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  • 7/29/2019 SARAF Bell Palsy

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    Bell Palsy

    Last Updated: March 5, 2007

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    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

    Author InformationIntroductionClinicalDifferentialsWorkupTreatmentMedicationFollow-upPicturesBibliography

    Author: Michael Lambert, MD, Fellowship Director of Emergency Ultrasound,

    Clinical Assistant Professor, Department of Emergency Medicine, Resurrection

    Medical CenterMichael 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, andSociety 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 ofMedicine, 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

    DisclosureINTRODUCTION Section 2 of 10

    Author InformationIntroductionClinicalDifferentialsWorkupTreatmentMedicationFollow-upPicturesBibliography

    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.

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    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 thecerebellopontine 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 in65 in a lifetime.

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    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

    Author InformationIntroductionClinicalDifferentialsWorkupTreatmentMedicationFollow-upPicturesBibliography

    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 thereduced 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 increasedirritability to the sensory neural mechanism.

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    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 thefacial 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 thelower 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.

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    DIFFERENTIALS

    Diabetes Mellitus, Type 1 - A Review

    Diabetes Mellitus, Type 2 - A ReviewFractures, Mandible

    Herpes Zoster

    Multiple Sclerosis

    Tick-Borne Diseases, Lyme

    Other Problems to be Considered:

    Herpes zoster

    Pregnancy (especially third trimester)

    PolyneuritisAcute otitis

    Chronic otitis

    Temporal bone fracture

    Infectious mononucleosis

    Parotid tumors

    Sarcoidosis

    Cholesteatoma of the middle ear

    Aneurysm of vertebral, basilar artery, or carotid arteries

    Carcinomatous meningitis

    Facial trauma (blunt, penetrating, iatrogenic)

    Leukemic meningitis

    Leprosy

    Melkersson-Rosenthal syndrome

    Middle ear surgery

    Osteomyelitis of the skull base

    Skull base tumor

    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 inthe differential diagnosis may be confirmed or suspected based on the

    following diagnostic laboratory tests:

    o Complete blood count

    o Erythrocyte sedimentation rate

    o Thyroid function studies

    o Lyme titer

    o Serum glucose level

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    o Rapid plasma reagin (RPR) or Venereal Disease Research Laboratory

    (VDRL) test

    o Human immunodeficiency virus (HIV)

    o Cerebral spinal fluid analysis

    o Immunoglobulin M (IgM), immunoglobulin G (IgG), and

    immunoglobulin A (IgA) titers for CMV, rubella, HSV, hepatitis A,

    hepatitis B, hepatitis C, VZV,M pneumoniae, andB 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.

    o Facial CT scan or plain radiographs: Perform to rule out fractures or

    bony metastasis.

    o CT scan is indicated when stroke, or acquired immunodeficiency

    syndrome (AIDS)-CNS involvement is considered in differential

    diagnosis

    o 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 throughthe 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.

    o Electromyography (EMG) and nerve conduction velocities produce a

    graphic readout of the electrical currents displayed by stimulating thefacial 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.

    o In the nerve excitability test, the threshold of the electrical stimulus

    producing visible muscle twitching is determined.

    o Electroneurography (ENoG) compares evoked potentials on the paretic

    side versus the healthy side.

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    TREATMENT Section 6 of 10

    Author InformationIntroductionClinicalDifferentialsWorkupTreatmentMedicationFollow-upPicturesBibliography

    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

    o 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.

    o 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.

    o Artificial tears: Use these during waking hours to replace diminished

    or absent lacrimation.

    o 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.

    o 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.

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    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 causeprofound and varied metabolic effects. Modify the body's immune response to diverse

    stimuli.

    Drug Name

    Prednisone (Deltasone, Orasone, Sterapred) --

    Pharmacologic success may be the result of anti-

    inflammatory effect, which presumably decreasescompression of the facial nerve in the facial canal.

    Adult Dose 1 mg/kg/d PO for 7 d

    Pediatric Dose Administer as in adults

    Contraindications

    Documented 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

    Pregnancy B - Usually safe but benefits must outweigh the risks.

    Precautions

    Abrupt 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

    MEDICATION Section 7 of 10

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

    appropriate.

    Drug Name

    Acyclovir (Zovirax) -- Has demonstrated inhibitory

    activity directed against both HSV-1 and HSV-2, and

    infected cells selectively take it up.

    Adult Dose 4000 mg/24 h PO for 7-10 d

    Pediatric Dose2 years: 1000 mg PO divided qid for 10 d

    Contraindications Documented hypersensitivity

    InteractionsConcomitant use of probenecid or zidovudine prolongs

    half-life and increases CNS toxicity of acyclovir

    Pregnancy

    C - Safety for use during pregnancy has not been

    established.

    Precautions Caution in renal failure or when using nephrotoxic drugs

    FOLLOW-UP Section 8 of 10

    Author InformationIntroductionClinicalDifferentialsWorkupTreatmentMedicationFollow-upPicturesBibliography

    In/Out Patient Meds:

    Consider prednisone at an initial dose of 1 mg/kg/day.

    o

    Prednisone is a potent drug with a high risk of side effects. Theevidence 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.

    o 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 presumedcause 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

    o The largest portion of the facial nerve comprises efferent fibers thatstimulate muscles of facial expression. If the motor portion achieves

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    suboptimal regeneration, paresis of all or some of these facial muscles

    results.

    o This manifests as (1) oral incompetence, (2) epiphora (excessive

    tearing), and (3) nasal obstruction.

    Incomplete sensory regeneration

    o Dysgeusia (impairment of taste) may result.

    o Ageusia (loss of taste) may result.

    o Dysesthesia (impairment of sensation or disagreeable sensation to

    normal stimuli) may result.

    Aberrant reinnervation of the facial nerve

    o 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.

    o 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.

    o Group 1 regains complete recovery of facial motor function without

    sequelae.

    o Group 2 experiences incomplete recovery of facial motor function, but

    no cosmetic defects are apparent to the untrained eye.

    o 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:

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    Eye care

    o Protect the eye from foreign objects and sunlight.

    o Keep the eye well lubricated.

    o 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

    Author InformationIntroductionClinicalDifferentialsWorkupTreatmentMedicationFollow-upPicturesBibliography

    Caption: Picture 1. The facial nerve.

    View Full Size Image

    eMedicine Zoom View (Interactive!)

    Picture Type: Image

    BIBLIOGRAPHY Section 10 of 10

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