Bell's palsy

Bell's Palsy is a paralysis of the facial nerve resulting in inability to control facial muscles in 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 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 is thought that an inflammatory condition leads to swelling of the facial nerve (nervus facialis). 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 palsy has been found, but clinical and experimental evidence suggests herpes simplex type 1 infection may play a role.

Doctors may prescribe anti-inflammatory and anti-viral drugs. Early treatment is necessary for the drug therapy to have effect. The effect of treatment is still controversial. Most people recover spontaneously and achieve near-normal functions. Many show signs of improvement as early as 10 days after the onset, even without treatment.

Protect the eye. 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.

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Investigation

Bell's palsy (or facial 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, and salivation. 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 all branches of the facial nerve are involved, or whether the forehead muscles are spared. Since forehead muscles receive innervation from both sides of the brain, the forehead can still be wrinkled by a patient whose facial palsy is caused by a problem in the brain (central facial palsy) but not if the problems resides in the facial nerve itself (peripheral palsy).

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, of 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. In endemic areas Lyme disease may be the most common cause of facial palsy.

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. This medical parlance simply means that causes are unknown. Being a residual diagnostic category, the Bell's Palsy diagnosis likely spans different conditions which our current level of medical knowledge cannot distinguish. This may inject fundamental uncertainty into the discussion below of etiology, treatment options, recovery patterns etc, if they refer to a hodgepodge of different conditions. See also the section below on Other symptoms. Studies^[1] 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 is critically dependent on thoroughness.

Pathology

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, and they exhibit many of the same symptoms as people with Bell's palsy; this is often due to a traumatic birth which causes irreparable damage to the facial nerve, i.e. acute facial nerve paralysis.

In some research^[2] 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^[3] 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.

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 effective. ^[4]

Virus reactivation

Some vira are thought to establish a persistent (or latent) infection without symptoms, e.g. Epstein-Barr virus of the herpes family. Reactivation of an existing (dormant) viral infection has been suggested^[5] as cause behind the acute Bell's palsy. Studies^[6] 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.

Other symptoms

Some Bell’s palsy patients are reported ^[7]to have myriad neurological symptoms that are unexplained by facial nerve dysfunction. They include facial tingling, moderate or severe headache/neck pain, memory problems, balance problems, ipsilateral limb paresthesias, ipsilateral limb weakness, and a sense of clumsiness. This is yet an enigmatic facet of this condition.

Alternative medicine

In traditional Chinese medicine, considered an alternative medicine in the West, Bell's palsy is attributed to a "wind cold" attack brought about by exposure to wind.^[8]

Epidemiology

The annual incidence of Bell palsy is about 20 per 100,000 population, and the incidence increases with age^[9]. 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^[10]. Bell's Palsy is three times more likely to strike pregnant women than non-pregnant women.^[11] It is also considered to be four times more likely to occur in diabetics than the general population,^[12]

An annual incidence rate of 'about' 20 is often reported. Slightly different values are reported in the literature: 15^[13], 24^[14] , and 25-53^[15] (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.

Treatment

Treatment is a matter of controversy. In patients presenting with incomplete facial palsy, where the prognosis for recovery is very good, treatment may be unnecessary. However, patients presenting with complete paralysis, marked by an inability to close the eyes and mouth on the involved side, are usually treated with anti-inflammatory corticosteroids. Prednisolone, a corticosteroid, if used early in treatment of Bell's palsy, significantly^{[citation needed]} improves the chances of complete recovery at 3 and 9 months when compared to treatment with acyclovir, an anti-viral drug, or no treatment at all. The likely association of Bell's palsy with the herpes virus has led most American neurologists to prescribe a course of anti-viral medication (such as acyclovir) to all patients with unexplained facial palsy, although this large study showed no additional benefit from acyclovir beyond that from prednisolone alone.^[16] Surgical procedures to decompress the facial nerve have been attempted, but have not been proven beneficial. Acupuncture has also been studied, with inconclusive results.^[17]

A practice parameter from the American Academy of Neurology states that "corticosteroids are safe and probably effective, and that acyclovir is safe and possibly effective". ^[18]

People who think they may have Bell's Palsy should consult their doctor as soon as possible. Many times, the medications will not be effective if administered too late after the onset. Ideally, corticosteroids should be started within 72 hours of the onset of weakness.

Recovery

Even without any treatment, Bell's palsy tends to carry a good prognosis. In a study^[19] 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. Another study^[20] finds 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. A third study^[21] found a better prognosis for young patients, aged below 10 years old, while the patients over 61 years old presented the worst prognosis.

Complications

Major complications of the condition are chronic loss of taste (ageusia), chronic facial spasm 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 reflex is also affected; great care should 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 which 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 will lift, or when smiling, the eye will close (synkinesis).

In addition, around 6%.^{[citation needed]} of patients exhibit crocodile tear 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.

Famous persons with Bell's palsy

Well-known persons affected by Bell's palsy include:

• Stevie Benton, Bassist for Texas rock band, Drowning Pool^[22]
• Pierce Brosnan, Irish actor and producer^[23]
• Jean Chrétien, former prime minister of Canada^[24]
• George Clooney, American actor, director, producer and screenwriter^[25]
• Alexis Denisof, American actor.^[26]
• Graeme Garden, British comedy writer and performer, who has written about his experiences with the condition^[27]
• Tony Gonzalez, American NFL football player.^[28]
• Amy Goodman, American journalist and author.^[29]
• Katie Holmes, American Actress and star of First Daughter.
• Ralph Kiner, American baseball player in the 1940s and 1950s^[30]
• Curtis LeMay, was a general in the United States Air Force and the vice presidential running mate of independent candidate George C. Wallace in 1968 ^[31]
• Danny Louis, musician and member of Gov't Mule.^[32]
• Ralph Nader, American author, activist, politician^[23]
• Jim Ross, professional wrestling announcer for WWE^[33]
• Rick Savage, British musician and bassist for Def Leppard^[23]
• Jamey Sheridan, American actor. His condition was written into the show Law and Order: Criminal Intent and his character Captain James Deakins also had Bell's palsy. ^[34]
• Joseph C. Wilson, American diplomat.^[29]

References