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

AIM: To describe the aetiology and clinical characteristics of acute peripheral facial palsy (PFP) in children and investigate the utility of the European Federation of Neurological Societies (EFNS) criteria for diagnosing Borrelia-related PFP (BPFP) based on cerebrospinal fluid (CSF) testing and the Centers for Disease Control and Prevention (CDC) criteria based on serology. METHODS: We retrospectively identified children aged <18 years diagnosed with acute PFP between 2014 and 2020. We used the EFNS criteria as the gold standard and the CDC criteria for diagnosing BPFP. RESULTS: Out of 257 children with PFP, 93 (36%) fulfilled the EFNS or CDC criteria for BPFP. We found a discrepancy between the EFNS criteria with CSF testing and the CDC without CSF testing in 27 (14%) of the 190 children with available data. Of the 37 children with PFP and ≥2 symptoms of fever, fatigue, nausea/vomiting or meningeal symptoms, 31 (84%) fulfilled the EFNS criteria for BPFP. CONCLUSION: Borrelia is a common cause of PFF in children, and its prevalence is higher in children with systemic symptoms. Also, CSF testing did not have decisive management implications in most cases. Therefore, clinical evaluation and Borrelia serology could be the initial steps in the diagnosis of PFP in children.

Acute facial nerve palsy in children in a Lyme disease-endemic area in the Netherlands.

We assessed the prevalence of Lyme neuroborreliosis in children with acute facial nerve palsy in a Lyme-endemic region and patient characteristics associated with this. All children visiting one of three participating hospitals between January 2010 and December 2016 were included in the study. Of 104 children referred to the hospital with facial nerve palsy, 43% had Lyme neuroborreliosis and 57% idiopathic facial palsy. Characteristics significantly associated with Lyme neuroborreliosis were headache (55% versus 18%), meningeal irritation (21% versus 5%), presentation in summer months (69% versus 37%), and a previous tick bite (33% versus 7%).

Pediatric Bilateral Facial Paralysis: An Unusual Presentation of Lyme Disease.

Pediatric bilateral facial nerve paralysis (FNP) is a rare condition, representing less than 2% of all cases of FNP. The differential diagnosis of FNP is extensive (ranging from infectious, traumatic, neurologic, to idiopathic) and often can present as a diagnostic challenge. In contrast to unilateral presentation, bilateral FNP presents as a manifestation of serious systemic conditions, including meningitis (infectious and neoplastic), brain stem encephalitis, Guillain-Barre syndrome, sarcoidosis, Lyme disease, human immunodeficiency virus infection, leukemia, and vasculitis. In the evaluation of a child who presents with bilateral FNP, history plays the utmost role in the diagnosis. A history of rash consistent with erythema chronicum migrans, recent tick exposure, or travel to a Lyme disease-endemic area is highly suggestive that the facial paralysis is a result of Lyme disease. It is also important to recognize that Lyme disease is emerging as the most common infectious etiology of bilateral FNP in the pediatric population. In this case report, we describe a 16-year-old boy who presented to the emergency department with complaints of headache and bilateral FNP, an unusual presentation of Lyme disease.

Cephalic tetanus presenting with bilateral facial palsy.

We discuss the case and differential diagnoses of an elderly man who presented with bilateral facial palsy. He had injured his forehead in the garden during a fall on his face and the open wound was contaminated by soil. He then presented to the emergency department with facial weakness causing difficulty speaking. The penny dropped when he started developing muscle spasms affecting his lower jaw a day after admission. It also became clear that he could not open his mouth wide (lock jaw). The combination of muscle spasms and lock jaw (trismus) made tetanus the most likely possibility, and this was proven when he had samples taken from his wound and analysed under the microscope, which showed Clostridium tetani bacilli. C. tetani spores are widespread in the environment, including in the soil, and can survive hostile conditions for long periods of time. Transmission occurs when spores are introduced into the body, often through contaminated wounds. Tetanus in the United Kingdom is rare, but can prove fatal if there is a delay in recognition and treatment.

Intra-aural tick bite causing unilateral facial nerve palsy in 29 cases over 16 years in Kandy, Sri Lanka: is rickettsial aetiology possible?

BACKGROUND: Over the last two decades intra-aural tick infestation (otoacariasis) has been a common occurrence in the hilly central region in Sri Lanka. Very occasional detection of isolated unilateral facial nerve palsy associated with otoacariasis attributed to toxin damage of the nerve prompted us to study the clinico-epidemiology and aetio-pathology of the problem. METHODS: All cases having isolated unilateral facial nerve palsy associated with otoacariasis presented to, Ear Nose and Throat clinic at General Hospital Kandy, Sri Lanka from 2001 to 2016 were included in the study. The facial palsies were assessed with nerve conduction studies and, harvested ticks were identified. RESULTS: There were 29 patients with mean age of 46 years (range 22-76 years) with male to female ratio of 1:1.9. First 12 patients without specific treatment took 1-55 months for recovery and 4 had axonal degeneration. Last 5 patients were treated with doxycycline and recovered in 4 weeks. They had strong sero-conversion of immunofluorescence antibodies against spotted fever rickettsioses and the tick harvested from the last patient was PCR positive for rickettsial DNA. Identified ticks belonged to Dermacentor, Amblyomma, Rhipicephalus and Hyalomma species. CONCLUSIONS: On contrary to popular toxin theory, we were able to demonstrate treatable rickettsial aetio-pathology as the cause of otoacariasis associated lower motor facial palsy in Sri Lanka.

Serological Evidence of Borrelia Burgdorferi Infection in Mexican Patients with Facial Palsy.

BACKGROUND: Facial palsy is the most frequent manifestation of neuroborreliosis in the United States, Europe, and Asia, whereas in Mexico, its frequency is unknown. OBJECTIVE: We aimed to determine the frequency of Borrelia spp. infection in patients with acute facial palsy in Mexico. MATERIALS AND METHODS: In this cross-sectional, referral hospital-based survey, 191 patients with facial palsy were selected and clinical and epidemiologic data recorded. IgM and IgG serum antibodies to Borrelia burgdorferi were tested by enzyme-linked immunosorbent assay (ELISA) and confirmed by Western-Blot (WB). IgM and IgG antibodies against the herpes viruses HSV-1, HSV-2, cytomegalovirus, and Epstein-Barr virus were tested by ELISA. RESULTS: 71 patients (37%) tested positive by ELISA to either Borrelia spp. or the herpes viruses. Of 25 patients (13%) who tested positive for B. burgdorferi by ELISA, 23 (12%) were confirmed by WB; 14 had IgM and 9 had IgG antibodies. Among the 14 IgM-WB positive patients, two cases recognized antigens of B. burgdorferi sensu stricto (s.s.), 10 of Borrelia garinii and 2 of B. afzelii, whereas all 9 IgG-WB positive were reactive against B. burgdorferi s.s. 14 patients had facial palsy in addition to other clinical data compatible with Lyme borreliosis. Patients infected with B. burgdorferi s.s. had a longer recovery time and a significantly higher risk (odds ratio 4.4, 95% confidence interval 1.5-12.9) of recurrent facial palsy than patients infected with other Borrelia genospecies. CONCLUSIONS: Borrelia infection is frequent in facial palsy patients in Mexico, with B. burgdorferi s.s. and B. garinii being the most frequent causative species.

Bell's palsy and sudden deafness associated with Rickettsia spp. infection in Sweden. A retrospective and prospective serological survey including PCR findings.

BACKGROUND AND PURPOSE: Sixty patients with facial palsy and 67 with sudden deafness were retrospectively or prospectively examined for serological evidence of rickettsial infection; in six cases where cerebrospinal fluid was available, patients were also examined for presence of rickettsial DNA. METHODS: Rickettsial antibodies were detected in single or paired serum samples using immunofluorescence with Rickettsia helvetica as the antigen and in four cases also using western blot. Using PCR and subsequent direct cycle sequencing, the nucleotide sequences of the amplicons (17 kDa protein gene) in cerebrospinal fluid were analysed. RESULTS: Five out of 60 (8.3%) patients with facial palsy and eight of 67 (11.9%) with hearing loss showed confirmative serological evidence of infection with Rickettsia spp. An additional three and four patients in the facial palsy and hearing loss groups, respectively, showed evidence of having a recent or current infection or serological findings suggestive of infection. In four cases, the specificity of the reaction was confirmed by western blot. An additional 70 patients were seroreactive with IgG or IgM antibodies higher than or equal to the cut-off of 1:64, whereas 37 patients were seronegative. Only two of 127 patients had detectable antibodies to Borrelia spp. In three of six patients, rickettsial DNA was detected in the cerebrospinal fluid, where the obtained sequences (17 kDa) shared 100% similarity with the corresponding gene sequence of Rickettsia felis. CONCLUSIONS: These results highlight the importance of considering Rickettsia spp. as a cause of neuritis, and perhaps as a primary cause of neuritis unrelated to neuroborreliosis.

Cavernous sinus thrombosis as a result of a fungal infection: a case report.

Cavernous sinus thrombosis (CST) is a rare disease with the potential for significant morbidity and even death. Rapid diagnosis and aggressive medical and surgical management are imperative for patients with CST. The cause may be aseptic or infectious. When the cause is infectious in nature, it is most commonly from a bacterial origin. However, we present the case of a 57-year-old man with a fungally related CST that ultimately led to his death.

Rhinocerebral mucormycosis: five cases and a literature review.

OBJECTIVE: In this retrospective study, we describe our experience in the diagnosis and management of rhinocerebral mucormycosis (RCM), a rapidly lethal fungal infection. METHODOLOGY: Between 1997 and 2007, five patients hospitalized for suspicion of RCM. Computed tomography was performed in all cases, and diagnosis was confirmed after anatomopathological or mycological examination. All patients underwent medical and surgical treatment. Follow-up was clinical and radiological with a mean period of 17 months. RESULTS: All patients were diabetic. Exophthalmia, rhinorrhea, and ophthalmoplegia were the most frequent symptoms observed. One patient had loss of visual acuity and another exhibited peripheral facial palsy. One patient had extensive hemifacial cutaneous necrosis. Nasal endoscopy revealed black necrotic lesions in one case, and another patient had a tumefaction localised in the left middle meatus. Necrotic lesions were most often found in the orbit, the maxillary and the ethmoidal sinuses on computed tomography (four cases for each site). One patient had thrombophlebitis of the cavernous sinus, and another had an intracranial extension. All patients were administered ordinary insulin and intravenous amphotericin B. Surgical debridement of the nasal cavity and the involved sinuses was performed through lateral rhinotomy (four cases) or endoscopy (one case). Unilateral orbital exenteration was associated in two cases. Progression was favourable in four cases; one patient died from sepsis despite aggressive treatment. CONCLUSION: Early diagnosis is crucial for the management of RCM. Treatment of underlying disorders, use of intravenous amphotericin B, and aggressive surgical intervention are key in reducing morbidity and mortality rates.

Skull Base Fungal Osteomyelitis: A Case Report and Review of the Literature.

Skull base osteomyelitis (SBO) is an invasive infection refractory to therapy, closely linked with malignant otitis externa (MOE). It is characterized by a mild clinical presentation that can delay cross-sectional imaging considered as the key to revealing it. Skull base osteomyelitis typically affects elderly diabetics and immunocompromised patients (>70 years). It most commonly has an otogenic origin due to an extension of MOE. The prognosis can be very poor without the administration of adequate and timely therapy at an early disease stage. Nowadays, Pseudomonas aeruginosa remains the most common pathogen associated with SBO. Fungi are a rare cause of MOE. This report documents a rare case of otogenic SBO caused by Candida parapsilosis in a diabetic patient, with persistent otologic symptoms as clinical onset and resistance to medical treatment. Fungal MOE has more subtle symptoms and is more aggressive than its bacterial counterpart. When MOE is resistant to antibacterial drugs, this should raise the suspicion of a fungal etiology of MOE. The current guidelines do not exhaustively describe the diagnosis, antifungal drugs of choice, and optimum duration of treatment. The description of these rare clinical cases should help with the multidisciplinary management of this disease in order to optimize the diagnosis and therapeutic protocol.

CXCL13 in patients with facial palsy caused by varicella zoster virus and Borrelia burgdorferi: a comparative study.

High cerebrospinal fluid (CSF) concentrations of the chemokine CXCL13 have been associated with Lyme neuroborreliosis (LNB), and have recently been studied as a potential diagnostic marker. It has proven difficult to establish a reliable diagnostic cut-off, possibly in part due to heterogenicity of case-control groups. Our purpose was to investigate CSF CXCL13 concentrations in patients with similar clinical presentations, facial palsy. We retrospectively included patients with facial palsy associated with LNB (n = 21), or varicella zoster virus (VZV) (n = 26). Median CXCL13 concentrations were significantly higher in patients with LNB facial palsy compared to VZV facial palsy. Receiver-operating characteristic analyses yielded an optimal cut-off concentration at 34.5 pg/mL (sensitivity 85.7%, specificity of 84.6%), lower than that in previous studies. Although the analysis has potential, it is still not adequately established that CXCL13 provides additional, clinically useful, diagnostic information over current recommendations.

Lyme neuroborreliosis in children: Report of nine cases and a review of the literature.

Lyme neuroborreliosis is a bacterial infection caused by the dissemination and proliferation of a Borrelia species in the central nervous system. Neuroborreliosis occurs after transmission of the pathogen from an infected tick to a human host during a tick bite. We report nine cases of pediatric neuroborreliosis collected by the National Observatory of Pediatric Bacterial Meningitis in France between 2001 and 2012. The nine children, aged 4-13 years, were identified in northern and eastern France and had the following clinical features: meningeal irritation alone or with facial palsy, or isolated facial palsy. All cases showed anti-Borrelia antibodies in cerebrospinal fluid or serum, or with a positive Borrelia PCR in the CSF. The outcome was favorable in all cases after a 2- to 3-week course of third-generation cephalosporin. On the basis of these nine pediatric cases, this study provides an update on the epidemiology, pathophysiology, diagnostic strategy, and treatment of neuroborreliosis, with insight into the specific features of pediatric neuroborreliosis and the difficulties encountered in the diagnosis of this infection.

Cerebrospinal Fluid Pleocytosis and Elevated C-X-C Motif Chemokine Ligand 13 Value Predict Lyme Borreliosis in Children With Facial Palsy.

BACKGROUND: Lyme borreliosis (LB) is a common cause of acute facial palsy in children living in endemic areas for Borrelia burgdorferi. The need for lumbar puncture in diagnostics of LB in children with facial palsy has been questioned. Our aim was to evaluate the prevalence of LB and the diagnostic value of a cerebrospinal fluid (CSF) sample among children with an acute facial palsy. METHODS: We collected medical records and laboratory data of children and adolescents 0-16 years of age (n = 94) diagnosed with facial palsy between 2002 and 2016 in the Turku University Hospital. A positive B. burgdorferi serology in serum or CSF or a positive B. burgdorferi polymerase chain reaction in CSF were considered as signs of definite LB. C-X-C motif chemokine ligand 13 (CXCL13) values were measured in CSF samples from 28 children during 2014-2016. RESULTS: Lumbar puncture was performed on 84 of 94 children with facial palsy. LB was confirmed in 29 of 42 children with, and in 4 of 42 without, pleocytosis. The sensitivity and specificity of pleocytosis to predict LB were 88% (95% confidence interval, 78%-98%) and 75% (62%-88%), respectively, and the positive and negative predictive values were 69% (55%-83%) and 90% (81%-99%), respectively. An increased CSF CXCL13 value had 67% (51%-83%) sensitivity and 100% specificity for LB. CONCLUSIONS: Because serum serology can be negative at presentation, lumbar puncture is a valuable tool when diagnosing LB among children with facial palsy. Pleocytosis and increased protein and CXCL13 values in the CSF suggest LB as the cause of facial palsy.

Neurobrucellosis in a 9-year-old girl.

Brucellosis is a zoonotic multi-organ infectious disease most frequent in developing countries. Neurobrucellosis a quite rare but serious complication of brucellosis in the pediatric age group manifests with different neurological symptoms and signs. In the present case a 9-year-old girl was referred to our centre with a 9-months history of headache and back pain, facial nerve palsy and right upper limb weakness. She had undergone ventriculoperitoneal shunting surgery due to communicating hydrocephalus. Magnetic resonance imaging revealed a spinal extramedullary intradural mass, two epidural collections in the cervical spine and thickening/abnormal enhancement in the basal cisterns with invasion to medulla and pons. The patient's serum and cerebrospinal serologic tests were found positive for brucellosis. The patient was successfully treated by anti-brucella antibiotic therapy.

Borrelia burgdorferi sensu lato infection in patients with peripheral facial palsy.

The aims of the study were to determine the frequency of borrelial infection in patients with peripheral facial palsy (PFP) and to compare clinical and laboratory characteristics of patients with borrelial PFP and patients with PFP of unknown etiology. Adult patients with PFP who presented at our department between January 2006 and December 2013 qualified for the study if they had undergone lumbar puncture and also been tested for the presence of borrelial IgM and IgG antibodies in serum and cerebrospinal fluid (CSF) in indirect chemiluminescence immunoassay. Patients with PFP who had obvious signs/symptoms indicating a disease other than Lyme borreliosis (LB) were excluded. Patients who qualified for the study were classified into three groups according to the clinical and microbiological criteria: those having confirmed LB, those with possible LB, and those with PFP of unknown etiology. Of 589 patients diagnosed with PFP during the eight-year period, 436 patients (240 males, 196 females) with median age 42.5 years (15-87 years) qualified for the study. Among these patients, 64 (14.7%) fulfilled criteria for confirmed LB, 120 (27.5%) had a diagnosis of possible LB, and in 252 (57.8%) the cause of their PFP remained unknown. When compared with patients with unknown cause of PFP, the patients with confirmed LB were older, more often presented in summer, more often reported tick bites, more frequently had LB in the past, more often complained of constitutional symptoms and radicular pain, and more often had bilateral palsy and CSF pleocytosis. Among the patients with possible LB and patients with unknown cause of PFP there were no differences in frequency of constitutional symptoms, radicular pain, bilateral palsy or CSF pleocytosis. Presentation in summer, tick bites, constitutional symptoms and radicular pain, bilateral palsy, and CSF pleocytosis strongly suggest borrelial etiology of PFP.

Neurosyphilis presenting as cranial nerve palsy, an entity which is easy to miss.

Neurosyphilis is a rare disease that until the 2000s was almost eradicated due to population awareness of HIV and efficient treatment. Since then, the prevalence of the entity is rising due to risk-associated behaviour such as unprotected intercourse. Neurosyphilis is still a difficult entity to diagnose especially when combined with acute HIV infection which can influence the usual clinical course of disease. In rare occasions, both acute HIV and early syphilis infection can present as mono or multiple cranial nerve palsies. This case demonstrates a rare manifestation of misdiagnosed early syphilis infection combined with acute HIV infection in a 34-year-old man with prior history of unprotected sex with men.

[Mycoplasma pneumoniae: neurologic manifestations and diagnostic controversies. A case report]. / Mycoplasma pneumoniae: manifestaciones neurológicas y controversias diagnósticas. A propósito de un caso.

Mycoplasma pneumoniae (Mp) is responsible for 30% of the respiratory manifestations of the general population. Pneumonia occupies the first place within this group. Among the extra-respiratory forms (40%), the neurological ones are the most frequent. Meningoencephalitis and aseptic meningitis are the most common. The presentation of more than one clinical variant in the same patient associated with primoinfection by Mp is possible. In relation to the serological diagnosis, controversies in interpretation sometimes occur. This is a 7-year-old girl with conjunctival injection, cervical adenopathy, photophobia with bilateral papilla pseudoedema, and scaly rash that develops peripheral facial paralysis and aseptic meningitis. We will discuss diagnostic controversies.

Mycoplasma pneumoniae (Mp) es el agente causal de un 30% de las manifestaciones respiratorias de la población general. La neumonía ocupa el primer lugar dentro de este grupo. Las manifestaciones neurológicas representan las formas más frecuentes de presentación clínica extrapulmonar (40%). Las encefalitis y meningoencefalitis son las formas más habituales de sintomatología neurológica asociada a infección por Mp. La presentación de más de una variante clínica en un mismo paciente asociada a primoinfección por Mp es posible. El diagnóstico serológico plantea, habitualmente, controversias en su interpretación. A partir del caso de una niña de 7 años con inyección conjuntival, adenopatía cervical, rash descamativo y fotofobia con "pseudoedema de papila bilateral", que desarrolla durante su evolución parálisis facial periférica y meningitis aséptica, se analizarán las controversias que se plantean en relación con la interpretación diagnóstica asociada al compromiso neurológico por Mp.