II. Definitions

  1. Bell's Palsy (Facial Nerve Palsy)
    1. Idiopathic, acute Facial Nerve Paralysis

III. Background: History

  1. Named for Sir Charles Bell (1774-1842) who first described the syndrome

IV. Epidemiology

  1. Incidence: 15-30 per 100,000 per year (45,000 per year in U.S.)
  2. No gender predominance
  3. Ages most commonly affected 15 to 45 years old (peaks at age 40-49 years)

V. Pathophysiology

  1. Facial Nerve edema, compression or inflammation typically at Geniculate Ganglion (risk of ischemia, demyelination)
  2. Associated with Herpesvirus infection in 30% of cases

VI. Risk Factors

  1. Diabetes Mellitus (comorbid in 10% of cases)
  2. Pregnancy (associated with 3 fold increased risk)
  3. Immunosuppression
  4. Influenza A
  5. Herpesvirus infection (comorbid in 30% of cases)
    1. Herpes Simplex Virus
    2. Varicella Zoster Virus
    3. Epstein-Barr Virus

VII. History: Red Flags suggestive of other Facial Nerve Paralysis Cause

  1. Gradual onset over >2 weeks
    1. Suggests mass lesion
    2. Mass lesion may also cause a recurrent unilateral Bell's Palsy
  2. Forehead not involved
    1. Suggests Central Nervous System cause (supranuclear lesion)
    2. Facial Nerve motor nucleus is divided
      1. Dorsal aspect (forehead innervation) and ventral aspect (lower facial innervation)
      2. Both sides of the brain provide input to the dorsal aspect (forehead)
        1. Hence lack of forehead involvement implies an Upper Motor Neuron Lesion
      3. Only one side of the brain provides input to the ventral aspect (lower face)
  3. Bilateral involvement
    1. Suggests autoimmune Polyneuropathy
    2. Lyme Disease
  4. Recent new medications (e.g. Influenza Vaccine)
  5. Acute Unilateral Weakness in other distributions (suggests CNS Lesion)
    1. Extraocular Movement deficits
    2. Unilateral limb weakness
    3. Bulbar weakness
  6. Lyme Disease Risk Factors (Tick Bite, endemic Lyme Disease region during peak season)
    1. Lyme Disease
  7. Fever
    1. Consider infectious cause such as Otitis Media
  8. Rash
    1. Vesicular rash (Herpes Zoster, Ramsay Hunt Syndrome)
    2. Erythema Migrans (Lyme Disease)
  9. Hearing Loss and Vestibular Symptoms or Ataxia
    1. Acoustic Neuroma
  10. Children (esp. children age <5 years)
    1. Otitis Media
    2. Trauma
    3. Herpes Simplex Virus
    4. Varicella Zoster Virus
    5. Lyme Disease
    6. Malignancy (esp. Leukemia, Lymphoma, Brain Tumor)
      1. Associated with a 0.7% risk in children age <5 years (contrast with 0.3% overall)
      2. Evaluate for Hepatosplenomegaly and Lymphadenopathy, and ensure close follow-up
    7. References
      1. Claudius and Walsh (2022) EM:Rap 22(9): 8-9
      2. Walsh (2022) Am J Emerg Med 53:63-7 +PMID:34992025 [PubMed]

VIII. Exam

  1. Head and neck
    1. External Ear and ear canal (e.g. Otitis Externa, Herpes Zoster, Ramsay Hunt Syndrome)
    2. Tympanic Membrane (e.g. Otitis Media)
    3. Mouth and pharynx (e.g. Herpes Simplex Virus)
    4. Parotid Gland
  2. Neurologic Exam
    1. Perform complete Neurologic Exam including gait
    2. Perform extremity Motor Exam and Sensory Exam
    3. Cranial Nerve Exam
    4. Test Cranial Nerve 7 bilaterally on lower face and forehead (forehead MUST be involved in Bell's Palsy)
      1. Raise eyebrows, wrinkling forehead
      2. Close eyes tightly
      3. Frown
      4. Show teeth
      5. Pucker lips
  3. Skin
    1. Vesicular rash (Herpes Zoster, Ramsay Hunt Syndrome)
    2. Erythema Migrans (Lyme Disease)

IX. Symptoms

  1. Idiopathic Facial Nerve Paralysis developing over 1 to 3 days
  2. Associated symptoms or signs
    1. Hypoesthesia or dysesthesia (80%)
      1. Glossopharyngeal Nerve dysfunction
      2. Trigeminal Nerve dysfunction
    2. Facial or retroauricular pain (60%)
    3. Dysgeusia (57%)
    4. Hyperacusis (30%)
    5. Vagal nerve motor weakness (20%)
    6. Decreased Lacrimation (17%)
    7. Trigeminal Nerve motor weakness (3%)
    8. Synkinesis (e.g. mouth twitching while blinking, or winking while smiling)
  3. References
    1. Adour (1982) N Engl J Med 307: 348-51 [PubMed]

X. Signs: General

  1. Preserved facial Sensation
    1. However hypoesthesia or dysesthesia is common (see above)
  2. Mouth and nasolabial changes
    1. Loss of facial creases and flattening of nasolabial fold
    2. Corner of mouth droops
  3. Eye changes
    1. No closure or decreased closure of upper Eyelid
    2. Lower Eyelid sag
    3. Decreased tear production
  4. No furrow over forehead (forehead appears flattened)
    1. Critical to recognize when the forehead and Eyelid are not involved
    2. Lack of forehead and Eyelid involvement suggests an Upper Motor Neuron Lesion such as a CVA

XI. Signs: Facial Nerve Grading (House-Brackman)

  1. Grade 1: Normal Facial Nerve Function
  2. Grade 2: Mild Facial Nerve Dysfunction
    1. Gross
      1. Slight weakness on close examination
      2. Synkinesis slight
    2. Rest: Normal symmetry and tone
    3. Motor Exam
      1. Forehead: Moderate to good function
      2. Eyes: Complete closure with minimum effort
      3. Mouth: Slight asymmetry
  3. Grade 3: Moderate Facial Nerve Dysfunction
    1. Gross:
      1. Obvious difference between sides (not disfiguring)
      2. Synkinesis noticeable
    2. Rest: Normal symmetry and tone
    3. Motor Exam
      1. Forehead: slight to Moderate movement
      2. Eyes: Complete closure with effort
      3. Mouth: Slightly weak with maximal effort
  4. Grade 4: Moderately Severe Facial Nerve Dysfunction
    1. Gross
      1. Obvious weakness
      2. Disfiguring asymmetry
    2. Rest: Normal symmetry and tone
    3. Motor Exam
      1. Forehead: No motor function
      2. Eyes: Incomplete closure
      3. Mouth: Asymmetric with maximal effort
  5. Grade 5: Severe Facial Nerve Dysfunction
    1. Gross: Barely perceptible motion
    2. Rest: Asymmetry
    3. Motor Exam
      1. Forehead: No motor function
      2. Eyes: Incomplete closure
      3. Mouth: Slight movement
  6. Grade 6: Total Facial Nerve Paralysis
  7. References
    1. House (1985) Otolaryngol Head Neck Surg 93:146-7 [PubMed]

XII. Differential Diagnosis

XIII. Labs

  1. Labs are not indicated in isolated peripheral Facial Nerve Paralysis
  2. Serum Glucose is not routinely recommended
    1. Diabetes Mellitus does not cause Bell's Palsy, and is simply a predisposing factor
  3. Lyme Antibody titer is not routinely recommended
    1. Lyme peripheral facial palsy is almost always associated with other findings (e.g. Arthritis, facial swelling, rash)
    2. Isolated Facial Nerve Palsy is not typically due to Lyme Disease
    3. However, consider empiric therapy for Lyme Disease and lyme test with risk factors in endemic regions
      1. Bilateral Facial Nerve involvement (high risk, treat as Lyme Disease while testing)
      2. Known recent deer Tick Bite
      3. Erythema Migrans (diagnostic without Lyme Titer)
      4. Peak season in endemic Lyme Disease region
    4. References
      1. Kuiper (1992) Arch Neurol 49(9): 940-3 [PubMed]

XIV. Imaging: MRI Head With and Without Contrast

  1. Benefits
    1. MRI Identifies central causes (Schwannoma, Hemangioma, meningioma and Cholesteatoma)
    2. MRI offers prognostic information based on nerve contrast enhancement
  2. Indications
    1. Suspected central cause (see Red Flags above)
    2. Persistent or progressive peripheral Facial Nerve Palsy lasting >2 months
    3. Facial twitching or spasm
    4. Recurrent Bell Palsy

XV. Management: Corticosteroids and Antimicrobials

  1. Approach
    1. Start Corticosteroid within 72 hours of onset
    2. Antiviral may be considered in moderate to severe cases (House-Brackman Grade 4 and above)
    3. Consider Lyme Disease management if suggested by history or exam
      1. Doxycycline (preferred) 100 mg bid or Amoxicillin 500 mg tid for 14-21 days
  2. High dose Corticosteroids: Prednisone or Prednisolone (primary intervention)
    1. Adult
      1. Protocol 1: 60 to 80 mg orally daily for 7 days
      2. Protocol 2: 60 mg daily for 5 days, then taper off over 5 days
      3. Worse recover was associated with cummulative Prednisone dosing <450 mg
        1. de Almeida (2009) JAMA 302(9): 985-93 [PubMed]
    2. Child: 2 mg/kg/day (up to adult dosing) for 7 days
    3. NNT 10 for full recovery in Bell Palsy treated with early Corticosteroids (<72 hours from onset)
    4. Salinas (2010) Cochrane Database Syst Rev (3):CD001942 +PMID:20238317 [PubMed]
  3. Antiviral agents (optional)
    1. Mechanism
      1. Based on reactivated HSV hypothesis
    2. Indications
      1. Findings consistent with Herpes Zoster, herpes simplex or Ramsay Hunt Syndrome (e.g. vesicular rash)
      2. Antiviral may be considered in moderate to severe cases (House-Brackman Grade 4 and above)
    3. Efficacy of empiric antiviral use (excluding cases of herpetic, vesicular rashes which should be treated)
      1. Original studies showed synergistic benefit with antivirals in combination with Corticosteroids
        1. More recent studies show primary improvement with Corticosteroids
        2. Only marginal added benefit with antivirals
      2. Reasonable to offer antivirals in moderate to severe cases
        1. However patients should be counseled on low efficacy of antivirals
    4. Agents
      1. Acyclovir
        1. Adult: 400 mg five times per day for 7 days
        2. Child (>2 years): 80 mg/kg daily (max: 3200 mg/day) divided every 6 hours for 5 days
      2. Valacyclovir
        1. Age >12 years: 1 gram orally three times daily for 7 days
    5. References
      1. Gronseth (2012) Neurology 79(22): 2209-13 [PubMed]
      2. Adour (1996) Ann Otol Rhinol Laryngol 105:371-8 [PubMed]
      3. Hato (2007) Otol Neurotol 28: 408-13 [PubMed]
      4. Hato (2003) Otol Neurotol 24: 948-51 [PubMed]
  4. Other antimcrobial considerations
    1. Consider empiric Doxycycline in Lyme Disease endemic regions (esp. bilateral, peak tick season, known Tick Bite)

XVI. Management: Loss of Blink Reflex

  1. Rewetting the eye
    1. Frequent use of preservative-free artificial tears (every 15 to 30 minutes)
    2. Refresh PM ointment six times daily
  2. Protective glasses with side pieces
    1. Use in outdoors, drafty, dusty areas
    2. Alternatively can use eye shield or cup
  3. Avoid grinding, sanding, or sawing
  4. At night:
    1. Apply bland ointment (Refresh PM, Lacri-Lube)
    2. Tape eye shut
  5. Ophthalmology Consultation indicated for incomplete Eyelid closure persisting for weeks
    1. Risk of Keratitis, Corneal Ulcers and permanent ocular injury from dry, unprotected eye

XVII. Management: Associated Conditions

  1. Otitis Media or Mastoiditis Complications
    1. IV antibiotics
    2. Otolaryngology Consultation for possible wide incision of Tympanic Membrane
  2. Herpes Zoster Oticus (Ramsay Hunt Syndrome)
    1. See Herpes Zoster for antiviral agents
    2. May be associated with Tinnitus and Hearing Loss
    3. High dose Corticosteroids (1 mg/kg/day)
      1. Avoid in Diabetes Mellitus, Peptic Ulcer, Glaucoma

XVIII. Management: Referral Indications

  1. Otitis Media complications
  2. Mastoiditis complications
  3. Signs of secondary cause
    1. Intracranial lesion or nerve impingement
  4. Incomplete Eyelid closure persisting for weeks
    1. Risk of permanent ocular injury from drying
    2. Referral to ophthalmology for management beyond artificial tears
  5. Other procedure referrals NOT routinely recommended
    1. Facial Nerve decompression surgery (may rarely be indicated)
    2. Laser Therapy
    3. Hyperbaric oxygen
    4. Intratympanic Corticosteroid Injection
    5. Stellate Ganglion block
    6. Physical Therapy (no evidence of benefit in Bell's Palsy)
      1. May consider for >3 month of Grade 5-6 findings

XIX. Prognosis: Factors associated with poor prognosis

  1. Worse Prognosis with time needed for recovery
    1. No recovery by 3 weeks suggests worse prognosis (15% of cases)
    2. Further recovery occurs over 3-5 months
  2. Hyperacusis
  3. Diabetes Mellitus
  4. Hypertension
  5. Pregnancy
  6. Facial Nerve with severe degeneration by EMG
  7. Decreased tearing
  8. Age over 60 years
  9. Ramsay Hunt Syndrome (Herpes ZosterVirus)
  10. Severe pain
    1. Aural pain
    2. Anterior facial pain
    3. Radicular pain

XX. Complications

  1. Corneal Ulceration or Keratitis (due to incomplete Eyelid closure)
  2. Permanent Eyelid weakness
  3. Permanent facial asymmetry (e.g. impaired smiling)
    1. May be trigger social anxiety, depressed mood and other related complications
  4. Synkinesis (up to 26% of patients at one year from onset)
    1. Misdirected nerve regrowth leads to co-contraction of unrelated Muscles innervated by CN7
    2. Example: Blinking make occur with smiling

XXI. Prognosis

  1. Early full recovery (66 to 85%) within 3 weeks (higher rates after 8 weeks)
    1. Children age <14 years and pregnant women have full recovery in 90% of cases
  2. Prolonged recovery (15%) over 3-5 months (higher risk with bilateral or severe Bell's Palsy)
    1. Slight residual deficit: 12%
    2. Mild residual deficit: 13%
    3. Severe residual deficit: 4%
      1. Facial weakness
      2. Contracture or spasm
  3. Recurrence: 6.5 to 8% of cases (mean interval 10 years)
    1. Higher risk of recurrence in Diabetes Mellitus
    2. Complete recovery in 66% of recurrent cases

XXII. Course

  1. Maximal weakness at 3-7 days after onset
  2. Most cases (85%) improve within 3 weeks even without treatment
    1. Additional improvement may require up to 5 months
    2. Prolonged recovery duration required for nerve regeneration

XXIII. References

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