Prolonged contraction of the orbicularis oculus will result in temporary loss of vision in the involved eye, with
impairment of activity.

DDx

Diagnostic Considerations

Hemimasticatory spasm
Hemimasticatory spasm is analogous to hemifacial spasm and occurs with irritation to the motor trigeminal nerve. This
rare condition is a segmental myoclonus and presents with unilateral involuntary contractions of the trigeminally
innervated muscles of mastication (usually the masseter). Similar to hemifacial spasm, hemimasticatory spasm
responds to treatment with medications and botulinum toxin. However, less evidence exists that exploratory surgery
benefits patients with this condition.[5]

Myoclonic movements
Myoclonic movements affecting facial musculature also may arise from lesions at the brain or brainstem level. These
are distinguished from hemifacial spasm by the distribution of abnormal movements (more generalized, possibly
bilateral) and possibly by electrodiagnostic evaluation. Imaging studies may yield an underlying cause. Central
myoclonus responds to anticonvulsant management.

Oromandibular dystonia
Oromandibular dystonia refers to dystonia affecting the lower facial musculature, predominantly the jaw, pharynx, and
tongue. When oromandibular dystonia occurs in conjunction with blepharospasm, the disorder is termed Meige
syndrome.

Jaw-opening forms of oromandibular dystonia indicate primary involvement of the digastric and lateral pterygoid. Jaw-
closing oromandibular dystonia involves the masseter, temporalis, and medial pterygoid. Jaw deviation, indicating
predominant involvement of the lateral pterygoid, is rare.

Botulinum toxin is the preferred treatment for oromandibular dystonia and is most effective in the jaw-closure type.
Medications seldom yield acceptable results. When medications must be used, employ the same agents as for
blepharospasm.[6] Because of the risk of aspiration, never inject botulinum toxin into the tongue.

Craniofacial tremor
Craniofacial tremor may occur in association with essential tremor, Parkinson disease, thyroid dysfunction, or
electrolyte disturbance. It occurs rarely in isolation. Focal motor seizures must occasionally be distinguished from other
facial movement disorders, particularly hemifacial spasm. Postictal weakness and greater involvement of the lower
face are distinguishing features of focal motor seizures.

Facial chorea
Facial chorea occurs in the context of a systemic movement disorder (eg, Huntington disease, Sydenham chorea).
Chorea is a random, flowing, nonpatterned set of movements. A related disorder, spontaneous orofacial dyskinesia of
the elderly, is observed primarily in the edentulous. It usually responds to proper fitting of dentures.

Tics
Facial tics are brief, repetitive, coordinated, semipurposeful movements of grouped facial and neck muscles. Tics may
occur physiologically or in association with diffuse encephalopathy. Some medications (ie, anticonvulsants, caffeine,
methylphenidate, antiparkinsonian agents) are associated with producing tics. Single, repetitive, stereotyped
movements (eg, repetitive grimacing, throat clearing, vocalizations) define a simple tic disorder.

Facial myokymia
Facial myokymia appears as vermicular twitching under the skin, often with a wavelike spread. This is distinguished
from other abnormal facial movements by characteristic electromyogram discharges presenting as brief, repetitive
bursts of motor unit potentials firing at 2-60 Hz interrupted by periods of silence of up to a few seconds. Facial
myokymia may occur with any brainstem process. Severe cases may benefit from botulinum toxin. Most cases are
idiopathic and resolve without treatment over several weeks.

Tardive dyskinesia
Tardive dyskinesia appears as bilateral, irregular, and non-synkinetic muscular contractions. It occurs in the setting of
medication use, usually antipsychotics.

Differential Diagnoses
Huntington Disease

Myokymia

Tardive Dyskinesia

Workup

Workup
Approach Considerations
Early cases of hemifacial spasm may be difficult to distinguish from facial myokymia, tics, or myoclonus originating in
the cortex or brainstem. Neurophysiologic testing can be invaluable.

Spread and variable synkinesis on blink reflex testing and high-frequency discharges on electromyography (EMG) with
appropriate clinical findings are diagnostic. Stimulation of one branch of the facial nerve may spread and elicit a
response in a muscle supplied by a different branch. Synkinesis is not present in essential blepharospasm, dystonia,
or seizures. Needle EMG shows irregular, brief, high-frequency bursts (150–400 Hz) of motor unit potentials, which
correlate with clinically observed facial movements.

Imaging and Other Studies

Magnetic resonance imaging is the imaging study of choice, especially if an underlying compressive lesion is
suspected. Cerebral angiography offers little diagnostic value in hemifacial spasm. Ectatic blood vessels rarely are
identified, and it is difficult to correlate vessels with the facial nerve. Perform angiography and/or magnetic resonance
angiography prior to a vascular decompression surgical procedure. Angiography is often performed before
decompressive surgery to clarify the vascular anatomy, because it may identify an aneurysm or vascular anomaly.[7]

Laboratory Studies
There are no known biological markers for hemifacial spasm.

Treatment

Approach Considerations
In most patients with hemifacial spasm, the treatment of choice is injection of botulinum toxin under electromyographic
(EMG) guidance. Chemodenervation safely and effectively treats most patients, especially those with sustained
contractions. Relief of spasms occurs 3-5 days after injection and lasts approximately 6 months.

Medications used in the treatment of hemifacial spasm include carbamazepine and benzodiazepines for
noncompressive lesions. Carbamazepine, benzodiazepines, and baclofen also may be used in patients who refuse
botulinum toxin injections. Compressive lesions need to be treated surgically. Microvascular decompression surgery
may be effective for those patients who do not respond to botulinum toxin. A study of 246 patients who underwent
microvascular decompression surgery found no significant difference in outcomes and complications between patients
who had botulinum toxin injections prior to their first surgery and those who did not.[8]

Botulinum Toxin Injection

The treatment of choice for hemifacial spasm is botulinum toxin injection of botulinum toxin under EMG guidance. Side
effects of botulinum toxin injection (eg, facial asymmetry, ptosis, facial weakness) usually are transient. Most patients
report a highly satisfactory response. Caution patients that although botulinum toxin ablates the muscular spasm, the
sensation of spasm often persists.[9, 10]
Pharmacologic Therapy
Medications may be used in early hemifacial spasm (when spasms are mild and infrequent) or in patients who decline
botulinum toxin injection. Use medications in patients with noncompressive lesions and early idiopathic hemifacial
spasm. Response to medication varies but can be satisfactory in early or mild cases. The most helpful agents are
carbamazepine and benzodiazepines (eg, clonazepam). Often, medication effects attenuate over time, necessitating
more aggressive treatment.

Surgical Decompression
Treat compressive lesions surgically. Ectatic blood vessels cause hemifacial spasm by compressing the facial nerve as
it exits the brainstem. Surgical decompression of these blood vessels can yield excellent results.[11, 12, 13, 14] A
study evaluating the effect of microvascular decompression surgery on idiopathic hemifacial spasm with compression
on different zones of facial nerve found that proper detection of offending vessels and complete decompression may
increase cure rate.[15]

Patients with apparently idiopathic hemifacial spasm may benefit from posterior fossa exploration and microvascular
decompression. Myectomy rarely is required.

Medication

Medication Summary
The goal of pharmacotherapy is reduction of abnormal muscle contractions. Botulinum toxin type A is the treatment of
choice.[16, 17] Carbamazepine, benzodiazepines, and baclofen may also be used in patients who refuse BTX
injections or who are not surgical candidates.

Neuromuscular Blockers, Botulinum Toxins

Class Summary
Botulinum toxin type A is the drug of choice.[16, 17] It causes presynaptic paralysis of the myoneural junction and
reduces abnormal contractions. Therapeutic effects may last 3-6 months.

Botulinum toxin type B is useful in reducing excessive, abnormal contractions associated with blepharospasm[18] ;
binds to receptor sites on the motor nerve terminals and after uptake inhibits release of acetylcholine, blocking
transmission of impulses in neuromuscular tissue; 7-14 d after administering initial dose, assess patients for a
satisfactory response; increase doses 2-fold over previously administered dose for patients who experience
incomplete paralysis of the target muscle.

OnabotulinumtoxinA (BOTOX)
OnabotulinumtoxinA (BOTOX) is useful in reducing excessive, abnormal contractions associated with blepharospasm.
It binds to receptor sites on the motor nerve terminals and, after uptake, inhibits the release of acetylcholine, blocking
transmission of impulses in neuromuscular tissue. At 7-14 days after administration of the initial dose, assess patients
for a satisfactory response. Increase the dose 2-fold over the previously administered dose in patients who experience
incomplete paralysis of the target muscle.
RimabotulinumtoxinB (Myobloc)
When botulinum toxin injection is indicated and type A toxin is ineffective, injection with type B toxin
(rimabotulinumtoxinB [Myobloc]) should be considered.

AbobotulinumtoxinA (Dysport)
AbobotulinumtoxinA (Dysport) binds to receptor sites on the motor nerve terminals and, after uptake, inhibits release of
acetylcholine, blocking transmission of impulses in neuromuscular tissue. At 7-14 days after administration of the initial
dose, assess the patient for a satisfactory response. Increase the dose 2-fold over the previously administered dose in
patients who experience incomplete paralysis of the target muscle.

IncobotulinumtoxinA (Xeomin)
Xeomin, a botulinum toxin type A product, may be used if Botox proves unsuccessful, or is unavailable. It should
produce satisfactory results, though systematic trials of Xeomin for HFS have not yet been reported. Excess
administration will produce undesirable weakness and facial asymmetry.

Benzodiazepines

Class Summary
Benzodiazepines may potentiate the effects of gamma-aminobutyric acid (GABA) and facilitate inhibitory GABA
neurotransmission. It may act in the spinal cord to induce muscle relaxation. Treatment needs to be individualized for
each patient.

Clonazepam (Klonopin)
Clonazepam (Klonopin) is useful in suppressing muscle contractions by facilitating inhibitory GABA neurotransmission
and other inhibitory transmitters.

Skeletal Muscle Relaxants

Class Summary
Muscle relaxants may inhibit the transmission of monosynaptic and polysynaptic reflexes at the spinal cord level.

Baclofen (Lioresal, Gablofen)

Baclofen (Lioresal) may induce hyperpolarization of afferent terminals and inhibit both monosynaptic and polysynaptic
reflexes at the spinal level.

Anticonvulsants

Class Summary
Anticonvulsants are used to manage severe muscle spasms and provide analgesia and mild sedation. Anticonvulsants
are probably the best medications in terms of efficacy and long-term safety when botulinum toxin and surgery are not
options.

Carbamazepine (Tegretol, Equetro, Epitol, Carbatrol)

Carbamazepine (Tegretol) is effective in the treatment of hemifacial spasm and complex partial seizures. It appears to
act by reducing polysynaptic responses and blocking posttetanic potentiation. Once a response is attained, attempt to
reduce the dose to the minimum effective level or discontinue the drug at least once every 3 months. In patients who
cannot tolerate carbamazepine, consider oxcarbazepine (dosage not yet established).

Oxcarbazepine (Trileptal, Oxtellar XR)

Oxcarbazepine (Trileptal) is effective in partial complex epilepsy. It shows promise in hemifacial spasm. Oxcarbazepine
may be considered when first-line agents (eg, botulinum toxin, carbamazepine) have failed or are contraindicated.

Questions & Answers

Overview

What is hemifacial spasm?

What causes hemifacial spasm?

What is the preferred treatment for hemifacial spasm?

What is the pathophysiology of hemifacial spasm?

Which patient groups have the highest prevalence of hemifacial spasm?

What is the prognosis of hemifacial spasm?

Presentation

Which are the signs and symptoms of hemifacial spasm?

Which physical findings are characteristic of hemifacial spasm?

What are the possible complications of hemifacial spasm?

DDX

How is facial myokymia differentiated from hemifacial spasm?

How is hemimasticatory spasm differentiated from hemifacial spasm?

How are myoclonic movements differentiated from hemifacial spasm?

How is oromandibular dystonia differentiated from hemifacial spasm?

How is craniofacial tremor differentiated from hemifacial spasm?

How is facial chorea differentiated from hemifacial spasm?

How are tics differentiated from hemifacial spasm?

How is tardive dyskinesia differentiated from hemifacial spasm?

What are the differential diagnoses for Hemifacial Spasm?

Workup

How is hemifacial spasm diagnosed?

What is the role of imaging studies in the workup of hemifacial spasm?

What is the role of lab tests in the workup of hemifacial spasm?

Treatment

How is hemifacial spasm treated?

What is the role of botulinum toxin injection in the treatment of hemifacial spasm?

What is the role of medications in the treatment of hemifacial spasm?

What is the role of surgery in the treatment of hemifacial spasm?

Medications

Which medications are used in the treatment of hemifacial spasm?

Which medications in the drug class Anticonvulsants are used in the treatment of Hemifacial Spasm?

Which medications in the drug class Skeletal Muscle Relaxants are used in the treatment of Hemifacial Spasm?

Which medications in the drug class Benzodiazepines are used in the treatment of Hemifacial Spasm?

Which medications in the drug class Neuromuscular Blockers, Botulinum Toxins are used in the treatment of
Hemifacial Spasm?

Contributor Information and Disclosures

Author

Steven Gulevich, MD Centennial Medical Center, Colorado

Steven Gulevich, MD is a member of the following medical societies: American Academy of Neurology, Colorado
Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of
Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Nicholas Lorenzo, MD, MHA, CPE Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine
Health, Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview;
Founder and CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc; Chief Strategy Officer, Discourse LLC

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American
Academy of Neurology, American Association for Physician Leadership

Disclosure: Nothing to disclose.

Acknowledgements

Stephen A Berman, MD, PhD, MBA Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American
Academy of Neurology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Glenn Lopate, MD Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington
University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of
Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American
Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of
Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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