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Bell Palsy

PAGE 123

Bell Palsy

http://www.emedicine.com/emerg/topic56.htm

Last Updated: November 28, 2005

Synonyms and related keywords: Bell's palsy, facial nerve paralysis, facial paralysis, idiopathic facial paralysis, unilateral facial paralysis, cranial nerve VII paralysis, seventh cranial nerve paralysis, neurologic disorder, paralysis on one side of face, weakness on one side of face, drooling, tearing from eyes, upper respiratory infection, URI, viral infection, herpes simplex virus, HSV, Bell palsy

AUTHOR INFORMATION Section 1 of 10

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Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Pictures Bibliography

Author: Michael Lambert, MD, Fellowship Director of Emergency Ultrasound, Clinical Assistant Professor, Department of Emergency Medicine, Resurrection Medical Center

Michael Lambert, MD, is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Institute of Ultrasound in Medicine, and Society for Academic Emergency Medicine

Editor(s): Edward Bessman, MD, Chairman, Department of Emergency Medicine, John Hopkins Bayview Medical Center; Assistant Professor, Department of Emergency Medicine, Johns Hopkins University; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; J Stephen Huff, MD, Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia Health System; John Halamka, MD, Chief Information Officer, CareGroup Healthcare System, Assistant Professor of Medicine, Department of Emergency Medicine, Beth Israel Deaconess Medical Center; Assistant Professor of Medicine, Harvard Medical School; and Jonathan Adler, MD, Attending Physician, Department of Emergency Medicine, Massachusetts General Hospital; Division of Emergency Medicine, Harvard Medical School

INTRODUCTION Section 2 of 10

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Background: Bell palsy is one of the most common neurologic disorders affecting the cranial nerves. It is an abrupt, unilateral, peripheral facial paresis or paralysis without a detectable cause. This syndrome of idiopathic facial paralysis was first described more than a century ago by Sir Charles Bell, yet much controversy still surrounds its etiology and management. Bell palsy is certainly the most common cause of facial paralysis worldwide.

Keeping in mind that Bell palsy is a diagnosis of exclusion is imperative. Other disease states or conditions that present with facial palsies are often misdiagnosed as idiopathic.

Patients with Bell palsy frequently present to the ED before seeing any other health care professional. The appearance of a distorted face and the abrupt functional impairment are the driving forces that prompt emergency evaluation. Patients often fear they have had a stroke or have a tumor and that their distorted facial appearance will be permanent.

The emergency physician's role consists of the following:

Exclude other causes of facial paralysis.

Initiate appropriate treatment.

Protect the eye.

Arrange appropriate medical follow-up care.

Pathophysiology: Actual pathophysiology is unknown; this is an area of interminable debate. A popular theory champions inflammation of the facial nerve. During this process, the nerve increases in diameter and becomes compressed as it courses through the temporal bone.

The facial nerve courses through a portion of the temporal bone commonly referred to as the facial canal. The first portion of the facial canal (the labyrinthine segment) is narrowest. The tiny opening (about 0.66 mm in diameter) in this segment is known as the meatal foramen.

The facial nerve is subjected to tight confines on its journey through the facial canal. It seems logical that various inflammatory, demyelinating, ischemic, or compressive processes may impair neural conduction at this unique anatomic site.

Anatomy

The facial nerve (seventh cranial nerve) has 2 components. The larger portion comprises efferent fibers that stimulate the muscles of facial expression. The smaller portion contains taste fibers to the anterior two thirds of the tongue, secretomotor fibers to the lacrimal and salivary glands, and some pain fibers.

Pathway

The path of the facial nerve is very complex; this may be the reason the nerve is vulnerable to injury. Two portions of the facial nerve leave the brain at the cerebellopontine angle, traverse the posterior cranial fossa, dive into the internal acoustic meatus, pass through the facial canal in the temporal bone, then angle sharply backwards, where they pass behind the middle ear and exit the cranium at the stylomastoid foramen. From here, the facial nerve bisects the parotid gland, and then terminal branches burst out from the parotid plexus to supply the muscles of facial expression.

Frequency: In the US: The incidence of Bell palsy in the United States is approximately 23 cases per 100,000 persons. The condition affects approximately 1 person in 65 in a lifetime.

Internationally: The incidence is the same as in the United States.

Mortality/Morbidity: Bell palsy can cause aesthetic, functional, and psychological disturbances in patients who have residual nerve dysfunction during their recovery phase or in patients with incomplete healing.

Partial paralysis

Motor synkinesis (involuntary movement accompanying a voluntary movement)

Autonomic synkinesis (involuntary lacrimation after a voluntary muscle movement)

Race: Incidence of Bell palsy appears to be slightly higher in persons of Japanese descent.

Sex: No difference exists in sex distribution in patients with Bell palsy.

Age: Age affects the probability of contracting Bell palsy. The incidence is highest in persons aged 15-45 years. Bell palsy is less common in those younger than 15 years and in those older than 60 years. CLINICAL Section 3 of 10

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History: Most patients presenting to the ED suspect they have suffered a stroke or have an intracranial tumor. The most common complaint is of weakness on one side of their face.

Postauricular pains: Almost 50% of patients experience pain in the mastoid region. The pain frequently occurs simultaneously with the paresis, but precedes the paresis by 2-3 days in about 25% of patients.

Tear flow: Two thirds of patients complain about tear flow. This is due to the reduced function of the orbicularis oculi in transporting the tears. Fewer tears arrive at the lacrimal sac and overflow occurs. The production of tears is not accelerated.

Altered taste: While only one third of patients complain about taste disorders, four fifths of patients show a reduced sense of taste. This may be explained by only half the tongue being involved.

Dry eyes

Hyperacusis: Impaired tolerance to typical levels of noise due to an increased irritability to the sensory neural mechanism.

Physical: Findings of facial paralysis are easily recognizable on physical examination. A careful, complete examination excludes other possible causes of facial paralysis. Strongly consider other etiologies if all branches of the facial nerve are not affected.

The classic definition of Bell palsy describes mononeuric involvement of the facial nerve, yet other cranial nerves are probably affected. The facial nerve is the only cranial nerve eliciting obvious findings on physical examination because of its unique anatomical course from the brain to the lateral face.

Remember that weakness and/or paralysis from involvement of the facial nerve manifests as weakness of the entire face (upper and lower) on the affected side. Focus attention on the voluntary movement of the upper part of the face on the affected side.

In supranuclear lesions such as a cortical stroke (upper motor neuron; above the facial nucleus in the pons), the upper third of the face is spared while the lower two thirds are paralyzed. The orbicularis, frontalis, and corrugator muscles are innervated bilaterally, which explains the pattern of facial paralysis.

Test other cranial nerves; their examination results should be normal.

Tympanic membranes should not be inflamed; presence of infection raises possibility of complicated otitis media.

Causes: "All that glitters is not gold" (William Shakespeare)

The etiology of Bell palsy remains unclear, although vascular, infectious, genetic, and immunologic causes have all been proposed. Patients with other diseases or conditions sometimes develop a peripheral facial nerve palsy, but these are not classified as Bell palsy (see Differentials). Viral infections: Clinical and epidemiologic data lend credence to an infectious origin, which triggers an immunologic response, resulting in damage to the facial nerve. Pathogens leading the list include herpes simplex virus type 1 (HSV-1); herpes simplex virus type 2 (HSV-2); human herpesvirus (HHV); varicella zoster virus (VZV); Mycoplasma pneumoniae; Borrelia burgdorferi; influenza B; adenovirus; coxsackievirus; Ebstein-Barr virus; hepatitis A, B, and C; cytomegalovirus (CMV); and rubella virus.

Pregnancy: Bell palsy is uncommon in pregnancy; however, the prognosis is significantly worse in pregnant women with Bell palsy than among nonpregnant women with palsy.

Genetics: Recurrence rates (4.5-15%) and familial incidence (4.1%) have been addressed in various studies. Genetics may have a role in Bell palsy, but which factors are inherited is unclear.

DIFFERENTIALS Section 4 of 10

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Diabetes Mellitus, Type 1 - A Review Diabetes Mellitus, Type 2 - A Review Fractures, Mandible Herpes Zoster Multiple Sclerosis Tick-Borne Diseases, Lyme

Other Problems to be Considered: Herpes zosterPregnancy (especially third trimester)PolyneuritisAcute otitisChronic otitisTemporal bone fractureInfectious mononucleosisParotid tumorsSarcoidosisCholesteatoma of the middle earAneurysm of vertebral, basilar artery, or carotid arteriesCarcinomatous meningitisFacial trauma (blunt, penetrating, iatrogenic)Leukemic meningitisLeprosyMelkersson-Rosenthal syndromeMiddle ear surgeryOsteomyelitis of the skull baseSkull base tumorWORKUP Section 5 of 10

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Lab Studies: No specific laboratory tests exist to confirm the diagnosis of Bell palsy. Clinical setting determines tests that may be of value. Other potential causes in the differential diagnosis may be confirmed or suspected based on the following diagnostic laboratory tests:

Complete blood count

Erythrocyte sedimentation rate

Thyroid function studies

Lyme titer

Serum glucose level

Rapid plasma reagin (RPR) or Venereal Disease Research Laboratory (VDRL) test

Human immunodeficiency virus (HIV)

Cerebral spinal fluid analysis

Immunoglobulin M (IgM), immunoglobulin G (IgG), and immunoglobulin A (IgA) titers for CMV, rubella, HSV, hepatitis A, hepatitis B, hepatitis C, VZV, M pneumoniae, and B burgdorferi.

Imaging Studies: Bell palsy remains a clinical diagnosis. Imaging studies are not indicated in the ED. Excluding other causes of facial palsy may require one of the following imaging studies depending on clinical setting.

Facial CT scan or plain radiographs: Perform to rule out fractures or bony metastasis.

CT scan is indicated when stroke, or acquired immunodeficiency syndrome (AIDS)-CNS involvement is considered in differential diagnosis

MRI: For a suspected neoplasm of the temporal bone, brain, parotid gland, or other structure, or to evaluate for multiple sclerosis, MRI is the superior method of imaging. The course of the facial nerve through the intratemporal and extratemporal regions from the brain to the facial muscles and glands can be followed on MRI. MRI also may be considered in lieu of CT scan.

Other Tests: Electrodiagnosis of the facial nerve: These studies assess the function of the facial nerve. These tests are rarely performed on an emergent basis.

Electromyography (EMG) and nerve conduction velocities produce a graphic readout of the electrical currents displayed by stimulating the facial nerve and recording the excitability of the facial muscles it supplies. Comparison to the contralateral side helps determine the extent of nerve injury and has prognostic implications. This is not part of the acute workup.

In the nerve excitability test, the threshold of the electrical stimulus producing visible muscle twitching is determined.

Electroneurography (ENoG) compares evoked potentials on the paretic side versus the healthy side.

TREATMENT Section 6 of 10

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Emergency Department Care: The primary treatment of patients with Bell palsy in the ED is pharmacologic management. The remainder of care focuses on reassurance, eye care instructions, and appropriate follow-up care. Steroids

Treatment of Bell palsy with steroids remains controversial. Numerous research articles have been written on the benefit or uselessness of steroids to treat patients with Bell palsy.

Researchers seem to lean more toward using steroids as a means to optimize outcomes. Once the decision to use steroids is made, the consensus is to start immediately. Antiviral agents: Although there is insufficient research evaluating the efficacy of antiviral medicines in Bell palsy, most experts believe in a viral etiology. Therefore, antiviral agents seem a logical choice for pharmacologic management and are commonly recommended.

Eye care: The eyes are frequently unprotected in patients with Bell palsy. This leaves the eyes at risk for corneal drying and foreign body exposure. Manage with tear substitutes, lubricants, and eye protection.

Artificial tears: Use these during waking hours to replace diminished or absent lacrimation.

Lubricants are used during sleep. They may be used during waking hours if artificial tears cannot provide adequate protection. One disadvantage is blurred vision during waking hours.

Eyeglasses or shields protect the eye from injury and reduce drying by decreasing the air currents that come directly in contact with the exposed cornea.

Consultations: The patient's primary care physician or consultant should provide close follow-up care. Documentation should chart the progress of the patient's recovery.

Opinions vary widely on referral to a specialist. Some specific referral indications are listed below:

Neurologist: When other neurologic signs are identified on physical examination and for an atypical presentation of Bell palsy, referral is indicated.

Ophthalmologist: For any unexplained ocular pain or abnormal findings on physical examination of the eyes, the patient should be referred for further workup.

Otolaryngologist: In patients with persistent paralysis, prolonged weakness of the facial muscles, or recurrent weakness, referral is warranted.

Surgeon: Surgery to decompress the facial nerve is recommended occasionally for patients with Bell palsy. Patients with a poor prognosis identified by facial nerve testing or persistent paralysis appear to benefit the most from surgical intervention.

Since most patients recover without medication, physicians may be able to manage patients without prescribing medication. This watchful waiting plan is an option; however, some individuals with Bell palsy never completely recover. Both medications listed below have clinical trials that support and dispute their efficacy.

Drug Category: Corticosteroids -- Have anti-inflammatory properties and cause profound and varied metabolic effects. Modify the body's immune response to diverse stimuli.

Drug NamePrednisone (Deltasone, Orasone, Sterapred) -- Pharmacologic success may be the result of anti-inflammatory effect, which presumably decreases compression of the facial nerve in the facial canal.

Adult Dose1 mg/kg/d PO for 7 d

Pediatric DoseAdminister as in adults

ContraindicationsDocumented hypersensitivity; viral, fungal, connective tissue, and tubercular skin infections; peptic ulcer disease; hepatic dysfunction; GI disease

Interactions Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

PregnancyB - Usually safe but benefits must outweigh the risks.

PrecautionsAbrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Drug Category: Antiviral -- Herpes simplex infections may be a common cause of Bell palsy. Acyclovir is the most-used treatment, but other antiviral agents may be appropriate.

Drug NameAcyclovir (Zovirax) -- Has demonstrated inhibitory activity directed against both HSV-1 and HSV-2, and infected cells selectively take it up.

Adult Dose4000 mg/24 h PO for 7-10 d

Pediatric Dose2 years: 1000 mg PO qid for 10 d

ContraindicationsDocumented hypersensitivity

InteractionsConcomitant use of probenecid or zidovudine prolongs half-life and increases CNS toxicity of acyclovir

PregnancyC - Safety for use during pregnancy has not been established.

PrecautionsCaution in renal failure or when using nephrotoxic drugs

FOLLOW-UP Section 8 of 10

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In/Out Patient Meds: Consider prednisone at an initial dose of 1 mg/kg/day.

Prednisone is a potent drug with a high risk of side effects. The evidence of its usefulness continues to come under scrutiny in the literature. Until efficacy can be clearly defined, it should not be perceived as a standard of care.

With no contraindications and if the physician chooses to administer steroids, the best choice is prednisone at a high dose, as early as possible in the disease course. (Consider tapering on day 5 to 5 mg bid for 5 d.)

Administer acyclovir (Zovirax) 800 mg PO 5 times/d for 10 d; 20 mg/kg in patients younger than 2 years. Recent evidence supports HSV as the presumed cause in more than 70% of Bell palsy cases.

Complications: Most patients with Bell palsy recover without any cosmetically obvious deformities; however, approximately 5% are left with an unacceptably high degree of sequelae.

Incomplete motor regeneration

The largest portion of the facial nerve comprises efferent fibers that stimulate muscles of facial expression. If the motor portion achieves suboptimal regeneration, paresis of all or some of these facial muscles results.

This manifests as (1) oral incompetence, (2) epiphora (excessive tearing), and (3) nasal obstruction.

Incomplete sensory regeneration

Dysgeusia (impairment of taste) may result.

Ageusia (loss of taste) may result.

Dysesthesia (impairment of sensation or disagreeable sensation to normal stimuli) may result.

Aberrant reinnervation of the facial nerve

After the impaired neural conduction of the facial nerve begins the regeneration and repair process, some nerve fibers take an unusual course and connect to neighboring fibers. This aberrant reconnection produces unusual neurologic pathways.

When voluntary movements are initiated, they are accompanied by involuntary movements (eg, the movement of a closed eye following that of the uncovered one). These involuntary movements accompanying voluntary movement are termed synkinesis.

Prognosis: The natural course of Bell palsy varies from early complete recovery to substantial nerve injury with permanent sequelae. Prognostically, patients fall into 3 groups with roughly equal numbers in each group.

Group 1 regains complete recovery of facial motor function without sequelae.

Group 2 experiences incomplete recovery of facial motor function, but no cosmetic defects are apparent to the untrained eye.

Group 3 experiences permanent neurologic sequelae that are cosmetically and clinically apparent.

Most patients develop an incomplete facial paralysis during the acute phase. This group has an excellent prognosis for full recovery. Patients demonstrating complete paralysis are at higher risk for severe sequelae.

Of patients with Bell palsy, 85% achieve complete recovery. Ten percent are bothered by some asymmetry of facial muscles, while 5% experience severe sequelae.

Patient Education: Eye care

Protect the eye from foreign objects and sunlight.

Keep the eye well lubricated.

Educate the patient to report new ocular findings such as pain, discharge, or visual changes.

For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education article Bell Palsy.

PICTURES Section 9 of 10

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Caption: Picture 1. The facial nerve.

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BIBLIOGRAPHY Section 10 of 10

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Adams RD, Victor M, eds: Diseases of the spinal cord, peripheral nerve, and muscle. In: Principles of Neurology. 5th ed. NY: McGraw Hill; 1993:1175-1177.

Cousin GC: Facial nerve palsy following intra-oral surgery performed with local anaesthesia. J R Coll Surg Edinb 2000 Oct; 45(5): 330-3[Medline].

English JB, Stommel EW, Bernat JL: Recurrent Bell's palsy. Neurology 1996 Aug; 47(2): 604-5[Medline].

Helling TD, Neely JG: Validation of objective measures for facial paralysis. Laryngoscope 1997 Oct; 107(10): 1345-9[Medline].

Morgan M, Moffat M, Ritchie L, et al: Is Bell's palsy a reactivation of varicella zoster virus? J Infect 1995 Jan; 30(1): 29-36[Medline].

Morrow MJ: Bell's Palsy and Herpes Zoster Oticus. Curr Treat Options Neurol 2000 Sep; 2(5): 407-416[Medline].

Niparko JK, Mattox DE: Bell's palsy and herpes zoster oticus. In: Current Therapy in Neurologic Disease. 4th ed. Philadelphia: BC Decker; 1993:355-361.

O'Halloran HS, Sen HA, Baker RS: Accidental ocular perforation from self-inflicted facial palsy. Retina 1997; 17(2): 164-6[Medline].

O'Rahilly R, Muller F: Basic Human Anatomy: A regional Study of Human Structure. Philadelphia: WB Saunders Co; 1983:391-98.

Olson WH, Brumback RA, Christoferson LA: Practical Neurology for the Primary Care Physician. Springfield, Ill: Thomas Books; 1981:262.

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Victor M, Martin J: Disorders of the cranial nerves. WJM 2000; 173: 266-268.

Volter C, Helms J, Weissbrich B, et al: Frequent detection of Mycoplasma pneumoniae in Bell's palsy. Eur Arch Otorhinolaryngol 2004 Aug; 261(7): 400-4[Medline].

Wiederholt WC: Bell's palsy. In: Wiederhold WC, ed. Therapy for Neurologic Disorders. NY: Wiley; 1992:257.

Williamson IG, Whelan TR: The clinical problem of Bell's palsy: is treatment with steroids effective? Br J Gen Pract 1996 Dec; 46(413): 743-7[Medline].

Bell Palsy

http://www.emedicine.com/neuro/topic413.htm

Last Updated: June 2, 2005

Synonyms and related keywords: Bell's palsy, idiopathic facial paralysis, facial nerve compression, acute unilateral facial paralysis, bilateral facial palsy, Guillain-Barr syndrome, GBS, sarcoidosis, Lyme disease, meningitis, neoplastic meningitis, infectious meningitis, bilateral neurofibromas, neurofibromatosis type 2, ipsilateral facial palsy


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Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography

Author: Kim Monnell, DO, Consulting Staff, Department of Neurology, Sarasota Memorial Hospital Coauthor(s): Sally B Zachariah, MD, Chief, Department of Neurology, Veteran Affairs Medical Center of Bay Pines; Director, Associate Professor, Department of Neurology, Division of Strokes, University of South Florida College of Medicine; Suzan Khoromi, MD, Fellow, Pain and Neurosensory Mechanisms Branch, National Institute of Dental and Cranial Research, National Institutes of Health

Kim Monnell, DO, is a member of the following medical societies: American Academy of Neurology, and American Osteopathic Association

Editor(s): Milind J Kothari, DO, Program Director, Associate Professor, Department of Internal Medicine, Division of Neurology, Pennsylvania State University Hershey Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Florian P Thomas, MD, MA, PhD, DrMed, Associate Chief of Staff, St Louis VA Medical Center; Associate Director, Neurology Residency Program; Professor, Departments of Neurology, Molecular Virology, and Molecular Microbiology and Immunology, Saint Louis University School of Medicine; Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital; and Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants


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Background: Facial paralysis is a disfiguring disorder that has a great impact on the patient. Facial nerve paralysis may be congenital, neoplastic, or result from infection, trauma, toxic exposures, or iatrogenic causes. The most common cause of unilateral facial paralysis is Bell palsy, also known as idiopathic facial paralysis. Bell palsy is thought to account for approximately 60-75% of cases of acute unilateral facial paralysis.

In 1550, Fallopius noted the narrow lumen in the temporal bone through which a part of the seventh cranial nerve passes. In 1828, Charles Bell made the distinction between the fifth and seventh cranial nerves; he noted that the seventh nerve was involved mainly in the motor function of the face and the fifth nerve was concerned mainly with the sensory perception of the face.

Even today, controversy still surrounds the etiology and treatment of Bell palsy. Clinical features of Bell palsy that may help distinguish it from other causes of facial paralysis include sudden onset of unilateral facial paralysis (less than 48 hours), absence of signs and symptoms of CNS disease, and absence of signs and symptoms of ear or posterior fossa disease.

Pathophysiology: The course of the facial nerve is tortuous, both centrally and peripherally (see Image 1).

The facial nerve nucleus lies within the reticular formation of the pons, adjacent to the fourth ventricle. The facial nerve roots include fibers from the motor, solitary, and salivatory nuclei. The nervus intermedius comprises fibers from salivatory and solitary nuclei (it contains sensory fibers from the tongue, mucosa, and postauricular skin as well as parasympathetic fibers to the salivary and lacrimal glands). The fibers of the facial nerve then course around the sixth cranial nerve nucleus and exit the pons at the cerebellopontine angle. The fibers go through the internal auditory canal along with the vestibular portion of the eighth cranial nerve. The narrowest portion of the internal auditory canal is the labyrinthine segment. This is the location that is thought to be the most common site of compression of the facial nerve in Bell palsy.

The seventh cranial nerve contains parasympathetic fibers to the nose, palate, and lacrimal glands. The preganglionic parasympathetic fibers that originate in the salivatory nucleus join the fibers from nucleus solitarius to form the nervus intermedius. These fibers then synapse with the submandibular ganglion, which has fibers that supply the sublingual and submandibular glands. The fibers from the nervus intermedius also supply the pterygopalatine ganglion, which has parasympathetic fibers that supply the nose, palate, and lacrimal glands.

The facial nerve passes through the stylomastoid foramen in the skull and terminates into the zygomatic, buccal, mandibular, and cervical branches. These nerves serve the muscles of facial expression, which include frontalis, orbicularis oculi, orbicularis oris, buccinator, and platysma. Other muscles innervated by the facial nerve include stapedius, stylohyoid, posterior belly of the digastric, occipitalis, and anterior and posterior auricular muscles. All muscles of the facial nerve are derived from the second brachial arch.

The location of injury of the facial nerve in Bell palsy is peripheral to the seventh nerve nucleus. The injury is thought to occur near or at the geniculate ganglion. If the lesion is proximal to the geniculate ganglion, the motor paralysis is accompanied by gustatory and autonomic abnormalities. Lesions between the geniculate ganglion and the origin of the chorda tympani produce the same effect except that they spare lacrimation. If the lesion is at the stylomastoid foramen, it may result in facial paralysis only.

Bell palsy is thought to be caused by edema and ischemia resulting in compression of the facial nerve in its course through the bony canal. The cause of the edema and ischemia is still being debated. In the past, cold exposure (eg chilly wind, cold air conditioning, or driving with the car window down) were considered the only triggers to Bell palsy. However, most authors believe that the herpes simplex virus (HSV) is the most likely cause. Actually studying the causal relationship between HSV and Bell palsy is difficult because of the ubiquitous nature of HSV.

In 1972, McCormick first suggested that HSV is responsible for idiopathic facial paralysis. This was based on the analogy that HSV was found in cold sores, and he hypothesized that HSV may remain latent in the geniculate ganglion. Since then, autopsy studies have shown HSV in the geniculate ganglion of patients with Bell palsy. Murakami et al performed polymerase chain reaction (PCR) testing for HSV in the endoneural fluid of the seventh nerve of patients who underwent surgery for Bell palsy. Eleven of the 14 patients were found to have HSV in the endoneural fluid.

Assuming that HSV is the etiologic agent in Bell palsy is reasonable. If this is true, then the virus is most likely to travel up the axons of the sensory nerves and reside in the ganglion cells. At times of stress the virus will reactivate, causing local damage to the myelin. Thus, Bell palsy may be secondary to viral and/or autoimmune reactions causing the facial nerve to demyelinate, resulting in unilateral facial paralysis.

Frequency: In the US: The annual incidence of Bell palsy is approximately 23 per 100,000.

The right side is affected 63% of the time.

Persons with diabetes have a risk as much as 29% higher than persons without diabetes of being affected by Bell palsy. Thus, blood glucose levels at time of diagnosis of Bell palsy may detect undiagnosed diabetics.

Internationally: The highest incidence was found in a study in Seckori, Japan, in 1986 and the lowest incidence was found in Sweden in 1971. Most population studies generally show an annual incidence of 15-30 per 100,000.

Mortality/Morbidity: The majority of patients who suffer from Bell palsy have neurapraxia or local nerve conduction block. These patients are likely to have a prompt and complete recovery of the nerve. Patients with axonotmesis, with disruption of the axons, have a fairly good recovery but it is usually not complete. The risk factors thought to be associated with a poor outcome in patients with Bell palsy include (1) age greater than 60 years, (2) complete paralysis, and (3) decreased taste or salivary flow on the side of paralysis (usually 10-25% compared to the patient's normal side). Other factors thought to be associated with poor outcome include pain in the posterior auricular area and decreased lacrimation.

Patients generally have a good prognosis; approximately 80-90% of patients recover without noticeable disfigurement within 6 weeks to 3 months. Patients aged 60 years or older have an approximately 40% chance of complete recovery and have a higher rate of sequelae. Patients younger than 30 years have only a 10-15% chance of less than complete recovery and sequelae. If no recovery occurs by 4 months, then the patient is more likely to have sequelae from the disease, which include synkinesis, crocodile tears, and rarely hemifacial spasm.

Synkinesis is an abnormal contracture of the facial muscles while smiling or closing the eyes. It may be mild and result in slight movement of the chin when the patient blinks, eye closure with smiling, or contracture around the mouth while blinking. Crocodile tears are observed; patients shed tears while they eat.

Facial spasm is a very rare complication of Bell palsy. It occurs as tonic contraction of one side of the face. Spasms are more likely to occur during times of stress or fatigue and may occur during sleep. This condition may occur secondary to compression of the root of the seventh nerve by an aberrant blood vessel, tumor, or demyelination of the nerve root. It occurs most commonly in the fifth and sixth decades of life, and sometimes the etiology is not found. The presence of progressive facial hemispasm with other cranial nerve findings indicates a possibility of a brainstem lesion.

Diabetic patients are 30% more likely than nondiabetic patients to have only partial recovery; recurrence of Bell palsy is also more common among diabetic patients.

Bell palsy accounts for only 23% of bilateral facial paralysis. The majority of patients with bilateral facial palsy have Guillain-Barr syndrome (GBS), sarcoidosis, Lyme disease, meningitis (neoplastic or infectious), or bilateral neurofibromas (in patients with neurofibromatosis type 2).

Bell palsy recurs in 10-15% of patients. It may recur on the ipsilateral or contralateral side of the initial palsy. Recurrence usually is associated with a family history of recurrent Bell palsy. Approximately 30% of patients with recurrent ipsilateral facial palsy were found to have tumors of the seventh nerve or parotid gland. Patients with recurrent ipsilateral facial palsy should undergo MRI or high-resolution CT scan to rule out neoplastic or inflammatory (eg, multiple sclerosis, sarcoidosis) cause of recurrence.

Sex: Bell palsy appears to affect the sexes equally. However, young women aged 10-19 years are more likely to be affected than men in the same age group.

Pregnant women have a 3.3 times higher risk of being affected by Bell palsy than nonpregnant women; Bell palsy occurs most frequently in the third trimester.

Age: The lowest incidence is found in persons younger than 10 years and the highest incidence in persons aged 60 years or older.

CLINICAL Section 3 of 11

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History: Bell palsy is a diagnosis of exclusion. The diagnosis must be made on the basis of a thorough history and physical examination and use of diagnostic testing when necessary.

Symptoms of Bell palsy

Acute onset of unilateral upper and lower facial paralysis (over a 48-h period)

Posterior auricular pain

Decreased tearing

Hyperacusis

Taste disturbances

The paralysis must include the forehead and lower aspect of the face. The patient may report inability to close the eye or to smile on the affected side. He or she also may report increased saliva on the side of the paralysis. If the paralysis involves only the lower portion of the face, a central cause should be suspected (ie, supranuclear). If the patient complains of contralateral weakness or diplopia in conjunction with the supranuclear facial palsy, a stroke or intracerebral lesion should be strongly suspected.

Half of the patients affected with Bell palsy may complain of posterior auricular pain. Ask the patient if he or she has experienced trauma, which may account for the pain and facial paralysis.

One third of patients may experience hyperacusis in the ear ipsilateral to the paralysis, which is secondary to weakness of the stapedius muscle.

One sixth of patients experience decreased lacrimation.

Many patients report numbness on the side of the paralysis. Some authors believe that this is secondary to involvement of the trigeminal nerve, whereas other authors argue that this symptom is probably due to lack of mobility of the facial muscles and not lack of sensation.

If a patient has gradual onset of facial paralysis, weakness of the contralateral side, or history of trauma or infection, other causes of facial paralysis must be strongly considered. Patients who have bilateral facial palsy must be evaluated for GBS, Lyme disease, and meningitis.

If a patient is from the Northeast, Lyme disease should be considered as a cause of facial paralysis, and serologic testing should be performed.

Recurrent ipsilateral facial paralysis must raise the suspicion of a tumor of the seventh nerve or parotid gland. If the patient reports sudden onset of hearing loss and severe pain with the onset of facial paralysis, Ramsay Hunt syndrome must be considered.

Symptoms associated with seventh nerve neoplasm include slowly progressive paralysis, facial hyperkinesis, severe pain, recurrent palsy, and other cranial nerve involvement. Cerebellopontine tumors may affect the seventh, eighth, and fifth cranial nerves simultaneously. Patients with a progressive paralysis of the facial nerve lasting longer than 3 weeks should be evaluated for neoplasm.

Physical: Initial inspection of the patient demonstrates flattening of the forehead and nasolabial fold on the side affected with the palsy.

When the patient is asked to raise the eyebrows, the side of the forehead with the palsy will remain flat.

When the patient is asked to smile, the face becomes distorted and lateralizes to the side opposite the palsy.

The patient is not able to close the eye completely on the affected side. On attempted eye closure, the eye rolls upward and inward on the affected side. This is known as Bell phenomenon and is considered a normal response to eye closure.

A careful examination of the head, ears, eyes, nose, and throat (HEENT) must be carried out in all patients with facial paralysis.

The external auditory canal must be inspected for vesicles, injection, infection, or trauma.

The patient may have decreased sensation to pinprick in the posterior auricular area.

The patient who has paralysis of the stapedius muscle will report hyperacusis.

Bell phenomenon is observed on attempted eye closure.

With weakness/paralysis of the orbicularis oculi muscle (facial nerve innervation) and normal function of the levator muscle (oculomotor nerve innervation) and Mueller muscle (sympathetic innervation), eye closure may be partial or absent. The tear reflex may also be absent in many cases of Bell palsy. For these reasons the patient may have decreased tearing and susceptibility to corneal abrasion and dryness of the eye. The patient may appear to have loss of corneal reflex on the affected side; however, the contralateral eye blinks when testing the corneal reflex on the affected side.

A careful oral examination must be performed.

Taste and salivation are affected in many patients with Bell palsy.

Taste may be assessed by holding the tongue with gauze and testing each side of the tongue independently with salt, sugar, and vinegar. The mouth must be washed after testing with different substances. The affected side has decreased taste as compared to the normal side.

Careful neurologic examination is necessary in patients with facial paralysis. A neurologic abnormality warrants neurologic referral and further testing such as MRI of the brain, lumbar puncture, and electromyography (EMG) where appropriate.

Causes: See Pathophysiology.


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Acute Inflammatory Demyelinating Polyradiculoneuropathy Amyloid Angiopathy Anterior Circulation Stroke Arsenic Basilar Artery Thrombosis Benign Skull Tumors Brainstem Gliomas Cerebral Aneurysms Guillain-Barre Syndrome in Childhood Intracranial Hemorrhage Low-Grade Astrocytoma Lyme Disease Meningioma Meningococcal Meningitis Multiple Sclerosis Mbius Syndrome Neurofibromatosis, Type 2 Neurosarcoidosis Neurosyphilis Tuberculous Meningitis

Other Problems to be Considered: Basal skull fracturesBarotraumaBotulismCarcinomatosisCarotid disease and strokeDiphtheriaFacial injuriesForceps deliveryHIVIatrogenic (as in otologic, neurotologic, skull base, or parotid surgery)IdiopathicInfectionIntratemporal internal carotid artery aneurysmMalignant otitis externaMeningitisMumpsParotid tumorRamsay Hunt syndromeSarcomaTeratomaTetanusThalidomide exposureTraumaToxicVascularWegener vasculitis

WORKUP Section 5 of 11

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Lab Studies: In areas where Lyme disease is endemic, Lyme titers (IgM and IgG) should be obtained.

Blood glucose or hemoglobin A1c may be obtained to determine if the patient has undiagnosed diabetes.

Serum titers for HSV may be obtained, but this is usually not helpful owing to the ubiquitous nature of this virus.

Imaging Studies: If the history and physical examination lead to a diagnosis of Bell palsy, then immediate imaging is not necessary.

Imaging is not required because most patients with Bell palsy improve within 8-10 weeks. If the paralysis does not improve or worsens, imaging may be useful.

The MRI of patients with Bell palsy may show enhancement of the seventh nerve at, or near, the geniculate ganglion. However, if the paralysis progresses over weeks, the possibility is high of a neoplasm compressing the seventh cranial nerve. Tumors that compress or involve the seventh cranial nerve include schwannoma (most common), hemangioma, meningioma, and sclerosing hemangioma.

MRI is preferred for imaging the cerebellopontine angle.

If the patient has a history of trauma, CT scan of the temporal bone may be required.

If the patient has a palpable parotid mass, imaging may be necessary.

Other Tests: The following tests may be performed in the office setting. However, they require both the patient's and physician's time. They may be helpful in assessing the extent of the damage to the seventh nerve.

The stethoscope loudness test may be used to assess the functioning of the stapedius muscle. The patient wears the stethoscope, and the activated tuning fork is placed at the bell of the stethoscope. The loud sound will lateralize to the side of the paralyzed stapedius muscle

The Schirmer blotting test may be used to assess tearing function. The use of benzene will stimulate the nasolacrimal reflex, and the degree of tearing can be compared between the paralyzed and normal sides.

Salivary flow also may be tested. The physician places a small catheter into both the paralyzed and normal submandibular glands. The patient is then asked to suck on a lemon, and the salivary flow is compared between the 2 sides. The normal side is the control.

Useful tests for evaluation of the function of the facial nerve include nerve conduction testing and EMG.

These tests may aid in assessing the outcome of a patient who has persistent and severe Bell palsy. This test is most useful when performed 3-10 days after the onset of paralysis.

Nerve conduction responses are abnormal if a difference of 50% in amplitude between the paralyzed and normal side is detected; a difference of 90% between the 2 sides suggests a poorer prognosis.

May et al demonstrated that prognosis may be favorable if the motor amplitude of the affected side was greater than 25% of that of the normal side. An incomplete recovery was observed in patients whose results demonstrated less than 25% amplitude on the paralyzed side.

Blink reflexes can be used to measure conduction across the involved segment but they are commonly absent in Bell palsy.

Brainstem auditory-evoked response (BAER) may be obtained in patients with peripheral seventh nerve lesions and other neurologic involvement.

BAER measures the transmission of response through the brain stem and is effective in detecting, notably, retrocochlear lesions.

Hendrix and Melnick evaluated BAER of 17 patients with Bell palsy. They found no evidence of retrocochlear lesions of the auditory system in any of their patients with Bell palsy.

In another study by Shannon et al, BAER was recorded in 27 patients with Bell palsy; only 6 patients had prolonged brainstem transmission but normal auditory function.

These studies were small and do not support routine use of BAER in patients with Bell palsy. However, when a patient presents with multiple cranial neuropathies, ie, of CN VII and VIII, BAER may be useful.

Histologic Findings: A review of 12 autopsy cases of patients with Bell palsy was summarized in Peter Dyck's Peripheral Neuropathy. This stated that the majority of cases showed inflammatory changes around the mastoid cells and walls of the arteries. The most common site of involvement was the geniculate ganglion.

Surgical findings described constriction of the nerve at the stylomastoid foramen with swelling of the nerve itself. Microscopic findings showed an inflammatory reaction with infiltration of macrophages on the nerve.


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Medical Care: In general, persons with true Bell palsy have an excellent prognosis. The goals of treatment are to improve function of the facial nerve and reduce neuronal damage. Many issues must be addressed in treating patients with Bell palsy. The most important consideration is the onset of symptoms. Treatment may be considered for patients who have the onset of paralysis within 1-7 days of the initial office visit. The American Academy of Neurology published a practice parameter in 2001 stating that steroids are probably effective and acyclovir (with prednisone) is possibly effective for treatment of Bell palsy. There was insufficient evidence for any recommendation on facial decompression surgery. Because treatment of Bell palsy is still controversial, a study is currently being performed in Scotland. It is a placebo-controlled study that will determine the efficacy of prednisone versus prednisone and acyclovir versus placebo. The most widely accepted treatment for Bell palsy is corticosteroids. However, the use of steroids is still controversial because most patients recover without treatment.

The recommended dose of prednisone is 1 mg/kg or 60 mg/d for 6 days, followed by a taper, for a total of 10 days.

Many trials have been carried out to study the efficacy of prednisone in Bell palsy. Early studies had small numbers of patients and variable outcomes.

In 1972, Adour et al conducted a large, controlled clinical trial, which found that 89% of patients treated with prednisone had full recovery as compared to 64% of patients treated with placebo.

When using corticosteroids for the treatment of Bell palsy, caution should be used in patients with tuberculosis, peptic ulcer disease, diabetes mellitus, renal or hepatic dysfunction, or malignant hypertension.

Since HSV is widely accepted as the likely etiologic agent of Bell palsy, trials using acyclovir have been conducted. The dose of acyclovir is 400 mg orally 5 times per day.

A prospective randomized trial with 101 patients comparing prednisone and acyclovir demonstrated that the prednisone group had a better clinical recovery. In another prospective randomized trial with 99 patients, prednisone monotherapy was compared to the combination of prednisone and acyclovir. This study demonstrated that combination therapy was more effective in preventing nerve degeneration as measured by electrodiagnostic tests.

Whether to use prednisone alone or combination therapy is left to the discretion of the physician. For patients who have a contraindication to steroid therapy, acyclovir may be given as solitary treatment.

That eye care is imperative in Bell palsy is accepted universally. The patient's eye is at risk for drying, corneal abrasion, and corneal ulcers. Eye care includes artificial tears for use during the day as well as eyeglasses or shields. At night, eye lubricants may be used. If artificial tears are not effective during the daytime, then lubricants may be used; however, they may cause blurring of vision.

Surgical Care: Surgery for Bell palsy is controversial. In the past, surgical decompression of the facial nerve was considered for patients whose facial muscles demonstrated less than 90% of normal activity on electrophysiologic studies. Surgical decompression of the facial nerve involves a middle fossa craniotomy with an extradural approach. However, recent trials suggest this is not beneficial in patients with Bell palsy.

Consultations: If the initial impression based on the history and physical examination is not Bell palsy, then a neurologic or otolaryngologic consultation is needed.

If the paralysis persists for several months, a neurology or otolaryngology consultation should be sought.

Patients who report persistent dry eye or painful eye should be referred to an ophthalmologist.

the goals of pharmacotherapy are to reduce morbidity and prevent complications.

Drug Category: Corticosteroids -- Prednisone can be used but has many adverse effects including fluid retention, hypokalemia, myopathy, peptic ulcer, headache (pseudotumor), menstrual irregularities, cataracts, glaucoma, and manifestation of latent diabetes mellitus. Signs of infection may also be masked in patients taking prednisone. Physicians should use caution when using prednisone in patients with the aforementioned conditions.

Drug NamePrednisone (Deltasone, Orasone, Meticorten) -- Glucocorticoid absorbed readily from GI tract. It has anti-inflammatory and immune-modulating effects, and profound and varied metabolic effects.

Adult Dose1 mg/kg or 60 mg PO qd for 7 d followed by taper for total of 10 d

Pediatric Dose1 mg/kg PO qd for 6 d followed by taper for total of 10 d

ContraindicationsDocumented hypersensitivity; severe uncontrolled diabetes; systemic fungal infections; peptic ulcer disease; tuberculosis; severe osteoporosis; severe adverse reactions to corticosteroids

InteractionsDrugs that induce hepatic enzymes may increase clearancethese include phenobarbital, phenytoin, and rifampin; patients on aspirin or Coumadin must be monitored closely for GI bleeding

PregnancyB - Usually safe but benefits must outweigh the risks.

PrecautionsPatients are at risk for hyperglycemia, electrolyte abnormalities (especially hypokalemia in patients taking diuretics), osteoporosis, avascular necrosis, psychosis, and myopathy or worsening weakness in patients with myasthenia gravis; abrupt discontinuation of prednisone without taper puts patient at risk for adrenal crisis

Drug Category: Antiviral medication -- Acyclovir has been used in the treatment of Bell palsy in combination with prednisone or used alone in patients who cannot take prednisone.

Drug NameAcyclovir (Zovirax) -- Antiviral drug that has inhibitory activity against HSV-1, HSV-2, and VZV; selectively taken up by infected cells.

Adult Dose400 mg PO 5 times/d for 10 d

Pediatric Dose2 years: 20 mg/kg PO for 10 d


InteractionsProbenecid or zidovudine prolongs half-life and may increase CNS toxicity


PrecautionsCaution in renal failure or when using nephrotoxic drugs


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Further Outpatient Care: If the paralysis is not resolved or is progressing to complete paralysis, a thorough neurologic and HEENT examination should be performed to rule out neoplastic causes of seventh nerve palsy.

The patient should be monitored if the initial EMG shows the involved facial muscles to have less than 25% of the function of the normal side.

If the residual paralysis is severe, the patient should be referred for counseling.

Complications: Approximately 30% of patients with Bell palsy experience sequelae of the paralysis, which include incomplete motor recovery, incomplete sensory regeneration, and parasympathetic impairment.

Incomplete motor recovery may manifest as oral incompetence or epiphora.

Incomplete sensory recovery may result in dysgeusia (impairment of taste) or ageusia (loss of taste).

Parasympathetic impairment causes aberrant function of lacrimal glands, which manifests as crocodile tears; patients report shedding tears while eating.

Prognosis: The natural course of Bell palsy varies from early complete recovery to substantial nerve injury resulting in persistent paralysis and synkinesis.

One third of patients regain complete recovery of facial motor function without sequelae.

One third of patients have incomplete recovery of facial motor function. These patients do not have any noticeable abnormalities.

The remainder of patients suffer from permanent neurological and cosmetic abnormalities, which are apparent.

Patient Education: To prevent corneal abrasions, the patient should be educated concerning eye care.

They also should be encouraged to do facial muscle exercises using passive range of motion as well as actively closing their eyes and smiling.

For excellent patient education resources, visit eMedicine's Brain and Nervous System Center. Also, see eMedicine's patient education article Bell Palsy.

MISCELLANEOUS Section 9 of 11

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Medical/Legal Pitfalls: In most cases, the diagnosis of Bell palsy is straightforward as long as the patient underwent a thorough history and physical examination. Failure to recognize structural, infectious, or vascular lesions leading to seventh nerve damage may result in further deterioration of the patient's condition. For example, if other cranial nerve, motor, or sensory symptoms were present at the time, then treatable or preventable nervous system diseases should be sought. These may include stroke, GBS, basilar meningitis, or cerebellar pontine angle tumor.

PICTURES Section 10 of 11

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Caption: Picture 1. The facial nerve

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Hemifacial Spasm (HFS) http://emedicine.com/NEURO/topic154.htm

Last Updated: October 11, 2006

Synonyms and related keywords: craniofacial movement disorders, facial myoclonus, facial dystonia, botulinum toxin, BTX therapy


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Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography

Author: Steven Gulevich, MD, Department of Neurology, Swedish Medical Center of Englewood, Colorado

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

Editor(s): Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare; Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida College of Medicine; and Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants


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Background: 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.

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.

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

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

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 HFS.

Compressive lesions (eg, tumor, arteriovenous malformation, Paget disease) and noncompressive lesions (eg, stroke, multiple sclerosis plaque, basilar meningitis) may present as HFS. 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.Race: All races are affected equally.

Sex: A slight female preponderance exists in HFS.

Age: 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). CLINICAL Section 3 of 10

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History: Involuntary facial movement is the only symptom. Fatigue, anxiety, or reading may precipitate the movements.

Hemimasticatory spasm

Hemimasticatory spasm is analogous to HFS 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 HFS, hemimasticatory spasm responds to treatment with medications and BTX.

However, less evidence exists that exploratory surgery benefits patients with this condition.

Myoclonic movements

Myoclonic movements affecting facial musculature also may arise from lesions at the brain or brainstem level.

These are distinguished from HFS 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 (OMD) refers to dystonia affecting the lower facial musculature, predominantly the jaw, pharynx, and tongue.

When OMD occurs in conjunction with blepharospasm, the disorder is termed Meige syndrome.

Jaw-opening forms of OMD indicate primary involvement of the digastric and lateral pterygoid. Jaw-closing OMD involves the masseter, temporalis, and medial pterygoid.

Jaw deviation, indicating predominant involvement of the lateral pterygoid, is rare.

BTX is the preferred treatment for OMD 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.

Because of the risk of aspiration, never inject BTX 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 HFS.

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 BTX.

Most cases are idiopathic and resolve without treatment over several weeks.

Physical: The only physical finding in hemifacial spasm is involuntary facial movements.

Spontaneous HFS manifests with facial spasms that represent myoclonic jerks and are analogous to segmental myoclonus, which may affect other body regions.

Postparalytic HFS (following facial nerve trauma such as Bell palsy) manifests as facial synkinesis and contracture.

Causes: Idiopathic

Vascular compression

Facial nerve compression by mass

Brainstem lesion such as stroke or multiple sclerosis plaque

Secondary to trauma or Bell palsy


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Other Problems to be Considered: Benign essentialBlepharospasmOMDCraniofacial tremorFacial choreaTicsFacial myokymiaWORKUP Section 5 of 10

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Lab Studies: Early cases of HFS 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 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.

Blink reflex studies may reveal synkinesis, which 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 Studies: Magnetic resonance imaging is the imaging study of choice, especially if an underlying compressive lesion is suspected.

Perform angiography and/or magnetic resonance angiography prior to a vascular decompression surgical procedure.

Other Tests: Cerebral angiography offers little diagnostic value in HFS. Ectatic blood vessels rarely are identified, and it is difficult to correlate vessels with the facial nerve. As angiography may identify an aneurysm or vascular anomaly, it often is performed prior to decompressive surgery to clarify the vascular anatomy.

Procedures: In most patients, the treatment of choice is injection of BTX under 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.

Side effects of BTX injection (eg, facial asymmetry, ptosis, facial weakness) usually are transient.

Most patients report a highly satisfactory response.

Caution patients that although BTX ablates the muscular spasm, the sensation of spasm often persists.


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Medical Care: Use medications in patients with noncompressive lesions and early idiopathic HFS.

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.

Medications may be used in early HFS (when spasms are mild and infrequent) or in patients who decline BTX injection.

Surgical Care: Treat compressive lesions surgically.

Ectatic blood vessels cause HFS by compressing the facial nerve as it exits the brainstem.

Surgical decompression of these blood vessels can yield excellent results.

Patients with apparently idiopathic HFS may benefit from posterior fossa exploration and microvascular decompression.

Myectomy rarely is required.

MEDICATION Section 7 of 10

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

Drug Category: Toxins -- Botulinum toxin type A is the drug of choice. 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; 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.

Drug NameBotulinum toxin type A (BOTOX) -- Useful in reducing excessive, abnormal contractions associated with blepharospasm; 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.

Adult DoseInitial dosing: Inject 1.25-2.5 U (0.05-0.1 mL) IM into abnormally contracting muscles via hollow EMG needle; not to exceed 25 U when given as a single injection or 200 U when given as a cumulative dose in a 30 d period

Pediatric Dose12 years: Administer as in adults


InteractionsCaution in patients taking aminoglycoside antibiotics or any other drug that interferes with neuromuscular transmission as they may potentiate effect of botulinum toxin


PrecautionsDo not exceed recommended dosages and frequencies of administration; presence of antibodies may reduce effectiveness of therapy

Drug NameBotulinum toxin type B (Myobloc) -- When botulinum toxin injection is indicated and type A toxin is ineffective, injection with type B (Myobloc) should be considered.

Adult DoseNot established: This author suggests starting dose of 500-1000 U IM, divided among abnormally contracting muscles

Pediatric DoseNot established

ContraindicationsDocumented hypersensitivity to drug; patients with hypersensitivity to type A toxin, hypersensitivity to type B is significant concern, and use of type B in these patients is not recommended

InteractionsCaution in patients taking aminoglycoside antibiotics or any other drug that interferes with neuromuscular transmission because they may potentiate effect of botulinum toxin


PrecautionsLikely to cause pain at injection site for a few seconds immediately following administration

Drug Category: Benzodiazepines -- May potentiate effects of GABA and facilitate inhibitory GABA neurotransmission. May act in the spinal cord to induce muscle relaxation. Individualize treatment for each patient.

Drug NameClonazepam (Klonopin) -- Useful in suppressing muscle contractions by facilitating inhibitory GABA neurotransmission and other inhibitory transmitters.

Adult DoseInitial dose: 1.5 mg PO in 3 divided doses Maintenance dose: Increase initial dose by 0.5-1 mg PO q3d to a dose range of 0.05-0.2 mg/kg Alternative maintenance dose: 7-12 mg/d PO; not to exceed 20 mg/d

Pediatric Dose70 kg = 1500-2100 mg/kg/d


InteractionsMay decrease levels of dihydropyridine calcium antagonists and oral contraceptives; can reduce serum concentrations of carbamazepine, phenobarbital, phenytoin and valproic acid; when oxcarbazepine is given in doses above 1200 mg/d may increase phenytoin and phenobarbital serum concentrations significantly; oxcarbazepine can reduce serum concentrations of oral contraceptives and make oral contraceptives ineffective; can increase clearance of felodipine


PrecautionsMay decrease levels of dihydropyridine calcium antagonists and oral contraceptives; can reduce serum concentrations of carbamazepine, phenobarbital, phenytoin and valproic acid; when oxcarbazepine is given in doses above 1200 mg/d may increase phenytoin and phenobarbital serum concentrations significantly; oxcarbazepine can reduce serum concentrations of oral contraceptives and make oral contraceptives ineffective; can increase clearance of felodipine


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Prognosis: HFS is a progressive, nonfatal illness. It almost always responds favorably to treatment.

Patient Education: For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article BOTOX Injections.

MISCELLANEOUS Section 9 of 10

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Medical/Legal Pitfalls: At initial evaluation, consider HFS a symptom, not a diagnosis.

An abnormal neurologic examination (except for the facial movements) should prompt the search for an underlying cause (eg, compressive lesion, tumor, stroke).

Look for demyelinating disease as a cause when HFS presents before 50 years.


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Adler CH, Zimmerman RA, Savino PJ, et al: Hemifacial spasm: evaluation by magnetic resonance imaging and magnetic resonance tomographic angiography. Ann Neurol 1992 Oct; 32(4): 502-6[Medline].

Colosimo C, Chianese M, Giovannelli M, et al: Botulinum toxin type B in blepharospasm and hemifacial spasm. J Neurol Neurosurg Psychiatry 2003 May; 74(5): 687[Medline].

Cruccu G, Inghilleri M, Berardelli A, et al: Pathophysiology of hemimasticatory spasm. J Neurol Neurosurg Psychiatry 1994 Jan; 57(1): 43-50[Medline].

Elston JS: The management of blepharospasm and hemifacial spasm. J Neurol 1992 Jan; 239(1): 5-8[Medline].

Jankovic J, Schwartz K, Donovan DT: Botulinum toxin treatment of cranial-cervical dystonia, spasmodic dysphonia, other focal dystonias and hemifacial spasm. J Neurol Neurosurg Psychiatry 1990 Aug; 53(8): 633-9[Medline].

Jannetta PJ, Abbasy M, Maroon JC, et al: Etiology and definitive microsurgical treatment of hemifacial spasm. Operative techniques and results in 47 patients. J Neurosurg 1977 Sep; 47(3): 321-8[Medline].

Kraft SP, Lang AE: Cranial dystonia, blepharospasm and hemifacial spasm: clinical features and treatment, including the use of botulinum toxin. CMAJ 1988 Nov 1; 139(9): 837-44[Medline].

Mauriello JA, Leone T, Dhillon S, et al: Treatment choices of 119 patients with hemifacial spasm over 11 years. Clin Neurol Neurosurg 1996 Aug; 98(3): 213-6[Medline].

Moller AR: The cranial nerve vascular compression syndrome: I. A review of treatment. Acta Neurochir (Wien) 1991; 113(1-2): 18-23[Medline].

Moller AR: The cranial nerve vascular compression syndrome: II. A review of pathophysiology. Acta Neurochir (Wien) 1991; 113(1-2): 24-30[Medline].

Reimer J, Gilg K, Karow A, et al: Health-related quality of life in blepharospasm or hemifacial spasm. Acta Neurol Scand 2005 Jan; 111(1): 64-70[Medline].

NOTE:

Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER

Hemifacial Spasm excerptEyelid Myokymia

http://www.emedicine.com/oph/topic607.htm

Last Updated: August 4, 2005

Synonyms and related keywords: eyelid twitching, eyelid jumping, muscle contractions, blepharospasm, Meige syndrome, hemifacial spasm, spastic-paretic facial contracture, botulinum toxin A, BOTOX, BOTOX injections


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Author: Byron L Lam, MD, Professor, Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine

Byron L Lam, MD, is a member of the fol

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