Peripheral facial nerve palsy in children in a Borrelia high‐endemic area, a retrospective follow‐up study

Abstract

Acute peripheral facial nerve palsy (FNP) is a well-described cranial nerve dysfunction of the seventh cranial nerve. The facial nerve carries motor fibres to the facial musculature, efferent secretory fibres to the lacrimal and salivary glands as well as afferent sensory fibres from the anterior two-thirds of the tongue and the external auditory canal. The onset of FNP is normally acute and causes inability to control facial muscles on the affected side. The annual incidence of FNP in children has been reported from 5 to 21 per 100 000 children/year 1-5 with up to 43/100 000 among children under 6 years of age. 4 Differential diagnosis for acquired FNP is infections (ie Lyme borreliosis/neuroborreliosis, members of the herpesvirus family and enteroviruses), trauma, tumours, leukaemia, vascular diseases, iatrogenic causes and idiopathic facial palsy (IFP) (FNP of unknown cause despite appropriate diagnostic measures). Neuroborreliosis, a tick-borne infection, is the most common identifiable cause among children and is significantly more common in children than adults. 1,4,6 In Borrelia high-endemic areas, neuroborreliosis has been reported as the cause in 23%-65% of children with FNP. 1-4,7 Whereas FNP attributable to infections is more prevalent in children, the majority of FNP among adults is IFP. The incidence of IFP increases with age as shown in a UK study where the lowest incidence was among children 0-6 years of age; 6.6 per 100 000 person years, compared to 20.1 per 100 000 years among those 15-29 years of age. 8 Larger cohorts in adults have shown a yearly incidence of IFP around 30 per 100 000. 9

Clinical characteristics of children with Borrelia‐related peripheral facial palsy and utility of cerebrospinal fluid testing

“…4,10 A study from Stockholm, Sweden, showed an even higher prevalence (58%) of LNB, with a peak in October. 3 Two more Swedish studies (Gothenburg city with 10 surrounding municipalities and Kalmar County) also showed a higher LNB incidence during June-November, with a peak in August. European and Swedish guidelines recommend LP as a standard procedure when managing children with PFP.…”

“…27 However, other studies have shown that 14%-32% of children with BPFP had no CSF pleocytosis and that patients with LNB had negative CSF Bb antibodies, particularly when LP was performed early in the course of the disease. 3,13,15 Therefore, negative CSF Bb antibodies or the absence of pleocytosis do not necessarily rule out a diagnosis of LNB.…”

“…

Acute peripheral facial palsy (PFP) is the most common nerve palsy in children, with an annual incidence of up to 30/100 000 children per year. [1][2][3] The most common cause is Borrelia burgdorferi (Bb) infection in endemic areas and idiopathic peripheral facial palsy (IPFP) in non-endemic areas. Together, these two account for at least 90% of all PFP cases.

…”

“…4,7,9,12 This difference has resulted in significant variation in the management of PFP, with rates of CSF testing ranging from 0% to 90%. 3,4,10,13 The utility of CSF testing is also questioned, as CSF pleocytosis and intrathecal antibodies can be absent early in the trajectory of PFP. [14][15][16][17] Studies have attempted to identify clinical predictors to differentiate IPFP from LNB in children with PFP.…”

“…[14][15][16][17] Studies have attempted to identify clinical predictors to differentiate IPFP from LNB in children with PFP. 3,4,10 They identified that prodromal symptoms such as headache, fever, vomiting and nausea were more common in children with PFP due to LNB. However, these studies were performed mostly in the United States, and there are geographic differences in the clinical presentation of Lyme disease due to the different inflammatory activity of the Borrelia strains that are dominant in the United States and Europe.…”