Brain & Development 27 (2005) 504508 www.elsevier.

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Original article

Prognosis of Bells palsy in childrenanalysis of 29 cases

Wen-Xiong Chen, Virginia Wong*
Division of Neurodevelopmental Paediatrics, Department of Paediatrics and Adolescent Medicine, Faculty of Medicine, Queen Mary Hospital, The University of Hong Kong, Pokfulamg, Hong Kong SAR, China Received 10 November 2004; received in revised form 4 January 2005; accepted 6 January 2005

Abstract We report 29 children with 32 episodes of Bells palsy admitted to a university afliated hospital during an 8-year period (19952003). The peak age of onset was under 3 years. Three (10.3%) had recurrent attacks. Complete recovery occurred in all 32 episodes except 1 (3.1%) with partial recovery, having MRI evidence of parotitis shown in the contralateral side. The recovery rate within 3 weeks was 68.8%. There was statistically signicant increase in the duration of complete recovery for those with positive virological conrmation or mycoplasma infection. There was no signicant difference between the rate of recovery in those treated with a short course of steroid (NZ23 attacks) than those without steroid treatment (NZ9 attacks). As there were few studies in the natural course of children with Bells palsy, evidence based trials should be done to assess the natural course rather than giving steroid empirically as those with more protracted recovery might be viral in origin. q 2005 Elsevier B.V. All rights reserved.
Keywords: Facial nerve; Bells palsy; Recurrent Bells palsy; Virus infection; Mycoplasma infection; Children; Steroid

1. Introduction Bells palsy is the most common disease affecting the facial nerve. Bells palsy is dened as an idiopathic peripheral facial nerve paralysis of sudden onset and is considered the most common cause of facial nerve paralysis. The incidence of Bells palsy is 1540 per 100,000 [1]. Complete recovery occurs when the function of the facial nerve occurs between days 10 and 21 after the onset. A satisfactory outcome is also possible if recovery occurs between 3 weeks and 2 months. Recurrent Bells palsy is uncommon. This occurs in 29% of Bells palsy in adults [2,3]. More than two recurrences of Bells palsy are unusual. Pitts [2] reviewed 1700 cases of Bells palsy and identied only two patients with four episodes of recurrent Bells palsy. Yanagihara [4] identied only one out of 2414 cases with more than four recurrences. A case with 11 episodes of Bells palsy was also reported [7]. Reports of children with recurrent Bells palsy are even rarer. One study reported the incidence of recurrent Bells
* Corresponding author. Tel.: C852 2855 4485; fax: C852 2855 1523. E-mail address: vcnwong@hkucc.hku.hk (V. Wong).

palsy in children to be 6% [10]. Facial nerve palsy in children can result from Lyme disease, varicella, primary gingivo-stomatitis, herpes zoster oticus (Ramsay Hunt syndrome), coxsackie virus, EpsteinBarr virus, trauma, otitis media, human immunodeciency virus, demyelination, compression or tumor [11]. It is still controversial whether steroid treatment affects the natural course of Bells palsy [13] although some studies [1416] showed benet from oral steroids while other studies [1719] showed that there was no benet with steroid. We performed a retrospective study of a cohort of 29 children admitted to the University Department of Paediatrics and Adolescent Medicine in 19952003 with Bells palsy including 26 with single episode and three with recurrent episodes to analyze the natural course, and to see if there is any relationship between etiology or steroid treatment with prognosis.

2. Method The charts of all children with facial nerve palsy admitted to the University Department of Paediatrics and Adolescent Medicine at Queen Mary Hospital from 1995

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January to December 2003 were reviewed. The inclusion criteria were any children with acute isolated unilateral lower motor neuron type of facial nerve palsy. We have excluded those due to central nervous system disorders, neoplasms, otitis media, Melkersson-Rosenthal syndrome, trauma, or herpes zoster oticus (Ramsay Hunt syndrome). All children with Bells palsy were followed up until complete recovery. The clinical ndings and investigations were reviewed, including blood pressure, blood count, blood sugar, erythrocyte sedimentation rate (ESR), virological culture and titre, imaging studies with CT and/or MRI scan of brain, and brainstem auditory evoked potential (BAEP). Electrophysiological studies with electroneurography (ENoG), blink reex (BR) were performed with the Medelac 92A machine in some cases. 2.1. Serological results Of 32 episodes of facial palsy, positive serological group was dened as those with positive serological ndings (NZ10), while negative serological group was dened as those with negative serological ndings (NZ22). 2.2. Treatment with steroid Steroid group was regarded as those with steroid given (NZ23 out of 32 episodes) and non-steroid group was those without steroid given (NZ9). 2.3. Statistical analysis Chi-square test was used to compare clinical outcome between positive vs negative serological groups, and steroid vs non-steroid groups. Student t-test was used to compare the duration of recovery between positive and negative serological groups. All data were calculated by software SPSS 11.5.1. A P value of !0.05 is regarded as signicant.

3.1. Clinical course of Bells palsy The yearly admission of Bell palsy to our hospital was 25. The male to female ratio is 0.9/1. The mean age was 6 years 7 months (rangeZ9 days15.5 years). The distribution of children with Bells palsy in different age groups such as 03, 36, 69, 912, O12 years was shown in Fig. 1. The peak age distribution was under 3 years. The mean duration between onset of Bells palsy and admission to our hospital was 2 days (rangeZ112 days). There were 32 episodes altogether including three recurrent episodes. The ratio of the side affected was Left/RightZ1/0.8. Altogether 19 episodes had preceding events found with 15 episodes having upper respiration infection, two with minor head injury and two postvaccination. Two cases had positive family history of Bells palsy. Ten episodes had positive viral titre or mycoplasma pneumoniae infection. Twelve episodes had brain CT or/and MRI scans with 10 being normal and two with abnormalities. Brainstem MRI of one case with recurrent facial nerve palsy had inammatory change with facial nerve while another case with partial recovery, had parotitis in the contralateral side. Ten episodes had ENoG and/or BR done with abnormalities in eight, and four episodes had BAEP performed which was normal. Twenty-three episodes had steroid treatment. All except one had complete recovery. The duration of recovery ranged from 0.5 to 7 m. The time for recovery is shown by the Kaplan Meier curve (Fig. 2). 3.2. Relationship between clinical outcome and serological ndings or steroid treatment Altogether 31 episodes (96.9%) had complete recovery. We found that there was no signicant relationship between clinical outcome and positive serological ndings or steroid
11 10

3. Result A total of 54 medical records with facial nerve palsy were admitted to Queen Mary Hospital during the period 19952003, and of these 31 (57.4%) children with facial nerve palsy in our hospital were diagnosed as Bells palsy. Only 29 children with Bells palsy were included into this study (14 boys, 15 girls). We have excluded two cases with Bells palsy who defaulted follow-up after the rst visit. All 29 children with Bells palsy had normal blood pressure, blood counts and erythrocyte sedimentation rate. All children were followed up until complete recovery. Those children with incomplete recovery had follow-up to at least 2 years.
Number of cases
9 8 7 6 5 4

10

6 5

6 5

0-3 year

3-6 year

6-9 year

9-12 year

>12 year

Fig. 1. Distribution according to age groups (32 episodes).

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Recovery Function
1.0

Censored

0.8

0.6

0.4

0.2 0.0 0 10 20 30

Recovery Time ( month)

Fig. 2. Recovery function using Kaplan Meier curve (32 episodes).

treatment. However, we found that there was statistically signicant increase in the duration of complete recovery for those with positive virological conrmation or mycoplasma infection (Table 1).

studies [9,10] studied the value of ENoG and BR in children with Bells palsy. Our previous study showed that only direct response had prognostic signicance in predicting the outcome [9] whereas another pediatric study [10] reported that electrophysiological studies could not elucidate the cause or prognosis in children with recurrent facial nerve palsy. Most of the cases had complete recovery although their ENoG or BR had either normal or abnormal results. However, it is difcult for us to compare the prognostic value in predicting clinical outcome in this study as only four children out of 29 had both facial ENoG and BR performed. Brainstem auditory evoked potential (BAEP) study was rarely reported in children with Bells palsy. The BAEP was done in four children in this study, and the results showed no abnormalities. All children with Bells palsy in this study had normal CT brain or brain MRI except one child with recurrent facial nerve palsy, whose MRI of the brainstem showed inammatory change of the facial nerve while another case with partial recovery, had MRI evidence of parotitis in the contralateral side. One study [10] also reported no abnormalities for imaging scans in children with recurrent facial nerve palsy. 4.2. Etiology of Bells palsy The most likely etiology of Bells palsy is viral in origin. At present, the most likely cause seems to be HSV-1, where specic DNA can be isolated from the perineural uid of cases with Bells palsy [22]. Direct infectious or indirect para-infectious mechanisms followed by an immune reaction can be possible for viruses (cytomegalovirus, Epstein Barr virus, human herpesvirus 6, human herpesvirus 7, adenoviruses) or other pathogens infection. Nearly 60% children with Bells palsy in our study had preceding events found and mostly related to upper respiratory infection. Altogether 10 (31.3%) out of 32 episodes in our study had associated virus infections (such as herpes simplex virus, varicella zoster virus, or Epstein Barr virus) and mycoplasma pneumoniae infection. 4.3. Prognosis of Bells palsy with relationship to positive virological studies or mycoplasma pneumoniae infection The relationship of prognosis for children with Bells palsy associated with virus infections were rarely studied. It was interesting to nd in our study as there was no difference in clinical outcome between those with virus infections or mycoplasma pneumoniae infection and others with negative serological ndings (PO0.1). However, the duration of complete recovery was signicantly increased in children whose serological ndings were positive (P!0.007). This might mean that children with Bells palsy whose serological ndings were positive might have a longer duration to complete recovery. This is reassuring for parents in case the recovery lasted longer than the usual 2 weeks causing disgurement for

4. Discussion Nearly most children with Bells palsy in Hong Kong are referred to the pediatricians for further workup and treatment. Our Department of Pediatrics and Adolescent Medicine had been receiving most of the referrals in Hong Kong island. It is our impression that Bells palsy is uncommon in children as only 55 cases were seen within the same center over 20 years. Previously, we had reported 24 children with Bells palsy admitted to the same institute during 19861993 [9]. This report consisted of 29 (actually two cases defaulted follow-up and thus the total admission for this period should be 31) children with Bells palsy admitted in 19952003. There were few studies regarding the distribution of children with Bells palsy although one adult study [20] showed that the incidence increased with increasing age and another study showed that there were two peaks at the thirdfourth and sixth decades [21]. The evidence for a familial tendency of Bells palsy is rare [3], although two children with Bells palsy in our study had familial history of Bells palsy. 4.1. Electrophysiological tests and neuroimaging studies Different electrophysiological tests have been performed in adults with facial palsy to assess for prognosis. Electroneurography (ENoG), electromyography (EMG) and blink reex (BR) had been found to be useful. Only a few

Cumulative recovery

W.-X. Chen, V. Wong / Brain & Development 27 (2005) 504508 Table 1 Relationship between clinical outcome and positive serological ndings or steroid treatment in 29 children with Bells palsy (32 episodes) Clinical outcome Serological studiesa Positive Complete recovery Partial recovery P-value Duration of recovery (Months, MGSD) P-value 9 1 (PO0.1) (Positive vs negative) 3.36G2.12 (9 episodes) (P!0.007) (Positive vs negative) Negative 22 0 1.69G1.12 (22 episodes) Steroid Given 22 1 (PO0.5) (Given vs not given)

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Not given 9 0

M, mean; SD, standard deviation. a Including varicella zoster virus, EpsteinBarr virus, herpes simple virus, and mycoplasma pneumoniae.

the child, then one can the rest reassure the parents that complete recovery is likely in case there is positive virological evidence. This is important for such an alarming acute attack (Table 1). 4.4. Role of steroid treatment

One girl with partial recovery in our study was found after more than 2 years follow-up, and she had parotitis in the contralateral side three weeks after right Bells palsy. This suggested that the parotitis might be one of the causes of the incomplete recovery. 4.6. Recurrent Bells palsy

It is still controversial whether children with Bells palsy should be given steroid treatment or not. There were few studies regarding the relationship between prognosis and steroid treatment in children with Bells palsy. Salinas [17] and Salman [18] concluded that the available evidence from randomized controlled trials did not show signicant benet from treating Bells palsy with corticosteroids although others [1416] showed benet from oral steroids. Altogether 23 episodes in our study had steroid (12 mg/kg/d) treatment for 2 weeks course with slow titration down in the second week. Nine episodes of Bells palsy were not given steroid. After assessment of prognosis of children with or without steroid given, there was no signicant difference for clinical outcome found in our study (PO0.5), which indicated that steroid treatment might not accelerate the clinical recovery of children with Bells palsy. The use of steroid in children with Bells palsy had shown that steroid initiated at an early stage of childhood Bells palsy did not signicantly improve the outcome [19]. More randomized controlled trials with increased number of patients are needed to determine whether there is real benet for a short course of steroid therapy in children with Bells palsy. 4.5. Clinical course of Bells palsy The complete recovery rate was high in our study, with 31 episodes (96.9%) had complete recovery. The mean recovery time was 2.85 months (95% condence interval between 1.43 and 4.28 months). The recovery rate within 3 weeks for children with Bells palsy was 68.8%. The prognosis for children with Bells palsy who had not recovery by 7 months from onset is guarded as only 3.13% recovery probability was shown after 7 months with the Kaplan Meier curve (Fig. 2).

Recurrent facial nerve palsy is rarely reported in children. Currently, there is no denition of relapse or recurrence issue for Bells palsy. We presume that recurrence are seen in separate episodes which are not related to each other while relapse can be found in Bells palsy whose attacks may be more or less related to last episode. Thus, the prognosis and etiology may be different for recurrence or relapse. It may be difcult to differentiate the two conditions in the clinical scenario, though, we with our experience, having relapse with an interval more than 6 months are quite unlikely. Thus, for patients with recurrent facial nerve palsy on the same side within less than 6 months, the possibility of relapse is more likely. In one retrospective study [5] of 2568 patients with peripheral facial palsy seen during a 10-years period, 106 (8.2%) of 1293 patients with Bells palsy had recurrence. Some studies [8,6] reported that a positive family history of facial palsy indicated a higher risk of recurrence, and a poorer prognosis. Melkersson-Rosenthal syndrome (MRS) is a familial recurrent facial nerve palsy syndrome, characterized a classical triad of recurrent or persistent orofacial swelling, peripheral facial nerve paralysis and lingua plicata [23]. However, two children in our study with positive family history of Bells palsy had just one single episode of Bells palsy and complete recovery. One study [10] showed an incidence of 6% of 182 pediatric patients had recurrent Bells palsy, which was similar to adults. The rate of complete clinical recovery for this cohort was 70%, being lower than 90% reported for Bells palsy. Another Thai study [12] reported 2 (4.3%) out of 47 children with Bells palsy had recurrent Bells palsy, and the duration from onset to recurrence ranged from 6 months to 3.5 years. Recurrent Bells palsy may be due to either a recurrent viral attack or recrudescence of indolent viral antigens

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W.-X. Chen, V. Wong / Brain & Development 27 (2005) 504508 [8] Castillo Laita JA, Bunuel Alvarez JC, Castellote Olivito JM, Dominguez Fenolle MP, Cenarro Guerrero T, Calvo Ferrer C, et al. Prognosis in recurrent peripheral idiopathic facial paralysis in childhood. An Esp Pediatr 1992;36:4514. [9] Wong V. Outcome of facial nerve palsy in 24 children. Brain Dev 1995;17:2946. [10] Eidlitz-Markus T, Gilai A, Mimouni M, Shuper A. Recurrent facial nerve palsy in paediatric patients. Eur J Pediatr 2001;160:65963. [11] Siwula JM, Mathieu G. Acute onset of facial nerve palsy associated with Lyme disease in a 6 year-old child. Pediatr Dent 2002;24:5724. [12] Dhiravibulya K. Outcome of Bells palsy in children. J Med Assoc Thai 2002;85:3349. [13] Harney M, McConn Walsh R. Treatment controversies in Bells palsy. Ir Med J 2003;96:1978. [14] Williamson IG, Whelan TR. The clinical problem of Bells palsy: is treatment with steroids effective? Br J Gen Pract 1996;46:7437. [15] Adour KK, Ruboyianes JM, Von Doersten PG, Byl FM, Trent CS, Quesenberry Jr CP, et al. Bells palsy treatment with acyclovir and prednisone compared with prednisone alone: a double-blind, randomized, controlled trial. Ann Otol Rhinol Laryngol 1996;105: 3718. [16] Hato N, Matsumoto S, Kisaki H, Takahashi H, Wakisaka H, Honda N, et al. Efcacy of early treatment of Bells palsy with oral acyclovir and prednisolone. Otol Neurotol 2003;24:94851. [17] Salinas RA, Alvarez G, Alvarez MI, Ferreira J. Corticosteroids for Bells palsy (idiopathic facial paralysis). Cochrane Database Syst Rev 2002;(1):CD001942. [18] Salman MS, MacGregor DL. Should children with Bells palsy be treated with corticosteroids? A systematic review J Child Neurol 2001;16:5658. [19] Unuvar E, Oguz F, Sidal M, Kilic A. Corticosteroid treatment of childhood Bells palsy. Pediatr Neurol 1999;21:8146. [20] Rowlands S, Hooper R, Hughes R, Burney P. The epidemiology and treatment of Bells palsy in the UK. Eur J Neurol 2002;9:637. [21] Valenca MM, Valenca LP, Lima MC. Idiopathic facial paralysis (Bells palsy): a study of 180 patients. Arq Neuropsiquiatr 2001;59: 7339. [22] Murakami S, Mizobuchi M, Nakashiro Y, Doi T, Hato N, Yanagihara N. Bells palsy and herpe simplex virus: identication of viral DNA in endoneurial uid and muscle. Ann Intern Med 1996; 124:2730. [23] Levenson MJ, Ingerman M, Grimes C, Anand KV. MelkerssonRosenthal syndrome. Arch Otolaryngol 1984;110:5402.

within the facial nerve due to repeated viral exposures. Recurrent Bells palsy on the same side is common with herpes simplex infection but rare with varicella zoster infection. Facial palsy which recurs on the opposite side is usually idiopathic in origin [1]. In this study, 3 (10.3%) out of 29 children with Bells palsy admitted during 19952003 had recurrent facial nerve as compared to 6 (25%) out of 24 children with Bells palsy in our previous study during 19861993 [9]. However, none of the recurrent cases in our previous study had any positive virological studies. All three cases of recurrent Bells palsy had two episodes, negative family history, being on alternate sides in two and on the same side in one, and with complete recovery from 2 weeks to 4 months. The duration from onset to recurrence was from 4 months to 6 years and 10 months. For recurrences of facial nerve palsy, 70% occur within 10 years after the original episode. Pitts [2] reported that two attacks are usually separated by more than 1 year.

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