The "Bell's smile" is characterized by an asymmetry caused by paralysis of one side of the face.

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Bell's palsy

Bell's palsy (facial palsy) is characterised by facial drooping on the affected half, due to malfunction of the facial nerve (VII cranial nerve), which controls the muscles of the face. 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. The paralysis is of the infranuclear/lower motor neuron type. Bell’s palsy affects about 40,000 people in the United States every year. It affects approximately 1 person in 65 during a lifetime. Until recently, its cause was unknown in most cases, but it has now been related to both Lyme disease and Herpes simplex. more...

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Symptoms

Additional symptoms that may accompany the condition are pain around the ear and loss of taste. In the great majority of patients, only one side of the face is affected. Detection of sensory loss, hearing loss, or ataxia during examination militates against the diagnosis of Bell's palsy and suggests the need for further evaluation.

Investigation

Clinicians should determine whether all branches of the facial nerve are involved, or whether the forehead muscles are spared. Since these 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 rather than in the facial nerve itself.

Diagnosis

Bell's palsy is a diagnosis of exclusion; in many cases, no specific cause can be ascertained.

Pathology

It is supposed to be the result of inflammation of the facial nerve, which produces pressure on the nerve as it exits the skull within its bony canal. Patients with facial palsy for which an underlying cause can be readily found are not generally considered to have Bell's palsy per se. These underlying problems 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 irrepairable damage to the facial nerve, i.e.acute facial nerve paralysis.

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.

In recent years, two new suspects have been added to the possible causes of Bell's palsy. 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 subsequent observation of an increased incidence of antibodies to the Herpes simplex virus in patients with Bell's palsy has led many specialists to believe that this agent is the most likely underlying cause in areas where Lyme disease is uncommon.

Epidemiology

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, and it is more common in the elderly than children .

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How should we manage Bell's palsy?
From Journal of Family Practice, 10/1/04 by Keith B. Holten

Recommendations for these management issues are found in the guideline that was funded and developed by the American Academy of Neurology. Their Quality Standards Subcommittee, Practice Committee, and Board of Directors approved the recommendations. The target audience is physicians.

Patients with Bell's palsy are the target population of this guideline. The objective is to summarize evidence regarding effectiveness of steroids, acyclovir, or surgical facial nerve decompression for improved functional outcomes in facial nerve palsy (Bell's palsy). The evidence categories for this guideline are therapeutic effectiveness and treatment. Outcomes considered are 1) relative rate and 95% confidence interval for good return of facial function, and 2) relative rate and 95% confidence interval for complete return of facial function. The rating scheme is updated to comply with the SORT taxonomy. (1)

* GUIDELINE RELEVANCE AND LIMITATIONS

Bell's palsy results from damage to the 7th (facial) cranial nerve and affects 40,000 Americans each year. It is seen commonly in pregnant women and diabetics, as well as those with viral illnesses. Besides facial paralysis, other symptoms of Bell's palsy may include pain, hypersensitivity to sound in the affected ear, and impairment of taste. The common cold sore viruses, herpes simplex virus, and other herpes viruses are the likely pathogens causing many cases of Bell's palsy. The prognosis for Bell's palsy is good and most patients get better within 2 weeks. Over 80% recover facial nerve function within 3 months. (2)

A lengthy bibliography accompanies the guideline. The guideline is weakened by lack of a cost-effectiveness analysis.

* GUIDELINE DEVELOPMENT AND EVIDENCE REVIEW

The authors searched the National Library of Medicine's Medline database from 1966 to June 2000. The resultant prospective studies for treatments with steroids, acyclovir, or surgery were screened for outcome evidence. There are 25 references. A meta-analysis of patient data and a systematic review of the evidence were performed. Quality and strength of evidence were weighted according to a rating scheme.

* SOURCE FOR THIS GUIDELINE

Grogan PM, Gronseth GS. Practice parameter: Steroids, acyclovir, and surgery for Bell's palsy (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2001; 56:830-836.

* OTHER GUIDELINES ON BELL'S PALSY

* Assessment: Neurologic risk of immunization Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.

This older guideline refers to a few cases of Bell's palsy associated with the plasma-derived hepatitis B vaccine used from 1982 to 1988. Since then the recombinant product has replaced the plasma-derived vaccine.

Source: Fenichel GM. Assessment: Neurologic risk of immunization: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 1999; 52:1546-1552. [30 references]

REFERENCES

(1.) Ebell M, Siwek J, Weiss BD, et al. Strength of recommendation taxonomy (SORT): A patient-centered approach to grading evidence in the medical literature. Am Fam Physician 2004; 69:548-556.

(2.) NINDS Bell's Palsy Information Page. National Institute of Neurological Disorders and Stroke. April 2003. Access at: www.ninds.nih.gov/ health_and_medical/disorders/ bells_doc.htm. Accessed on August 6, 2004.

PRACTICE RECOMMENDATIONS

Grade B Recommendations

* Treatment with oral corticosteroids improves facial function.

* Treatment with acyclovir, combined with steroids, improves facial function.

Grade C Recommendations

* Facial nerve decompression does not improve facial function.

* Do steroids change the course of Bell's palsy?

* What is the role of surgery for Bell's palsy?

* Should antiviral therapy be initiated for all patients?

Keith B. Holten, MD

University of Cincinnati College of Medicine, Cincinnati, Ohio

Correspondence: Keith B. Holten, MD, Clinton Memorial Hospital/University of Cincinnati Family Practice Residency, 825 W. Locust St., Wilmington, OH, 45177. E-mail: keholtenmd@cmhregional.com.

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