Practice Essentials

Bell palsy, also termed idiopathic facial paralysis (IFP), is the most common
cause of unilateral facial paralysis. It is one of the most common neurologic
disorders of the cranial nerves (see the image below). In the great majority of
cases, Bell palsy gradually resolves over time, and its cause is unknown.
Signs and symptoms
Signs and symptoms of Bell palsy include the following:
 Acute onset of unilateral upper and lower facial paralysis (over a 48-hr
period)
 Posterior auricular pain
 Decreased tearing
 Hyperacusis
 Taste disturbances
 Otalgia
 Weakness of the facial muscles
 Poor eyelid closure
 Aching of the ear or mastoid
 Tingling or numbness of the cheek/mouth
 Epiphora
 Ocular pain
 Blurred vision
 Flattening of forehead and nasolabial fold on the side affected by palsy
 When patient raises eyebrows, palsy-affected side of forehead remains
flat
 When patient smiles, face becomes distorted and lateralizes to side
opposite the palsy
See Clinical Presentation for more specific information on the signs and
symptoms of Bell palsy.
Diagnosis
Examination for Bell palsy includes the following:
 Otologic examination: Pneumatic otoscopy and tuning fork examination,
particularly if evidence of acute or chronic otitis media
 Ocular examination: Patient often unable to completely close eye on
affected side
 Oral examination: Taste and salivation often affected
 Neurologic examination: All cranial nerves, sensory and motor testing,
cerebellar testing
Grading
The grading system developed by House and Brackmann categorizes Bell
palsy on a scale of I to VI, [3, 4, 5] as follows:
Grade I: normal facial function
Grade II: mild dysfunction
Grade III: moderate dysfunction
Grade IV: moderately severe dysfunction
Grade V: severe dysfunction
Grade VI: total paralysis
See Clinical Presentation for more specific information on patient history and
physical examination for Bell palsy.
Testing
Although there are no specific diagnostic tests for Bell palsy, the following
may be useful for identifying or excluding other disorders:
 Rapid plasma reagin and/or venereal disease research laboratory test or
fluorescent treponemal antibody absorption test
 HIV screening by enzyme-linked immunosorbent assay and/or Western
blot
 Complete blood count
 Erythrocyte sedimentation rate
 Thyroid function
 Serum glucose
 CSF analysis
 Blood glucose
 Hemoglobin A1c
 Antineutrophil cytoplasmic antibody levels
 Salivary flow
 Schirmer blotting test
 Nerve excitability test
 Computed tomography
 Magnetic resonance imaging
See Workup for more specific information on testing and imaging modalities
for Bell palsy.
Management
Goals of treatment: (1) improve facial nerve (seventh cranial nerve) function;
(2) reduce neuronal damage; (3) prevent complications from corneal exposure
Treatment includes the following:
 Corticosteroid therapy (prednisone) [6, 7]
 Antiviral agents [6, 8]
 Eye care: Topical ocular lubrication is usually sufficient to prevent corneal
drying, abrasion, and ulcers [9]
Surgical options
Surgical treatment options include the following:
 Facial nerve decompression
 Subocularis oculi fat lift
 Implantable devices (eg, gold weights) placed into the eyelid
 Tarsorrhaphy
 Transposition of the temporalis muscle
 Facial nerve grafting
 Direct brow lift
See Treatment and Medication for more specific information regarding
pharmacologic and other therapies for Bell palsy.

Background
Bell palsy, more appropriately termed idiopathic facial paralysis (IFP), is the
most common cause of unilateral facial paralysis. Bell palsy is an acute,
unilateral, peripheral, lower-motor-neuron facial nerve paralysis that gradually
resolves over time in 80-90% of cases.
Controversy surrounds the etiology and treatment of Bell palsy. The cause of
Bell palsy remains unknown, though the disorder appears to be a polyneuritis
with possible viral, inflammatory, autoimmune, and ischemic etiologies.
Increasing evidence implicates herpes simplex type I and herpes zoster virus
reactivation from cranial-nerve ganglia. [10] (See Etiology.)
Bell palsy is one of the most common neurologic disorders affecting the
cranial nerves, and it is the most common cause of facial paralysis worldwide.
It is thought to account for approximately 60-75% of cases of acute unilateral
facial paralysis. Bell palsy is more common in adults, in people with diabetes,
and in pregnant women. (See Epidemiology.)
Diagnosis
Determining whether facial nerve paralysis is peripheral or central is a key
step in the diagnosis. A lesion involving the central motor neurons above the
level of the facial nucleus in the pons causes weakness of the lower face
alone. Thorough history taking and examination, including the ears, nose,
throat, and cranial nerves, must be performed. (See Presentation.)
The minimum diagnostic criteria include paralysis or paresis of all muscle
groups on one side of the face, sudden onset, and absence of central nervous
system (CNS) disease. Note that the diagnosis of IFP can be made only after
other causes of acute peripheral palsy have been excluded. (See DDx.)
If the clinical findings are doubtful or if paralysis lasts longer than 6-8 weeks,
further investigations, including gadolinium-enhanced magnetic resonance
imaging (MRI) of the temporal bones and pons, should be
considered. [11] Electrodiagnostic tests (eg, stapedius reflex test, evoked facial
nerve electromyography [EMG], audiography) may help to improve the
accuracy of prognosis in difficult cases. (See Workup.)
Treatment
Treatment of Bell palsy should be conservative and guided by the severity and
probable prognosis in each particular case. Studies have shown the benefit of
high-dose corticosteroids for acute cases. [12, 13] Although antiviral treatment
has also come into use, evidence is now available indicating that it may not be
beneficial. [12](See Treatment and Medication.)
Topical ocular therapy is useful in most cases, with the exception of those in
which the condition is severe or prolonged. In these cases, surgical
management is best. Several procedures are aimed at protecting the cornea
from exposure and achieving facial symmetry. These procedures reduce the
need for constant use of lubrication drops or ointments, may improve
cosmesis, and may be needed to preserve vision on the affected side. (See
Treatment.)
Patient education
To prevent corneal abrasions, patients should be instructed about eye care.
They also should be encouraged to do facial muscle exercises using passive
range of motion, as well as actively close their eyes and smile.
For patient education information, see the Brain and Nervous System Center,
as well as Bell’s Palsy.

Anatomy
In 1550, Fallopius noted the narrow foramen in the temporal bone through
which a part of the seventh cranial nerve (facial nerve) passes; this feature is
now sometimes called the fallopian canal or the facial canal. 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 that the fifth nerve primarily conducted sensation from the face.
The facial nerve contains parasympathetic fibers to the nose, palate, and
lacrimal glands. Its course is tortuous, both centrally and peripherally. The
facial nerve travels a 30-mm intraosseous course through the internal auditory
canal (with the eighth cranial nerve) and through the internal fallopian canal in
the petrous temporal bone. This bony confinement limits the amount that the
nerve can swell before it becomes compressed.
The nucleus of the facial nerve 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 preganglionic parasympathetic
fibers that originate in the salivatory nucleus join the fibers from nucleus
solitarius to form the nervus intermedius.
The nervus intermedius is composed of sensory fibers from the tongue,
mucosa, and postauricular skin, as well as parasympathetic fibers to the
salivary and lacrimal glands. 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 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 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 the
frontalis, orbicularis oculi, orbicularis oris, buccinator, and platysma muscles.
Other muscles innervated by the facial nerve include the stapedius,
stylohyoid, posterior belly of the digastric, occipitalis, and anterior and
posterior auricular muscles. All muscles innervated by the facial nerve are
derived from the second branchial arch. See the images below.

The facial nerve.

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The facial nerve.

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Pathophysiology
The precise pathophysiology of Bell palsy remains an area of debate. The
facial nerve courses through a portion of the temporal bone commonly
referred to as the facial canal. A popular theory proposes that edema and
ischemia result in compression of the facial nerve within this bony canal. The
cause of the edema and ischemia has not yet been established. This
compression has been seen in MRI scans with facial nerve enhancement. [14]
The first portion of the facial canal, the labyrinthine segment, is the narrowest;
the meatal foramen in this segment has a diameter of only about 0.66 mm.
This is the location that is thought to be the most common site of compression
of the facial nerve in Bell palsy. Given the tight confines of the facial canal, it
seems logical that inflammatory, demyelinating, ischemic, or compressive
processes may impair neural conduction at this site.
Injury to the facial nerve in Bell palsy is peripheral to the nerve’s 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.
Etiology
Herpes simplex virus
In the past, situations that produced cold exposure (eg, chilly wind, cold air
conditioning, or driving with the car window down) were considered to be the
only triggers for Bell palsy. Several authors now believe, however, that the
herpes simplex virus (HSV) is a common cause of Bell palsy, though a
definitive causal relationship of HSV to Bell palsy may be difficult to prove
because of the ubiquitous nature of HSV.
The hypothesis that HSV is the etiologic agent in Bell palsy holds that after
causing primary infection on the lips (ie, cold sores), the virus travels up the
axons of the sensory nerves and resides in the geniculate ganglion. At times
of stress, the virus reactivates and causes local damage to the myelin.
This hypothesis was first suggested in 1972 by McCormick. [15] Autopsy
studies have since shown HSV in the geniculate ganglion of patients with Bell
palsy. Murakami et al performed polymerase chain reaction (PCR) assay
testing on the endoneural fluid of the facial nerve in patients who
underwent surgery for Bell palsy and found HSV in 11 of 14 cases. [16]
Additional support for a viral etiology was seen when intranasal, inactivated
influenza vaccine was strongly linked to the development of Bell
palsy. [17, 18] With those cases, however, it is not clear whether another
component of the vaccine caused the paresis, which was then accompanied
by a reactivation of HSV infection.
Additional causes
Besides HSV infection, possible etiologies for Bell palsy include other
infections (eg, herpes zoster, Lyme disease, syphilis, Epstein-Barr viral
infection, cytomegalovirus, human immunodeficiency virus [HIV],
mycoplasma); inflammation alone; and microvascular disease (diabetes
mellitus and hypertension). Bell palsy has also been known to follow recent
upper respiratory infection (URI). [19, 20, 21, 22,23, 24]
Bell palsy may be secondary to viral and/or autoimmune reactions that cause
the facial nerve to demyelinate, resulting in unilateral facial paralysis.
A family history of Bell palsy has been reported in approximately 4% of cases.
Inheritance in such cases may be autosomal dominant with low penetration;
however, which predisposing factors are inherited is unclear. [25] The family
history may also be positive for other nerve, nerve root, or plexus disorders
(eg, trigeminal neuralgia) in siblings. [26] In addition, there are isolated reports
of familial Bell palsy with neurologic deficits, including ophthalmoplegia [27] and
essential tremor. [28] A rare form of familial Bell palsy has a predilection for
juvenile females. [29]
Because there is a strong environmental predisposition to Bell palsy, due to
the common viral etiology, a positive family history may or may not indicate a
true genetic etiology.

Epidemiology
In the United States, the annual incidence of Bell palsy is approximately 23
cases per 100,000 persons. [7] Very few cases are observed during the
summer months. 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
cases per 100,000 population.
Bell palsy is thought to account for approximately 60-75% of cases of acute
unilateral facial paralysis, with the right side affected 63% of the time. It can
also be recurrent, with a reported recurrence range of 4-14%. [20]
Though bilateral simultaneous Bell palsy can develop, it is rare. It accounts for
only 23% of bilateral facial paralysis and has an occurrence rate that is less
than 1% of that for unilateral facial nerve palsy. [30, 31] The majority of patients
with bilateral facial palsy have Guillain-Barré syndrome, sarcoidosis, Lyme
disease, meningitis (neoplastic or infectious), or bilateral neurofibromas (in
patients with neurofibromatosis type 2).
Persons with diabetes have a 29% higher risk of being affected by Bell palsy
than do persons without diabetes. Thus, measuring blood glucose levels at
the time of diagnosis of Bell palsy may detect undiagnosed diabetes. 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. [32]
Bell palsy is also more common in people who are immunocompromised or in
women with preeclampsia. [33]
Sex- and age-related demographics
Bell palsy appears to affect the sexes equally. However, young women aged
10-19 years are more likely to be affected than are men in the same age
group. Pregnant women have a 3.3 times higher risk of being affected by Bell
palsy than do nonpregnant women; Bell palsy occurs most frequently in the
third trimester.
In general, Bell palsy occurs more commonly in adults. A slightly higher
predominance is observed in patients older than 65 years (59 cases per
100,000 people), and a lower incidence rate is observed in children younger
than 13 years (13 cases per 100,000 people). The lowest incidence is found in
persons younger than 10 years, and the highest incidence is in persons aged
60 years or older. Peak ages are between 20 and 40 years. The disease also
occurs in elderly persons aged 70-80 years. [34]

Prognosis
The natural course of Bell palsy varies from early complete recovery to
substantial nerve injury with permanent sequelae (eg, persistent paralysis and
synkinesis). Prognostically, patients fall into 3 groups:
 Group 1 - Complete recovery of facial motor function without sequelae
 Group 2 - Incomplete recovery of facial motor function, but with no
cosmetic defects that are apparent to the untrained eye
 Group 3 - Permanent neurologic sequelae that are cosmetically and
clinically apparent
Approximately 80-90% of patients with Bell palsy recover without noticeable
disfigurement within 6 weeks to 3 months. Use of the Sunnybrook grading
scale for facial nerve function at 1 month has been suggested as a means of
predicting probability of recovery. [35]
Most 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 with 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 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/or
long-term sequelae.
The sooner the recovery, the less likely are the chances that sequelae will
develop, as summarized below:
 If some restoration of function is noted within 3 weeks, then the recovery
is most likely to be complete
 If the recovery begins between 3 weeks and 2 months, then the ultimate
outcome is usually satisfactory
 If the recovery does not begin until 2-4 months from the onset, likelihood
of permanent sequelae, including residual paresis and synkinesis, is
higher
  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
Bell palsy recurs in 4-14% of patients, with one source suggesting a
recurrence rate of 7%. It may recur on the same or opposite side of the initial
palsy. Recurrence usually is associated with a family history of recurrent Bell
palsy. Higher recurrence rates among patients were reported in the past;
however, many of these patients were found to have an underlying etiology for
the recurrence, eliminating the diagnosis of Bell palsy, an idiopathic
disease. [36]
Patients with recurrent ipsilateral facial palsy should undergo MRI or high-
resolution computed tomography (CT) scanning to rule out a neoplastic or
inflammatory (eg, multiple sclerosis, sarcoidosis) cause of recurrence.
Recurrent or bilateral disease should suggest myasthenia gravis.
Sequelae
Most patients with Bell palsy recover without any cosmetically obvious
deformities. Approximately 30% of patients, however, experience long-term
symptoms following the paralysis, and approximately 5% are left with an
unacceptably high degree of sequelae. Bell palsy sequelae include incomplete
motor regeneration, incomplete sensory regeneration, and aberrant
reinnervation of the facial nerve.
Incomplete motor regeneration
The largest portion of the facial nerve is composed of efferent fibers that
stimulate muscles of facial expression. Suboptimal regeneration of this portion
results in paresis of all or some of these facial muscles. This manifests as (1)
oral incompetence, (2) epiphora (excessive tearing), and (3) nasal obstruction.
Incomplete sensory regeneration
Dysgeusia or ageusia (impairment or loss of taste, respectively) may occur
with incomplete regeneration of the chorda tympani. Incomplete regeneration
of other afferent branches may result in dysesthesia (impairment of sensation
or disagreeable sensation to normal stimuli).
Aberrant reinnervation of the facial nerve
During regeneration and repair of the facial nerve, some neural fibers may
take an unusual course and connect to neighboring muscle fibers. This
aberrant reconnection produces unusual neurologic pathways. When
voluntary movements are initiated, they are accompanied by involuntary
movements (eg, eye closure associated with lip pursing or mouth grimacing
that occurs during blinking of the eye). The condition in which involuntary
movements accompany voluntary movements is termed synkinesis.