Definitions

bell-palsy

Bell's palsy

Bell's palsy is a paralysis of the facial nerve resulting 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 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's 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 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.

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 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, including to the forehead.

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.

The degree of nerve damage can be assessed using the House-Brackman Score.

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 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. See also the section below on Other symptoms. Studies 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.

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. In a few cases, bilateral facial palsy has been associated with acute HIV infection.

In some research 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 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 effective.

Virus reactivation

Some viruses are thought to establish a persistent (or latent) infection without symptoms, e.g. the Zoster virus of the face and Epstein-Barr viruses, both of the herpes family. Reactivation of an existing (dormant) viral infection has been suggested as cause behind the acute Bell's palsy. Studies 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

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". This is yet an enigmatic facet of this condition.

Alternative medicine

In traditional Chinese medicine, Bell's palsy is attributed to exposure to wind - more specifically due to wind-cold attacking the Shaoyang (liver, gall bladder) and Yangming (stomach, large intestine) channels as well as the tendons and muscles. This is thought to result in an obstruction of Qi (vital energy) and blood in these areas that leads to malnourishment of the tendons and muscles and thus a propensity for the facial muscles to become lax or paralyzed Formal studies of the effects of acupuncture on Bell's Palsy are inconclusive (see below, Treatment).

Epidemiology

The annual incidence of Bell palsy is about 20 per 100,000 population, and the incidence increases with age. Bell’s palsy affects about 40,000 people in the United States every year. It affects approximately 1 person in 65 during a lifetime. Familial inheritance has been found in 4–14% of cases. Bell's Palsy is three times more likely to strike pregnant women than non-pregnant women. It is also considered to be four times more likely to occur in diabetics than the general population.

A range of annual incidence rates have been reported in the literature: 15, 24, and 25-53 (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 of Bell's palsy is a matter of controversy. Two Cochrane reviews from 2004 underlined the need for larger, properly designed clinical trials to evaluate antiviral drugs or corticosteroids for Bell's palsy. The effect of treatment is difficult to evaluate experimentally because spontaneous recovery (without any treatment) is common. 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. Early treatment (within 3 days after the onset) seems to be necessary for therapy to be effective.

Prednisolone, a corticosteroid, if used early in treatment of Bell's palsy, significantly improves the chances of complete recovery at 3 and 9 months when compared to treatment with the anti-viral drug acyclovir or no treatment at all.

The possible link between Bell's palsy and the herpes simplex and varicella zoster virus has led to the prescription of anti-viral medications (such as acyclovir or valaciclovir) to patients with unexplained facial palsy. Recently (2007), a large randomized clinical trial reported no additional benefit from acyclovir beyond that from prednisolone alone.

The efficacy of acupuncture remains unknown because the available studies are of low quality (poor primary study design or inadequate reporting practices). Surgical procedures to decompress the facial nerve have been attempted, but have not been proven beneficial.

A 2005 practice parameter from the American Academy of Neurology states that "corticosteroids are safe and probably effective, and that acyclovir is safe and possibly effective".

Physiotherapy is also a vital part of Bell's palsy since it is a nerologic condition. Facial kabat techniques and criostimulation along with exercises based on facial mimicry have shown good results in clinical practice.

Recovery

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. Another study 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 found a better prognosis for young patients, aged below 10 years old, while the patients over 61 years old presented a worse 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 lifts involuntarily.

In addition, around 6% 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.

See also

References

External links

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