Peripheral Facial Palsy in Children: A Cerebrospinal Fluid Study

Seven children and five adults with acute, peripheral facial palsy were investigated by clinical, blood and cerebrospinal fluid (CSF) examinations. Routine blood examinations and blood and CSF serological tests were normal. In all children studied the CSF showed an increased number of mononuclear white cells despite the absence of clinical signs of meningitis in all but one. Two of the twelve patients studied had normal CSF. Contrary to that in adults the outcome in children was excellent, all recovered totally within three months. The present study indicates that acute, peripheral facial palsy is a manifestation of a generalized disorder with subclinical pathology of the central nervous system, and that in children a lumbar puncture should be done, even if there are no signs of meningitis.     

Lyme borreliosis--the most frequent cause of acute peripheral facial paralysis in childhood

A prospective hospital-based multicentre study in Lower Saxony allowed to investigate the frequency of acute peripheral facial palsy due to Lyme borreliosis and its clinical and laboratory characteristics. Diagnosis of Lyme Borreliosis was based on detection of IgM antibodies against Borrelia burgdorferi in serum and CSF as well, using an IgM capture ELISA. Between June 1986 and October 1987 27 consecutive cases with acute peripheral facial palsy were studied. Lyme borreliosis is the main cause of peripheral facial palsy in childhood. It was verified serologically in two thirds of the cases. All cases with a positive history for a tick bite and/or an erythema migrans in the head-neck region showed ipsilateral neurological affection suggesting a direct invasion via the affected nerve by Borrelia burgdorferi. Peripheral facial palsy due to Lyme borreliosis represents a monosymptomatic meningoradiculitis. All children with Lyme borreliosis revealed a lymphocytic CSF pleocytosis, whereas in cases of unknown etiology CSF findings usually were normal. Therefore, in any case of facial palsy with an inflammatory CSF syndrome Lyme borreliosis has to be suspected unless proven otherwise.     

Peripheral facial palsy in childhood--Lyme borreliosis to be suspected unless proven otherwise

27 consecutive cases with acute peripheral facial palsy were studied for Lyme borreliosis. In 16 out of 27 children Lyme borreliosis could be diagnosed by detection of specific IgM antibodies in CSF. CSF findings allow a clear distinction according to etiology. All children with facial palsy due to Lyme borreliosis revealed lymphocytic CSF pleocytosis, whereas in cases of unknown etiology CSF was usually normal. Bilateral facial palsy occurred only in children with Lyme borreliosis. All cases with a positive history of tick bite and/or erythema migrans in the head-neck region showed ipsilateral neurological affection suggesting a direct invasion via the affected nerve by Borrelia burgdorferi.     

Peripheral Facial Palsy in Childhood-Lyme Borreliosis to be Suspected Unless Proven Otherwise

ABSTRACT. 27 consecutive cases with acute peripheral facial palsy were studied for Lyme borreliosis. In 16 out of 27 children Lyme borreliosis could be diagnosed by detection of specific IgM antibodies in CSF. CSF findings allow a clear distinction according to etiology. All children with facial palsy due to Lyme borreliosis revealed lymphocytic CSF pleocytosis, whereas in cases of unknown etiology CSF was usually normal. Bilateral facial palsy occurred only in children with Lyme borreliosis. All cases with a positive history of tick bite and/or erythema migrans in the head-neck region showed ipsilateral neurological affection suggesting a direct invasion via the affected nerve by Borrelia burgdorferi.     

Tick-borne Borrelia-Meningitis in Children An Outbreak in the Kalmar Area during the Summer of 1984

ABSTRACT. Nine children, aged 5 to 11 years, with subacute or chronic meningitis were studied. Symptoms started during the summer season in all patients and in eight of the patients the disease began with a localized erythematous lesion (ECM), mostly in the face. In one patient only there was a history of an insect bite at the site of the erythema. The neurological abnormalities included aseptic meningitis, peripheral facial nerve palsy (5/9) and oculomotor nerve palsy (1/9). Most children complained of headache, fatigue, loss of appetite and had a low grade fever. High antibody titers to Borrelia spirochetes in serum and/or cerebrospinal fluid (CSF) were demonstrated by ELISA in eight of the nine patients and by indirect immunofluorescence assay (IFA) in three patients. All patients had a dramatic improvement in their general condition and became afebrile within three days of institution of i.v. penicillin G treatment (i.v. cefuroxime in one patient).     

Tick-borne Borrelia-meningitis in children. An outbreak in the Kalmar area during the summer of 1984

Nine children, aged 5 to 11 years, with subacute or chronic meningitis were studied. Symptoms started during the summer season in all patients and in eight of the patients the disease began with a localized erythematous lesion (ECM), mostly in the face. In one patient only there was a history of an insect bite at the site of the erythema. The neurological abnormalities included aseptic meningitis, peripheral facial nerve palsy (5/9) and oculomotor nerve palsy (1/9). Most children complained of headache, fatigue, loss of appetite and had a low grade fever. High antibody titers to Borrelia spirochetes in serum and/or cerebrospinal fluid (CSF) were demonstrated by ELISA in eight of the nine patients and by indirect immunofluorescence assay (IFA) in three patients. All patients had a dramatic improvement in their general condition and became afebrile within three days of institution of i.v. penicillin G treatment (i.v. cefuroxime in one patient).     

Prolonged nonbacterial meningitis: clinical aspects and cerebrospinal fluid findings

Ten to 28 days after hospital admission cell count and/or total protein concentration showed no decrease or further increase in 33 children (25 boys, 8 girls) between 2 to 15-years of age who suffered from acute aseptic meningitis (causative agents: mumps virus in 9 children, enterovirus in 5 children). Three of these children had cerebral palsy as a possible predisposing factor. The onset of prolonged aseptic meningitis was protracted in some children. At hospital admission the clinical features of this disorder differed not from those in uncomplicated acute aseptic meningitis. In 4 children a one-sided and in 4 patients a doublesided peripheral facial paralysis occurred as a transitory complication. One child showed transient arterial hypertension. EEG was normal in most of the children or revealed a slight general slowing only. Apart from a slight enlargement of the ventricles in 3 children cerebral CT showed no abnormality. Complaints like vertigo, headache, and vomiting persisted for weeks or months in part of the children. During the course of the disease CSF reflected two different reactions: 1. further increase of total protein in combination with a minimal cellular response, affecting 2-10 years old boys and girls equally; protein electrophoresis revealing the pattern of severe blood-CSF barrier disturbance, 2. persistant elevation or further increase of both cell count and total protein occurring nearly exclusively in 6-15 years old boys, associated with the CSF-protein pattern of severe blood-CSF barrier disturbance and of oligoclonal gamma-fractions.(ABSTRACT TRUNCATED AT 250 WORDS)     

Clinically inapparent meningitis complicating periorbital cellulitis

Two young children with periorbital (preseptal) cellulitis were found to have meningitis despite having no signs of meningeal irritation and normal cerebrospinal fluid (CSF) cell counts and chemistries. These cases are reported to remind physicians caring for acutely ill children that periorbital cellulitis can have life-threatening complications and that meningitis can occur in the absence of significant clinical signs and in the presence of an initially normal CSF.     

Canalicular magnetic stimulation lacks specificity to differentiate idiopathic facial palsy from borreliosis in children.

OBJECTIVE: To investigate the role of transcranial magnetic stimulation (TMS) to differentiate between idiopathic facial nerve palsy (iFNP) and facial nerve palsy due to borreliosis (bFNP). PATIENTS AND METHODS: Transcranial and intracanalicular magnetic and peripheral electrical stimulation of the facial nerve together with clinical grading according to the House and Brackmann scale were performed in 14 children and adolescents with facial palsy (median age 11.5yr, range 4.6-16.5yr). Serum and cerebrospinal fluid (CSF) were evaluated for antibodies against Borrelia burgdorferi and CSF cell count, glucose and protein content were screened with methods of routine laboratory testing. Data of patients were compared with normal values established in 10 healthy subjects (median age 10.2yr, range 5.1-15.3yr). RESULTS: Patients with iFNP showed a significant decrease in MEP amplitude to canalicular magnetic stimulation compared with healthy controls (p=0.03). However, MEP amplitude did not discriminate sufficiently between the two groups, because the ranges of dispersion of MEP amplitudes overlapped. Patients with bFNP had normal MEP amplitudes to canalicular magnetic stimulation compared with normal subjects. CONCLUSION: Diagnostic assessment by TMS failed to provide a reliable diagnostic criterion for distinguishing between iFNP and bFNP in children and adolescents.     

Recurrent facial nerve palsy in paediatric patients

The aim of the study was to investigate the clinical presentation and prognosis of recurrent facial nerve palsy (RFNP) in children. The files of 182 patients referred to the Schneider Children's Medical Centre of Israel for neurological evaluation of isolated peripheral facial nerve palsy between October 1992 and December 1998 were reviewed. RFNP was found in 11 patients (9 females, 2 males), with an incidence of 6%. In two males, the aetiology was traced to Melkersson-Rosenthal syndrome and these patients were separated from the rest of the group. Three children had two episodes of facial nerve paresis which completely resolved clinically within several weeks. Six other children underwent electrophysiological studies. Two of the latter with residual neurological damage, and one child with abnormal blink reflex only, showed decreased facial nerve conduction velocity and abnormal blink reflex. Three children with complete recovery had disturbed blink reflex only with normal nerve conduction. Brain imaging studies as well as laboratory work-up were non-contributory in all cases. CONCLUSION: The frequency of recurrent facial nerve palsy in children was similar to that in adults. The most significant factors in the evaluation of recurrent facial nerve palsy are medical history and physical findings at diagnosis and after short follow-up. In our patients, electrophysiological studies did not have either clinical or prognostic significance. The rate of full clinical recovery is about 70%, lower than in Bell palsy.     

Borrelia infection in children

All children (less than or equal to 15 years) admitted during 1986 to Sachs Children's Hospital and presenting signs of facial palsy and/or meningitis, or with a history of known tick bite followed by headache, fatigue and muscle pain, were investigated for antibodies to Borrelia in serum and cerebrospinal fluid. (The hospital's catchment area has a high incidence of tick-borne Borrelia infections.) Significantly elevated antibody titre was found in 15 of the 33 patients, in three cases only in cerebrospinal fluid. Eight of the 15 children had facial palsy, which was concomitant with meningitis in six cases. Intravenous penicillin was given to all 15 patients with positive antibody titre, and additionally to three severely ill small children with facial palsy and meningitis. Furthermore, two cases of erythema chronicum migrans, which is considered pathognomonic for Borrelia infection, were treated with penicillin perorally. Cases of Borrelia infection occurred throughout the year, but with a peak in August. To emphasize the variety of symptoms, three cases are presented in some detail.     

Borrelia Infection in Children

ABSTRACT. All children (15 years) admitted during 1986 to Sachs Children's Hospital and presenting signs of facial palsy and/or meningitis, or with a history of known tick bite followed by headache, fatigue and muscle pain, were investigated for antibodies to Borrelia in serum and cerebrospinal fluid. (The hospital's catchment area has a high incidence of tick-borne Borrelia infections.) Significantly elevated antibody titre was found in 15 of the 33 patients, in three cases only in cerebrospinal fluid. Eight of the 15 children had facial palsy, which was concomitant with meningitis in six cases. Intravenous penicillin was given to all 15 patients with positive antibody titre, and additionally to three severely ill small children with facial palsy and meningitis. Furthermore, two cases of erythema chronicum migrans, which is considered pathognomonic for Borrelia infection, were treated with penicillin perorally. Cases of Borrelia infection occurred throughout the year, but with a peak in August. To emphasize the variety of symptoms, three cases are presented in some detail.     

Children with meningeal signs: predicting who needs empiric antibiotic treatment

BACKGROUND: Since delayed diagnosis and treatment of bacterial meningitis worsens patient prognosis, clinicians have a low threshold to perform a lumbar puncture or to start empiric antibiotic treatment in patients suspected of having meningitis. OBJECTIVE: To develop a decision rule, including cerebrospinal fluid (CSF) indices and clinical characteristics, to determine whether empiric antibiotic treatment should be started in children with meningeal signs. DESIGN: Multivariable logistic regression analysis of retrospectively collected data. Bacterial meningitis was defined as a CSF leukocyte count of more than 5/ micro L with positive bacterial culture findings from CSF or blood specimens. SETTING: Pediatric emergency department of a pediatric university hospital. PATIENTS: A total of 227 children (aged 1 month to 15 years) with meningeal signs. MAIN OUTCOME MEASURE: The diagnostic value of adding early obtainable CSF indices to clinical characteristics to predict bacterial meningitis. RESULTS: Independent predictors of bacterial meningitis from early obtainable CSF indices were the CSF polymorphonuclear leukocyte count and the CSF-blood glucose ratio. The diagnostic value (area under the receiver operating characteristic curve) of this CSF model was 0.93. Application of the model together with clinical characteristics could predict early the absence of bacterial meningitis in 69 (30%) of the 227 patients so that empiric antibiotic treatment could be safely withheld. CONCLUSION: A diagnostic decision rule that uses clinical characteristics at admission, the CSF polymorphonuclear leukocyte count, and the CSF-blood glucose ratio is a useful tool for deciding whether to start empiric antibiotics in children with meningeal signs.     

Cerebrospinal fluid immunoglobulins in children.

Cerebrospinal fluid (CSF) immunoglobulins were measured in 62 normal children, in 9 children with purulent meningitis, and in 10 children with presumptive viral meningitis. The mean values in normal children were IgA 0, IgM 0, and IgG 0.84 +/- 1.4 mg/100 ml (+/- SD). The mean levels of all CSF immunoglobulins were raised in acute bacterial meningitis and were significantly greater than the levels found in viral meningitis. CSF IgM was 0.16 +/- 0.5 mg/100 ml in viral meningitis compared with 2.64 +/- 2.06 mg/100 ml in bacterial meningitis (P less than 0.01). However, these values overlapped to a considerable extent and, generally, measurement of CSF immunoglobulins did not enhance diagnostic accuracy in this group of children.     

Cerebrospinal fluid immunoglobulins in children

Cerebrospinal fluid (CSF) immunoglobulins were measured in 62 normal children, in 9 children with purulent meningitis, and in 10 children with presumptive viral meningitis. The mean values in normal children were IgA 0, IgM 0, and IgG 0.84 +/- 1.4 mg/100 ml (+/- SD). The mean levels of all CSF immunoglobulins were raised in acute bacterial meningitis and were significantly greater than the levels found in viral meningitis. CSF IgM was 0.16 +/- 0.5 mg/100 ml in viral meningitis compared with 2.64 +/- 2.06 mg/100 ml in bacterial meningitis (P less than 0.01). However, these values overlapped to a considerable extent and, generally, measurement of CSF immunoglobulins did not enhance diagnostic accuracy in this group of children.     

Opsoclonus in a child with neuroborreliosis: Case report and review of the literature

Opsoclonus consists of massive erratic rapid eye jerks. They may occur in isolation or in association with myoclonus and ataxia, i.e., opsoclonus-myoclonus syndrome (OMS). We report the case of a 9-year-old girl who suffered from headaches for several days and was shown to have opsoclonus and left peripheral facial palsy. Work-up excluded the diagnosis of neuroblastoma, but CSF analysis showed aseptic meningitis, and serology for Borrelia burgdorferi (Lyme) was positive. The outcome was favorable with complete regression of symptoms after treatment with ceftriaxone 2 g/day for 3 weeks. Although rare, the diagnosis of Lyme neuroborreliosis must be raised in the presence of isolated opsoclonus, particularly if the clinical picture is incomplete and if other features, such as peripheral facial palsy and pleocytosis in the CSF, are present.     

Evaluation of the polymerase chain reaction for the detection ofBorrelia burgdorferi in cerebrospinal fluid of children with acute peripheral facial palsy

Neuroborreliosis occasionally represents a diagnostic problem, especially in the early stage of the infection. The polymerase chain reaction (PCR) offers an attractive alternative to antibody testing. The aim of our study was to investigate the diagnostic potential of PCR in comparison to antibody tests in CSF of children with facial palsy. In contrast to other manifestations of neuroborreliosis, facial palsy is a welldefined clinical entity in which CSF findings allow an early distinction according to aetiology. The study included 17 children with neuroborreliosis, defined by the detection of specific IgM antibodies in CSF, and 20 children with facial palsy of unknown cause. Primers used for the nested PCR were generated from conserved sequences of the OspA-gene. Most of the cases in both subgroups have been examined within a few days after the onset of the paresis. Only in 2 out of 17 cases with neuroborreliosis could specific DNA be amplified. The PCR gave negative results in all cases of the control group.Conclusion The IgM capture ELISA is superior to PCR to support the clinical diagnosis of neuroborreliosis.     

Lyme neuroborreliosis in pediatrics: A retrospective,descriptive study in southwest France

BackgroundThe neurological effects of Lyme borreliosis in children are varied and their clinical progression is not widely reported in the French literature. We carried out a retrospective study to describe the clinical characteristics of Lyme neuroborreliosis in children in southwest France and their clinical progression at 6 months.MethodsThis study was carried out at Toulouse University Hospital during the period 2006–2017 using patient records. Case definition was based on the combined French clinical and laboratory diagnostic criteria.ResultsIn total, 26 children were included. The median age was 8 years (4–14 years). The different neurological symptoms reported were: meningoradiculitis (62%), which was usually associated with facial palsy (54%); isolated facial palsy (15%); isolated meningitis (8%); polyradiculoneuritis (4%); benign intracranial hypertension (4%) and myelomeningoradiculitis (4%). The most common functional symptoms were headaches (54%), the perception of asthenia (42%), neck pain (27%), and a loss of appetite (19%). Patients with laboratory meningitis (84%) often had no signs of meningism or headaches (38%).ConclusionThe majority of the cases involved meningoradiculitis but other, less common, neurological conditions have been described. The clinical signs suggestive of meningitis are not very marked and might delay the diagnosis.     

Acute mastoiditis. Diagnosis and complications

Thirty children with acute mastoiditis were identified over a 12-year-period and their hospital records were reviewed retrospectively. All had abnormal tympanic membranes and 26 (87%) had swelling above or posterior to the ear that deviated the pinna. Findings on mastoid roentgenograms included clouding (n = 12) and osteitis (n = 7); six were normal. From 13 patients, bacteria were recovered from normally sterile sites and included Pneumococcus (n = 5), group A streptococcus (n = 3), Haemophilus (n = 2), and anaerobes (n = 3). Complications occurred in 13 children, including subperiosteal abscess (n = 7), meningitis (n = 4), osteitis (n = 7), facial palsy (n = 1), and subdural empyema and brain abscess (n = 1). Four of the six children with neurological complications had no external signs of acute mastoiditis on physical examination. Overall, 19 (63%) of the children recovered without mastoidectomy. We conclude that children without meningitis or subperiosteal abscess may be treated initially with antimicrobial therapy plus myringotomy. The need for mastoidectomy should be reassessed in children who fail to respond in 24 to 48 hours.     

Lyme borreliosis in children

A total of 27 children with clinical symptoms indicative of Lyme borreliosis are described, 21 of which were seropositive. CNS symptoms were found in 17 of the seropositive childen (81%). Of these 21, 7 were CSF negative. Another 3 (with Bell's palsy and/or aseptic meningitis) were initially CSF negative but developed specific CSF titres 80, 65 and 120 days after the first lumbar puncture, respectively. Thus, seropositive children with aseptic meningitis and without initial signs of an infectious aetiology should be checked for a neuroborreliosis even when CSF negative in the first lumbar puncture. Antibiotic therapy undertaken in 26 children showed clinical recovery (Sodium penicillin, 300,000–500,000 units/kg per day for 14 days). One developed residual hypoacusis. Comparison of enzyme-linked immunosorbent assay (ELISA) IgG values from 27 cases with those of 30 healthy controls showed that elevated ELISA titres are a good indication of the disease. However, Lyme borreliosis can only be diagnosed correctly if the clinical symptoms conform with specific titres.Abbreviations Bb Borrelia burgdorferi - CNS central nervous system - CSF cerebrospinal fluid - EBV Epstein-Barr virus - ECM erythema chronicum migrans - ELISA enzyme-linked immunosorbent assay - Ig immunoglobulin - Lb Lyme borreliosis