Management of Lower Motor Neurone Facial Nerve Palsy
Flowchart
Introduction

Facial nerve palsy occurs in around 25 children per 100,00{Rowlands, 2002 #34}0 per year (1). Bell’s Palsy (idiopathic lower motor neurone facial nerve) palsy occurs in 1 in 60 people in their lifetime and is a diagnosis of exclusion.  
 
The proximity of the New Forest increases the prevalence of Lyme’s disease in this region.  The prevalence of Lyme disease has a bimodal distribution with peaks at 5-9 years and 45-59 years (2). 
 
A 5-year service evaluation conducted at University Hospital Southampton found 42.5% of Lyme serology tests were positive in children presenting with lower motor neurone facial nerve palsy (3). 
 
The peak in the paediatric age group and high local prevalence has implications for the appropriate management of lower motor neurone facial nerve palsy locally and greater consideration has to be given to Lyme disease as an underlying cause of facial nerve palsy. 
 
The 5-year service evaluation at Southampton noted significant variation in management and this guideline hopes to address this issue and provide a guide for standardised management across the region. (3)
Definitions

Bell's Palsy
Also known as idiopathic facial nerve palsy, this refers to a lower motor neurone facial weaknesses of no clear structural cause, i.e. is a diagnosis of exclusion
Pathogenesis

Bell’s Palsy is an idiopathic lower motor neuron facial nerve palsy and is a diagnosis of exclusion. 
 
In this region, Lyme disease should be high on the list of differential causes; the pathogenesis has been postulated to involve direct infiltration and nerve damage by the bacterium (4). 
 
Other causes identified in a USA study include herpes zoster virus (4%), varicella (6%), acute otitis media 12%) and coxsackie virus (2%) (5). It is important to consider malignancy and this has been identified as a cause of facial paralysis in up to 12% of cases (5). Idiopathic facial nerve palsy is a diagnosis of exclusion and prevalence varies between 9-50% of paediatric cases (5).
Diagnosis

History
  • Thorough history to include: onset and progression of facial palsy. Commonly unilateral facial weakness is observed and occurs acutely. Forehead sparing should NOT occur as this suggests upper motor neurone pathology and therefore a central cause. Patients with upper motor neurone signs need full neurological assessment and appropriate cranial imaging.
  • Less commonly: mild pain in or behind ear, facial numbness, hearing impairment or hyperacusis, disturbed taste, dry eyes. 
  • Any recent infections, tic exposure or bites (NB lack of history of tick exposure/bite does not exclude Lyme) or history of trauma. 
  • Ask about pain, weight loss or any other systemic symptoms. 
 
Examination
  • Thorough examination including faces, eyes, ENT, cranial nerves, peripheral nervous system, joints and skin. Remember to check a BP. 
  • Ensure there are no ‘red flag’ features on history or examination.

Red Flags
Forehead sparing (i.e. UMN lesion) or other abnormal neurology
Neurological examination should be otherwise entirely normal in Bell’s palsy. 
Look for signs of intracranial lesion. 
Loss of corneal reflex may indicate a very proximal lesion. 
Middle ear infection, effusion, hearing loss, vertigo, ear discharge
Look for vesicles – Ramsey-Hunt syndrome
Children with acute otitis media and facial palsy – refer to ENT
Consider more serious ENT pathology such as cholesteatoma - discuss with ENT
Parotid mass
Consider referral to ENT
Bilateral Palsy
Consider Guillain Barre or multiple sclerosis. 
Discuss with paediatric neurology
Severe Pain
Consider Ramsay Hunt syndrome and herpes zoster infection.  Vesicles not always present but pain is a feature. 
Bruising or organomegaly  ​
Consider oncological diagnoses
Hypertension
Can cause facial palsy and has been a presenting feature of coarctation of the aorta in case reports
Investigations
  • FBC and film
  • Lyme Serology
  • Consider CT or MRI if any red flag or atypical features
Management

Eye care 
  • Clinitas multi or hyloforte drops up to hourly if required:
  • VitA Pos ointment at night, and frequently during the day if unable to get drops in, very poor or no closure, or ointment preferred (as forms a protective barrier for longer)
  • If no closure and corneal exposure, use moist chambers or tape lid closed at night if tolerated
  • Ophthalmology follow up in 1-2 weeks – d/w local opthalmology
 
Antibiotics
 
  • Under 9 years 
    • Oral amoxicillin
    • 33 Kg and over: oral amoxicillin 1 gram 3 times/day for 21 days
    • Under 33 Kg: oral amoxicillin 30 mg/Kg three times/day for 21 days
 
  • Under 9 years if penicillin allergy
    • Azithromycin 10mg/kg (max 500mg) once a day for 17 days (gives high dose 21 day therapy as per NICE guideline)
 
  • Age 9-12 years:
    • Oral doxycycline*: 
    • Over or equal to 45 kg: Oral doxycycline 100mg twice/day for 21 days (or 200 mg once/day for 21 days)
    • Under 45 Kg: Oral doxycycline 5 mg/kg in 2 divided doses on day 1 followed by 2.5 mg/kg daily in 1 or 2 divided doses for a total of 21 days.  
  • NB Doxycycline is only available in 50mg/100mg capsule or 100mg dispersible tablets (with frequent supply problems for dispersible) so rounded prescribing is advised. When prescribing doxycycline remind to use of sunblock and direct bright sunlight avoidance where possible.  
  • Or - Oral amoxicillin (as above), use doxycycline in penicillin allergy.
 
*NICE says “At the time of publication (April 2018), doxycycline did not have a UK marketing authorisation for this indication in children under 12 years and is contraindicated. The use of doxycycline for children aged 9 years and above in infections where doxycycline is considered first line in adult practice is accepted specialist practice. The prescriber should follow relevant professional guidance, taking full responsibility for the decision. Informed consent should be obtained and documented. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.”
 
  • Age 12 years and over:
    • Oral doxycycline 100mg twice/day for 21 days (or 200 mg once/day for 21 days)
    • Alternative is oral amoxicillin 1 gram 3 times/day for 21 days

Steroids
  • Consider Prednisolone 1mg/kg (40mg maximum) for 10 days if patients presents within 72 hours of symptom onset. No need to wean. 2016 Cochrane review (10) highlights benefit in paediatric as well as adult patients. 
 
Antivirals
  • Use if history of herpes in patient or family members.  
  • Evidence is not strong but there may be some benefit in selected cases: The 2015 Cochrane review (11) found low-quality evidence from randomised controlled trials showing a benefit from the combination of antivirals with corticosteroids compared to corticosteroids alone. Corticosteroids alone were more effective than antivirals alone and antivirals plus corticosteroids were more effective than placebo or no treatment. There was no benefit from antivirals alone over placebo. There was moderate-quality evidence that the combination of antivirals and corticosteroids reduced sequelae of Bell's palsy compared with corticosteroids alone.
Referrals

Ophthalmology
Clinically indicated if eye is not fully closing.
 
ENT
Consider if red flags or particular concerns. If acute otitis media present discuss with ENT. If any ear symptoms and < 3 years – refer to ENT.  
 
Neurology
Consider if focal or evolving neurological signs or any other cause for concern. 
 
Paediatric Infectious Diseases
Discuss if any concern or confusion regarding interpretation of Lyme serology 
 
Physiotherapy
Not indicated at diagnosis as most resolve. However if still present at 6 week outpatient follow up, referral to Wessex facial nerve palsy services could be considered. See following link for referral form: http://www.uhs.nhs.uk/OurServices/Brainspineandneuromuscular/TheWessexFacialNerveCentre/ForhealthprofessionalsWhentoreferpatientswithfacialparalysis.aspx
 
Speech & Language Therapy
Consider if difficulties with swallow or communication.
Follow Up and Prognosis

  1. Ensure Lyme serology and other results will be chased up in a timely fashion. If Lyme serology is negative – contact patient/ family to stop antibiotics. Do not stop antibiotics if results indeterminate or equivocal (note that IgM may be negative/IgG positive even in apparently early disease – continue antibiotics if unclear).
  2. Ward review within 7 days to ensure no deterioration. The symptoms should be stable within a few days.  If not, consider an enlarging lesion or alternative diagnosis. At this point, stop antibiotics if Lyme serology is negative and this has not already been done Do not stop antibiotics if results indeterminate or equivocal. 
  3. Outpatient clinic appointment in 3- 6 weeks to review progress.
  4. Subsequent review is subject to the patient’s level of recovery. 

Prognosis is very good with resolution in the majority of children with complete resolution in 2 months in many children and by 6 months in the majority (7,8). 
 
If no recovery at 3 weeks, then alternative diagnoses must be considered.
Scope

This guideline applies to all patients under the age of 18 years presenting with lower motor neurone facial nerve palsy.
 
This guideline is limited to the paediatric population of Wessex due to the prevalence of Lyme’s disease in the New Forest, which can present as facial weakness and can be effectively treated with appropriate antibiotics.
Purpose

As a condition Lower Motor Neurone Facial Nerve Palsy is often idiopathic or secondary to Lyme disease and, in these cases, associated with a very good prognosis. 
 
Therefore, this guideline aims to ensure management of paediatric lower motor neurone facial nerve palsy is as consistent as possible with appropriate supportive treatments e.g. eye-care initiated.  It also aims to ensure that more sinister diagnoses are not overlooked; treatable aetiologies are promptly identified and managed.
Implementation

Training and dissemination via the Wessex Neurology Network, the Paediatric ED group and the PIER website. 
Process for Monitoring Effectiveness 

Effectiveness and adherence to the guideline will be monitored by regional audit of practice and ED group audits. 
References
  1. Rowlands S, Hooper R, Hughes R. The epidemiology and treatment of Bell's palsy in the UK. Eur J Neurol2002;9:63-67.
  2. Esposito S, Bosis S, Sabatini C, Tagliaferri L, Principi N. Borrelia Burgdorferi infection and Lyme disease in children. International Journal of Infectious Diseases. 2013. Vol 17 (3); 153-158.
  3. Steed D, Pryde K. G340(P) Paediatric Bell’s palsy: A 5 year review of cases presenting to a large UK teaching hospital. Arch Dis Child 2016: A198-A199.
  4. Christen H, Bartlau N, Hanefeld F. Peripheral facial palsy in childhood. Lyme borreliosis to be suspected unless proven otherwise. Acta Paediatr Scand 1990;79:1219-24.
  5. Cook S, Macartney K, Rose C, Hunt P, Eppes S, Reilly J. Lyme Disease and Seventh Nerve Paralysis in Children. American Journal of Otolaryngology. 1997. Vol 18 (5); 320-323
  6. Grundfast K, Guarisco J, Thomsen J. Diverse etiologies of facial paralysis in children. Int J Pediatr Otorhinolaryngol. 1990;19:223-39.
  7. Ashtekar , Joishy M, Joshi R. Do we need to give steroids in children with Bell's palsy? Emerg Med J. 2005 Jul; 22(7): 505–507.
  8. Hughes GB. Practical management of Bell’s palsy. Otolaryngology Head Neck Surgery 1990;102:658–63.
  9. Lockhart P, Daly F, Pitkethly M, Comerford N, Sullivan F. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2009 Oct 7; (4).
  10. Madhok VB, Gagyor I, Daly F, Somasundara D, Sullivan M, Gammie F, Sullivan F. Corticosteroids for Bell's palsy (idiopathic facial paralysis). Cochrane Database of Systematic Reviews 2016, Issue 7. Art. No.: CD001942. DOI: 10.1002/14651858.CD001942.pub5
  11. Finsterer J.  Management of peripheral facial nerve palsy. Eur Arch Otorhinolaryngology. 2008 Jul; 265(7): 743–752.
  12. Gagyor I, Madhok VB, Daly F, Somasundara D, Sullivan M, Gammie F, Sullivan F. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database of Systematic Reviews 2015, Issue 11. Art. No.: CD001869. DOI: 10.1002/14651858.CD001869.pub8
Document Version: 
2.0

Lead Author: 
Kate Pryde, Paediatric Consultant

Additional Author:
Neeraj Bhangu, Paediatric Registrar
Saul Faust, Paediatric Infectious Disease Consultant
Approving Network:
Wessex Neurosciences Clinical Network

Date of Approval: 
07/2020

Review Date:
07/2023