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Bells palsy
Search date February 2006 Julian Holland

Neurological disorders

QUESTIONS What are the effects of treatments in adults and children? . . . . . . . . . . . . . . . . . . . . . . .2 INTERVENTIONS TREATMENTS Likely to be beneficial Corticosteroids plus antiviral treatment . . . .4 Unknown effectiveness Antiviral treatment . . . . . . . . . . . . . . . . . .3 Corticosteroids . . . . . . . . . . . . . . . . . . . .2 Key Messages Bells palsy is characterised by unilateral, acute partial or acute paralysis of the face, which may occur with mild pain, numbness, increased sensitivity to noise and altered taste. Up to 30% of people with acute peripheral facial palsy have other, identifiable causes, including stroke, tumours, middle ear disease or Lyme disease. Severe pain is more consistent with Ramsay Hunt syndrome due to herpes zoster infection, which has a worse prognosis than Bells palsy. Bells palsy is most common in people aged 15-40 years, and pregnant women may be at higher risk. Bells palsy may be caused by reactivation of herpes viruses in the cranial nerve ganglia. Most people make a spontaneous recovery within 3 weeks but up to 30% may have residual problems. We dont know whether corticosteroids or antiviral treatment improve recovery of motor function or cosmetically disabling sequelae compared with placebo or with other treatments. Combined treatment with aciclovir plus corticosteroids may be more effective than steroids alone. Aciclovir should only be prescribed in pregnancy under guidance of an obstetrician. Valaciclovir may be an acceptable alternative to aciclovir treatment, but we dont have any good quality research to show that it is effective. We dont know whether facial nerve decompression surgery is beneficial in Bells palsy as no studies of adequate quality have been found. Facial nerve decompression surgery . . . . . .4 See glossary

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Clin Evid 2006;15:12.

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Neurological disorders

Bells palsy
DEFINITION Bells palsy is an idiopathic, acute, unilateral paresis or paralysis of the face in a pattern consistent with peripheral facial nerve dysfunction, and may be partial or complete, occurring with equal frequency on the right and left sides of the face. While other possible causes need to be excluded, there is increasing evidence that Bells palsy is caused by herpes viruses.1 Additional symptoms of Bells palsy may include mild pain in or behind the ear, oropharyngeal or facial numbness, impaired tolerance to ordinary levels of noise, and disturbed taste on the anterior part of the tongue.2 Severe pain is more suggestive of herpes zoster virus infection (shingles) and possible progression to a Ramsay Hunt syndrome, but another cause should be carefully excluded. Up to 30% of people with an acute peripheral facial palsy will not have Bells palsy; other causes may include stroke, tumour, trauma, middle ear disease and Lyme disease. Features such as sparing of movement in the upper face (central pattern) or weakness of a specific branch of the facial nerve (segmental pattern) suggest an alternative cause.3 In children under 10 years of age, Bells palsy is less common (under 40%) so an alternative cause should be carefully excluded.4 The assessment should identify acute suppurative ear disease (including mastoiditis), a parotid mass or Lyme disease in endemic areas.5 The incidence is about 20/100 000 people a year, or about 1/60 people in a lifetime.4 Bells palsy has a peak incidence between the ages of 15 and 40 years. Men and women are equally affected, although the incidence may be increased in pregnant women.4 Bells palsy is probably caused by reactivated herpes viruses from the cranial nerve ganglia. Herpes simplex virus-1 may be detected in up to 50% of cases and herpes zoster virus in approximately 30% of cases. Herpes zoster associated facial palsy more frequently presents as zoster sine herpete (without vesicles), although 6% of patients will subsequently develop vesicles (Ramsay Hunt syndrome).6 Thus, treatment plans for the management of Bells palsy should recognise the high incidence of herpes zoster virus, which is associated with worse outcomes.7 Inflammation of the facial nerve initially results in reversible neuropraxia, but ultimately Wallerian degeneration ensues. Overall, Bells palsy has a fair prognosis without treatment. Significant improvement occurs within 3 weeks in 85% of people and within 35 months in the remaining 15%.4 Patients failing to show signs of improvement by 3 weeks may have suffered significant degeneration of the facial nerve or have an alternative diagnosis that requires identification by specialist examination or investigations, such as computed tomography or magnetic resonance imaging. Overall, 71% of people will recover facial muscle function (61% of people with complete palsy, 94% of people with partial palsy).4 The remaining 29% are left with mild to severe residual facial muscle weakness, 17% with contracture and 16% with hemifacial spasm or synkinesis.4 Incomplete recovery of facial expression may have a long term impact on quality of life. The prognosis for children with Bell's palsy is generally good, with a high rate (> 90%) of spontaneous recovery, in part due to the high frequency of partial paralysis.4 However, children with complete palsies may suffer poor outcomes as frequently as do adults.8

INCIDENCE/ PREVALENCE

AETIOLOGY/ RISK FACTORS

PROGNOSIS

AIMS OF To prevent patients progressing from partial to complete facial palsies; to maximise the rate of INTERVENTION recovery; to increase the proportion of patients making a full recovery; to reduce the incidence of motor synkinesis and contracture; to avoid morbidity to the eye, with minimum adverse effects. OUTCOMES Grade of recovery of motor function of the face; presence of sequelae (motor synkinesis, autonomic dysfunction, hemifacial spasm); time to full recovery. Clinical Evidence search and appraisal February 2006. We performed a broad search for RCTs of any type of corticosteroid, antiviral agent, or corticosteroid plus antiviral agent in adults and children with Bell's palsy, and included any RCT of sufficient quality. Trials used different scoring systems for reporting outcomes.

METHODS

QUESTION OPTION

What are the effects of treatments in adults and children? CORTICOSTEROIDS

One systematic review found no significant difference between corticosteroids (cortisone acetate, prednisolone, methylprednisolone) and control in recovery of facial motor function or reduction of cosmetically disabling sequelae after 6 months. One RCT identified by the review found that prednisolone reduced the time to recover facial nerve function compared with placebo. However, the review found no significant difference between treatments in motor synkinesis and autonomic dysfunction at 12 months. One RCT identified by a systematic review found that prednisolone produced full recovery more often than aciclovir 2
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alone, but found no significant difference in motor synkinesis between treatment groups at 12 weeks. One RCT identified by a systematic review found limited evidence that aciclovir plus prednisolone produced full recovery of facial function more often compared with prednisolone alone after 4 months. Benefits: Corticosteroids versus placebo or no specific treatment: We found one systematic review (search date 2003, 4 RCTs, 179 people).9 The first RCT identified by the review (26 people aged 1276 years) compared cortisone acetate versus placebo; the second RCT (51 people, ages not specified) compared prednisolone plus vitamins versus vitamins alone; the third RCT (42 children aged 2474 months) compared methylprednisolone versus no treatment; and the fourth RCT (62 people aged 1584 years) compared prednisolone plus vitamins versus saline solution plus vitamins. The review found no significant difference between corticosteroids (cortisone acetate, prednisolone, methylprednisolone) and control in the proportion of people with incomplete recovery of facial motor function after 6 months (3 RCTs: 13/59 [22%] of people with corticosteroid v 15/58 [26%] of people with control; RR 0.86, 95% CI 0.47 to 1.59). When data from two quasi-randomised trials found by the review10,11 were added to the pooled estimate, the result remained non-significant (RR 0.69, 95% CI 0.42 to 1.16; absolute numbers not reported; see comment below). The review also found no significant difference between corticosteroid and control in the proportion of people with cosmetically disabling sequelae after 6 months (3 RCTs, 117 people: 8/59 [14%] of people with corticosteroid v 9/58 [16%] of people with control; RR 0.86, 95% CI 0.38 to 1.98). When data from two quasi-randomised trials found by the review10,11 were added to the pooled estimate, the result remained non significant (RR 0.82, 95% CI 0.39 to 1.73; absolute numbers not reported). The fourth RCT identified by the review found that prednisolone significantly reduced the time to recover facial nerve function compared with placebo (18.8 days with prednisolone v 35.6 days with control; P = 0.005).12 It also found that prednisolone increased full recovery compared with placebo at 12 months, although the significance of this difference was not reported (83% with prednisolone v 75% with control). The review found no significant difference between treatments in motor synkinesis and autonomic dysfunction at 12 months (5/32 [16%] with prednisolone v 7/30 [24%] with control; RR 0.67, 95% CI 0.24 to 1.88).9 Corticosteroids versus aciclovir: See benefits of antiviral treatment, p 3. Corticosteroids plus antiviral treatment versus either treatment alone: See benefits of corticosteroids plus antiviral treatment, p 4. Corticosteroids versus placebo or no specific treatment: One RCT identified by the review reported temporary sleep disturbances with prednisolone (3/32 [9.4%] with prednisolone; results for placebo group not reported).9,12 The side effects of steroid treatment are well documented, including diabetes, hypertension, glaucoma, psychosis, fluid and electrolyte disturbances, gastrointestinal tract haemorrhage, and aseptic necrosis of the hip.15 The high incidence of abnormal glucose tolerance in patients with Bells palsy warrants special caution. Corticosteroids versus placebo or no specific treatment: Of the two quasirandomised trials identified by the review, one compared corticosteroids (preparation not stated) versus supportive therapy only, and used alternation in matched participants as the method of allocation.9,10 The other compared dexamethasone versus placebo, and used allocation according to the day of admission.9,11 ANTIVIRAL TREATMENT

Neurological disorders

Harms:

Comment:

OPTION

One systematic review identified no RCTs comparing aciclovir versus placebo. One RCT identified by the review found that prednisolone produced full recovery more often than aciclovir alone, but found no significant difference in motor synkinesis between treatment groups at 12 weeks. Another RCT identified by the review found limited evidence that aciclovir plus prednisolone produced full recovery of facial function more often compared with prednisolone alone after 4 months. Benefits: Antiviral treatment versus placebo: We found one systematic review (search date 2003), which found no RCTs comparing aciclovir versus placebo.14 Antiviral treatment versus corticosteroids: We found one systematic review (search date 2003, 1 RCT, 3

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Bells palsy
101 people), which identified one RCT comparing aciclovir (2400 mg per day for 10 days) versus prednisolone (1 mg/kg for 10 days then tapered to zero over the next 6 days).14 The RCT found that prednisolone produced full recovery significantly more often compared with aciclovir alone at 12 weeks (partial recovery: 12/54 [22%] with aciclovir v 3/47 [6%] with prednisolone; RR 3.48, 95% CI 1.05 to 11.1). However, it found no significant difference in motor synkinesis between treatment groups (motor synkinesis: 13/54 [24%] with aciclovir v 11/47 [23%] with prednisolone; RR 1.03, 95% CI 0.51 to 2.07).14 Antiviral treatment plus corticosteroids versus either treatment alone: See benefits of corticosteroids plus antiviral treatment, p 4. Harms: Antiviral treatment versus corticosteroids: The RCT identified by the review did not report any adverse events.14 Aciclovir treatment at doses up to 4000 mg/day in divided doses results in nausea and diarrhoea in up to 4% of people.15 The systematic review identified three further studies of aciclovir, which were not of sufficient quality for inclusion.14 In the RCT identified by the review, the 10 day duration of administration of aciclovir was shorter than the 16 days of prednisolone, which may have biased the study against aciclovir.14 Aciclovir should only be prescribed in pregnancy under the guidance of an obstetrician. Aciclovir requires five times daily dosing and demonstrates poorer bioavailability than valaciclovir (a prodrug of aciclovir),16 which has shown greater effectiveness in the management of shingles. CORTICOSTEROIDS PLUS ANTIVIRAL TREATMENT

Comment:

OPTION

One RCT identified by a systematic review found limited evidence that aciclovir plus prednisolone produced full recovery of facial function more often compared with prednisolone alone after 4 months. Benefits: Corticosteroids plus antiviral treatment versus placebo or no treatment: We found no systematic review or RCTs (see comment). Corticosteroids plus antiviral treatment versus either treatment alone: We found one systematic review (search date 2003, 1 RCT, 119 people), which identified one RCT comparing aciclovir (400 mg 5 times daily for 10 days) plus prednisolone versus prednisolone alone (see comment below).14 It found that after 4 months, aciclovir plus prednisolone produced full recovery of facial function significantly more often compared with prednisolone alone (moderate or moderately severe dysfunction measured using a facial function scoring system: 4/53 [8%] of people with aciclovir plus prednisolone v 11/46 [24%] with prednisolone alone; RR 0.32, 95% CI 0.11 to 0.92; see comment below).14 In the RCT, 20/119 (17%) of people enrolled in the trial were lost to follow up. It is not clear to which treatment group these belonged. Results were calculated from the 99/119 (83%) people who completed the trial. Corticosteroids plus antiviral treatment versus either treatment alone: The RCT identified by the review reported mild to moderate gastrointestinal complaints, which did not require treatment. No numbers were reported.14 Aciclovir should only be prescribed in pregnancy under the guidance of an obstetrician. Aciclovir requires five times daily dosing and demonstrates poorer bioavailability than valaciclovir (a prodrug of aciclovir),16 which has shown greater effectiveness in the management of shingles. Corticosteroids plus antiviral treatment versus placebo or no treatment: One additional observational study (56 people) comparing valaciclovir plus prednisolone versus no medical treatment found that valaciclovir plus prednisolone increased complete recovery compared with no medical treatment in people older than 60 years (complete recovery: AR 10/10 [100%] with valaciclovir plus prednisolone v 5/12 [42%] with no medical treatment, P < 0.01).17 FACIAL NERVE DECOMPRESSION SURGERY

Harms:

Comment:

OPTION

One systematic review identified no RCTs of facial nerve decompression surgery for people with Bells palsy. 4
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Bells palsy
Benefits: Harms: We found one systematic review (search date 2000), which found no RCTs of facial nerve decompression surgery for people with Bells palsy.18 The systematic review found reports of permanent unilateral deafness in four nonrandomised prospective studies of facial nerve decompression in people with Bells palsy.18 One study of people with complete facial palsy undergoing facial nerve decompression found that 4/41 (10%) people had conductive deafness and 2/41 (5%) people had sensory-neural deafness after 1 year.10 We found one multicentre, prospective, non-randomised, observational study, which compared total facial nerve decompression surgery versus no surgery.13 All participants were treated with prednisolone and were offered surgery if serial electrophysiological testing showed severe nerve degeneration within 2 weeks of the onset of their palsy. The study found that there were significantly more complete recoveries with surgery plus prednisolone compared with prednisolone alone (31/34 [91%] with surgery plus prednisolone v 15/33 [42%] with prednisolone alone; P = 0.0002). Two people who had decompression surgery reported adverse effects; one person reported conductive hearing loss, and one person experienced a cerebrospinal fluid leak. No other adverse effects for either group were reported. The study had significant recruitment problems and the numbers are clearly small; surgery was only performed in specialised centres.

Neurological disorders

Comment:

GLOSSARY
Autonomic dysfunction occurs when aberrant regeneration of the facial nerve causes inappropriate autonomic activation with facial motor activity, for example crocodile tears tearing with chewing. Contracture An abnormal, often permanent, shortening of the facial muscles resulting in facial distortion or deformity. Hemifacial spasm is a generalised involuntary mass contracture of the facial muscles. Motor synkinesis is caused by aberrant regeneration of the facial nerve which causes synchronous movements of different parts of the face for example, dimpling of the chin occurring simultaneously with each blink. Neuropraxia is reversible nerve dysfunction without the degeneration or loss of nerve axons. Ramsay Hunt syndrome is characterised by acute facial paralysis with herpetic (herpes zoster virus) blisters of the skin of the ear canal or tongue. Other symptoms may include vertigo, ipsilateral hearing loss, and tinnitus. Wallerian degeneration describes the sequelae of axonal injury and subsequent removal of axonal and myelin debris by Schwann cells and invading macrophages.

REFERENCES
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Neurological disorders

Bells palsy
Julian Holland Department of Otolaryngology Head and Neck Surgery St Michaels Hospital Bristol UK
Competing interests: None declared.

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