Bell's palsy

Bell's palsy

A person attempting to show his teeth and raise his eyebrows

with Bell's palsy on his right side.

Bell's palsy or idiopathic facial paralysis[1] is a dysfunction of cranial nerve VII (the facial nerve)
that results in inability to control facial muscles on the affected side. Several conditions can cause a
facial paralysis, e.g., brain tumor, stroke, and Lyme disease. However, if no specific cause can be
identified, the condition is known as Bell's palsy. Named after Scottish anatomist Charles Bell, who
first described it, Bell's palsy is the most common acute mononeuropathy (disease involving only
one nerve) and is the most common cause of acute facial nerve paralysis.

Bell's palsy is defined as an idiopathic unilateral facial nerve paralysis, usually self-limiting. The
trademark is rapid onset of partial or complete palsy, usually in a single day. It can occur bilaterally
resulting in total facial paralysis in around 1% of cases.[2]

It is thought that an inflammatory condition leads to swelling of the facial nerve. The nerve travels
through the skull in a narrow bone canal beneath the ear. Nerve swelling and compression in the
narrow bone canal are thought to lead to nerve inhibition, damage or death. No readily identifiable
cause for Bell's palsy has been found.

Corticosteroids have been found to improve outcomes while anti-viral drugs have not.[3] Early
treatment is necessary for steroids to be effective. Most people recover spontaneously and achieve
near-normal to normal functions. Many show signs of improvement as early as 10 days after the
onset, even without treatment.

Often the eye in the affected side cannot be closed. The eye must be protected from drying up, or
the cornea may be permanently damaged resulting in impaired vision. In some cases denture
wearers experience some discomfort.
Signs and symptoms

Facial nerve: the facial nerve's nuclei are in the brainstem (they are represented in the diagram as a
„θ“). Orange: nerves coming from the left hemisphere of the brain.　Yellow: nerves coming from
the right hemisphere of the brain. Note that the forehead muscles receive innervation from both
hemispheres of the brain (represented in yellow and orange).

Bell's palsy is characterized by facial drooping on the affected half, due to malfunction of the facial
nerve (VII cranial nerve), which controls the muscles of the face. Facial palsy is typified by
inability to control movement in the facial muscles. The paralysis is of the infranuclear/lower motor
neuron type.

The facial nerves control a number of functions, such as blinking and closing the eyes, smiling,
frowning, lacrimation, salivation, flairing nostriles and raising eyebrows. They also innervate the
stapedial (stapes) muscles of the middle ear and carry taste sensations from the anterior two thirds
of the tongue.

Clinicians should determine whether the forehead muscles are spared. Due to an anatomical
peculiarity, forehead muscles receive innervation from both sides of the brain. The forehead can
therefore still be wrinkled by a patient whose facial palsy is caused by a problem in one of the
hemispheres of the brain (central facial palsy). If the problem resides in the facial nerve itself
(peripheral palsy) all nerve signals are lost on the ipsilateral (same side of the lesion) half side of the
face, including to the forehead (contralateral forehead still wrinkles).

One disease that may be difficult to exclude in the differential diagnosis is involvement of the facial
nerve in infections with the herpes zoster virus. The major differences in this condition are the
presence of small blisters, or vesicles, on the external ear and hearing disturbances, but these
findings may occasionally be lacking (zoster sine herpete).

Lyme disease may produce the typical palsy, and may be easily diagnosed by looking for Lyme-
specific antibodies in the blood or erythema migrans. In endemic areas Lyme disease may be the
most common cause of facial palsy.

The degree of nerve damage can be assessed using the House-Brackmann score.
Although defined as a mononeuritis (involving only one nerve), patients diagnosed with Bell’s
palsy may have "myriad neurological symptoms" including "facial tingling, moderate or severe
headache/neck pain, memory problems, balance problems, ipsilateral limb paresthesias, ipsilateral
limb weakness, and a sense of clumsiness" that are "unexplained by facial nerve dysfunction".[4]
This is yet an enigmatic facet of this condition.

Cause
Some viruses are thought to establish a persistent (or latent) infection without symptoms, e.g. the
Zoster virus of the face[5] and Epstein-Barr viruses, both of the herpes family. Reactivation of an
existing (dormant) viral infection has been suggested[6] as cause behind the acute Bell's palsy.
Studies[7] suggest that this new activation could be preceded by trauma, environmental factors, and
metabolic or emotional disorders, thus suggesting that stress - emotional stress, environmental
stress (e.g. cold), physical stress (e.g. trauma) - in short, a host of different conditions, may trigger
reactivation.

Pathology

Anatomy of the face.

It is thought that as a result of inflammation of the facial nerve, pressure is produced on the nerve
where it exits the skull within its bony canal, blocking the transmission of neural signals or
damaging the nerve. Patients with facial palsy for which an underlying cause can be found are not
considered to have Bell's palsy per se. Possible causes include tumor, meningitis, stroke, diabetes
mellitus, head trauma and inflammatory diseases of the cranial nerves (sarcoidosis, brucellosis,
etc.). In these conditions, the neurologic findings are rarely restricted to the facial nerve. Babies can
be born with facial palsy.[8] In a few cases, bilateral facial palsy has been associated with acute HIV
infection.

In some research[9] the herpes simplex virus type 1 (HSV-1) was identified in a majority of cases
diagnosed as Bell's palsy. This has given hope for anti-inflammatory and anti-viral drug therapy
(prednisone and acyclovir). Other research[6] however, identifies HSV-1 in only 31 cases (18
percent), herpes zoster (zoster sine herpete) in 45 cases (26 percent) in a total of 176 cases clinically
diagnosed as Bell's Palsy. That infection with herpes simplex virus should play a major role in
cases diagnosed as Bell's palsy therefore remains a hypothesis that requires further research.

In addition, the herpes simplex virus type 1 (HSV-1) infection is associated with demyelination of
nerves. This nerve damage mechanism is different from the above mentioned - that oedema,
swelling and compression of the nerve in the narrow bone canal is responsible for nerve damage.
Demyelination may not even be directly caused by the virus, but by an unknown immune system
response. The quote below captures this hypothesis and the implication for other types of treatment:

It is also possible that HSV-1 replication itself is not responsible for the damage to the facial nerves
and that inhibition of HSV-1 replication by acyclovir does not prevent the progression of nerve
dysfunction. Because the demyelination of facial nerves caused by HSV-1 reactivation, via an
unknown immune response, is implicated in the pathogenesis of HSV-1-induced facial palsy, a new
strategy of treatment to inhibit such an immune reaction may be also effective.[6]

Diagnosis
Bell's palsy is a diagnosis of exclusion; by elimination of other reasonable possibilities. Therefore,
by definition, no specific cause can be ascertained. Bell's palsy is commonly referred to as
idiopathic or cryptogenic, meaning that it is due to unknown causes. Being a residual diagnostic
category, the Bell's Palsy diagnosis likely spans different conditions that our current level of
medical knowledge cannot distinguish. This may inject fundamental uncertainty into the discussion
below of etiology, treatment options, recovery patterns etc. See also the section below on Other
symptoms. Studies[4] show that a large number of patients (45%) are not referred to a specialist,
which suggests that Bell’s palsy is considered by physicians to be a straightforward diagnosis that is
easy to manage. A significant number of cases are misdiagnosed (ibid.). This is unsurprising from a
diagnosis of exclusion, which depends on a thorough investigation.

Treatment
In patients presenting with incomplete facial palsy, where the prognosis for recovery is very good,
treatment may be unnecessary. Patients presenting with complete paralysis, marked by an inability
to close the eyes and mouth on the involved side, are usually treated, some of them with smile
surgery. Early treatment (within 3 days after the onset) is necessary for therapy to be effective.[10]
Steroids have been shown to be effective at improving recovery while antivirals have not.

Steroids

Corticosteroid such as prednisone significantly improves recovery at 6 months and are thus
recommended.[1]

Antivirals

Antivirals (such as acyclovir) are ineffective in improving recovery from Bell's palsy beyond
steroids alone.[11] They were however commonly prescribed due to a theoretical link between Bell's
palsy and the herpes simplex and varicella zoster virus.[3]

Surgery
Surgery may be able to improve outcomes in facial nerve palsy that has not recovered.[12] A number
of different techniques exist.[12] Smile surgery or smile reconstruction is a surgical procedure that
restores the smile for people with facial nerve paralysis.

Complementary therapy

The efficacy of acupuncture remains unknown because the available studies are of low quality (poor
primary study design or inadequate reporting practices).[13]

Prognosis
Even without any treatment, Bell's palsy tends to carry a good prognosis. In a 1982 study, when no
treatment was available, of 1,011 patients, 85% showed first signs of recovery within 3 weeks after
onset. For the other 15%, recovery occurred 3–6 months later. After a follow-up of at least 1 year or
until restoration, complete recovery had occurred in more than two thirds (71%) of all patients.
Recovery was judged moderate in 12% and poor in only 4% of patients.[14] Another study found that
incomplete palsies disappear entirely, nearly always in the course of one month. The patients who
regain movement within the first two weeks nearly always remit entirely. When remission does not
occur until the third week or later, a significantly greater part of the patients develop sequelae.[15] A
third study found a better prognosis for young patients, aged below 10 years old, while the patients
over 61 years old presented a worse prognosis.[7]

Major complications of the condition are chronic loss of taste (ageusia), chronic facial spasm, facial
pain and corneal infections. To prevent the latter, the eyes may be protected by covers, or taped shut
during sleep and for rest periods, and tear-like eye drops or eye ointments may be recommended,
especially for cases with complete paralysis. Where the eye does not close completely, the blink
reflex is also affected, and care must be taken to protect the eye from injury.

Another complication can occur in case of incomplete or erroneous regeneration of the damaged
facial nerve. The nerve can be thought of as a bundle of smaller individual nerve connections that
branch out to their proper destinations. During regrowth, nerves are generally able to track the
original path to the right destination - but some nerves may sidetrack leading to a condition known
as synkinesis. For instance, regrowth of nerves controlling muscles attached to the eye may
sidetrack and also regrow connections reaching the muscles of the mouth. In this way, movement of
one also affects the other. For example, when the person closes the eye, the corner of the mouth lifts
involuntarily.

In addition, around 6%[citation needed] of patients exhibit crocodile tear syndrome, also called
gustatolacrimal reflex or Bogorad’s Syndrome, on recovery, where they will shed tears while
eating. This is thought to be due to faulty regeneration of the facial nerve, a branch of which
controls the lacrimal and salivary glands. Gustatorial sweating can also occur.

Epidemiology
The annual incidence of Bell's palsy is about 20 per 100,000 population, and the incidence increases
with age.[16] Bell’s palsy affects about 40,000 people in the United States every year. It affects
approximately 1 person in 65 during a lifetime.[citation needed] Familial inheritance has been found in 4–
14% of cases.[17] Bell's Palsy is three times more likely to strike pregnant women than non-pregnant
women.[18] It is also considered to be four times more likely to occur in diabetics than the general
population.[19]
A range of annual incidence rates have been reported in the literature: 15,[17] 24,[20] and 25-53[4] (all
rates per 100,000 population per year). Bell’s palsy is not a reportable disease, and there are no
established registries for patients with this diagnosis, which complicates precise estimation.

History
This lesion was described by Sir Charles Bell, a Scottish surgeon. In 1829 he presented three cases
at the Royal Society of London. Two cases were idiopathic and the third was due to a tumour of the
parotid gland.

References
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http://en.wikipedia.org/wiki/Bell%27s_palsy