Granulomatosis with polyangiitis presenting with unilateral facial nerve palsy and nasal septum perforation.

Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis of unknown cause that has several systemic manifestations. The disease is characterised by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can involve the central nervous system. This case report presents a case of GPA with facial nerve palsy as the first manifestation of the disease, which has been rarely reported in the medical literature.

Our experience on the management of acute mastoiditis in pediatric acute otitis media patients.

OBJECTIVE: The incidence of complications due to acute otitis media (AOM) in childhood has decreased significantly with the use of new antibiotics in recent years. However, acute mastoiditis (AM) is still the most common complication that can lead to further intracranial conditions with high morbidity. Our study aimed to evaluate the clinical characteristics of children with AM and identify possible indicators for further intracranial complications associated with this condition. METHODS: Children hospitalized in our clinic with a diagnosis of AM were reviewed. Demographic data, disease-related symptoms, types of complications accompanied by AM, medical/surgical treatments modalities, and culture results were screened. The patients were divided into two groups as those with and without intracranial complications (ICCs). Routine complete blood count tests, biochemical analysis, and C-reactive protein (CRP) level measurement were evaluated and compared between the groups. RESULTS: Of the 28 AM patients, five (17.9%) had isolated AM. Complications associated with AM included sub-periosteal abscess (28.6%), facial paralysis (25%), meningitis (17.9%), meningitis with sigmoid sinus thrombosis (7.1%), and meningitis with cerebellar abscess (3.6%). Eight patients developed ICCs (28.6%), of whom three had more than one complication. Ceftriaxone was found to be the first-line medical treatment (57.1%). Streptococcus pneumoniae was the most common pathogen isolated from the cultures (42.9%). Three patients (10.7%) were treated non-surgically, eight (28.6%) with myringotomy and ventilation tube (VT) insertion, eight patients (28.6%) with abscess drainage and VT insertion, and nine (32.1%) with cortical mastoidectomy and VT insertion. There was no significant difference between the patients with and without ICCs in terms of complete blood count parameters. The CRP level and the CRP-albumin ratio were significantly higher in patients with ICCs than those without these complications (p < 0.001). CONCLUSION: AM remains to be the most common complication of AOM in childhood and can lead to further life-threatening conditions. Additional interventions according to the type of the complication with VT insertion is safe and effective in the management of AM. In patients with AM, it is of great importance to determine whether there is an accompanying ICC. The CRP-albumin ratio is a simple and reliable calculation to detect ICCs in patients with AM.

'Twenty syndrome' in neuromyelitis optica spectrum disorder.

A 30-year-old woman presented with recurrent hiccups, vomiting and painful diminution of vision and gait instability for 1 day. She had one-and-a-half syndrome, bilateral seventh cranial nerve paresis with bilateral symptomatic optic neuritis and left-sided ataxic haemiparesis. We described her disorder as the 'twenty syndrome' (11/2+7+7+2+2+½=20). MRI of her brain revealed demyelination predominantly in right posterolateral aspect of pons, medulla and bilateral optic nerves. Serum antiaquaporin-4 antibody came out positive. Thus, she was diagnosed as neuromyelitis optica spectrum disorder (NMOSD). She responded brilliantly to immunosuppressive therapy. This is the first ever reported case of the 'twenty syndrome' secondary to cerebral NMOSD.

Bilateral Peripheral Facial Paralysis Combined with HIV Meningitis During Acute HIV-1 Infection: A Case Report.

Here we reported a Chinese case of bilateral peripheral facial paralysis (PFP) in human immunodeficiency virusc (HIV) infected population. A 38-year-old homosexual male patient was referred to our hospital for bilateral facial paralysis. 21 days prior to admission he had developed high fever, chills, headache, fatigue, general malaise, nausea and vomiting. Neurological examination revealed bilateral ptosis of lower lip and cheeks, as well as failure of bilateral eyes closure. Analysis of cerebrospinal fluid (CSF) revealed pleocytosis, a marked rise of micro total protein and a marked rise of intrathecal lgG synthesis. The result of HIV-1 serology was positive by ELISA and that was confirmed by western blot. His CD4 + cell count was 180 cells/mm 3. HIV-1 viral load in CSF was almost 10 times higher than that in plasma. The patient's condition improved steadily and experienced complete resolution of bilateral PFP after 2 months.

Atypical clinical manifestations of Miller Fisher syndrome.

Miller Fisher syndrome (MFS) is characterized by a clinical triad of ophthalmoplegia, ataxia, and areflexia, and is closely associated with serum anti-GQ1b antibody. Although the clinical triad is the cardinal diagnostic clue, a variety of other symptoms and signs beyond the triad have been reported. To elucidate the frequency and characteristics of atypical clinical manifestations of MFS, we recruited 38 patients with MFS and evaluated the symptoms or signs beyond the classic triad. Eleven (29%) of 38 patients had atypical clinical manifestations of MFS such as headache (n = 6), delayed facial palsy (n = 3), divergence insufficiency (n = 2), and taste impairment (n = 2). Headache was localized to the periorbital (n = 3), temporal (n = 2), or whole (n = 1) area. Only one of them showed bilateral papilledema and an elevated opening pressure in cerebrospinal fluid analysis. Delayed facial palsy developed after the other signs have reached nadir (n = 1) or started to improve (n = 2), and did not follow a pattern of descending paralysis with other cranial neuropathies. Two patients showed divergence insufficiency without external ophthalmoplegia, and another two had taste impairment over the entire tongue without the other signs of facial and glossopharyngeal nerve involvements. Our study shows that approximately 30% of MFS patients can have atypical clinical manifestations beyond the classic triad. These results reflect the broad clinical spectrum of MFS, and might be associated with the presence of additional antiganglioside antibodies besides anti-GQ1b in patients with MFS.

Novel pre-therapeutic scoring system using patient and haematological data to predict facial palsy prognosis.

OBJECTIVES: We describe a novel scoring system, the facial Palsy Prognosis Prediction score (PPP score), which we test for reliability in predicting pre-therapeutic prognosis of facial palsy. We aimed to use readily available patient data that all clinicians have access to before starting treatment. DESIGN: Multicenter case series with chart review. SETTING: Three tertiary care hospitals. PARTICIPANTS: We obtained haematological and demographic data from 468 facial palsy patients who were treated between 2010 and 2014 in three tertiary care hospitals. Patients were categorised as having Bell's palsy or Ramsey Hunt's palsy. MAIN OUTCOME MEASURES: We compared the data of recovered and unrecovered patients. PPP scores consisted of combinatorial threshold values of continuous patient data (eg platelet count) and categorical variables (eg gender) that best predicted recovery. We created separate PPP scores for Bell's palsy patients (PPP-B) and for Ramsey Hunt's palsy patients (PPP-H). RESULTS: The PPP-B score included age (≥65 years), gender (male) and neutrophil-to-lymphocyte ratio (≥2.9). The PPP-H score included age (≥50 years), monocyte rate (≥6.0%), mean corpuscular volume (≥95 fl) and platelet count (≤200 000 /µL). Patient recovery rate significantly decreased with increasing PPP scores (both PPP-B and PPP-H) in a step-wise manner. PPP scores (ie PPP-B score and PPP-H score) ≥2 were associated with worse than average prognosis. CONCLUSIONS: Palsy Prognosis Prediction scores are useful for predicting prognosis of facial palsy before beginning treatment.

Vibrissal paralysis produces increased corticosterone levels and impairment of spatial memory retrieval.

This research was aimed at establishing how the absence of active whisking in rats affects acquisition and recovery of spatial memory. The mystacial vibrissae were irreversibly paralyzed by cutting the facial nerve's mandibular and buccal branches bilaterally in the facial nerve lesion group (N=14); control animals were submitted to sham-surgery (N=15). Sham-operated (N=11) and facial nerve-lesioned (N=10) animals were trained (one session, eight acquisition trials) and tested 24h later in a circular Barnes maze. It was found that facial nerve lesioned-animals adequately acquired the spatial task, but had impaired recovery of it when tested 24h after training as compared to control ones. Plasma corticosterone levels were measured after memory testing in four randomly chosen animals of each trained group and after a single training trial in the maze in additional facial nerve-lesioned (N=4) and sham-operated animals (N=4). Significant differences respecting the elevation of corticosterone concentration after either a single training trial or memory testing indicated that stress response was enhanced in facial nerve-lesioned animals as compared to control ones. Increased corticosterone levels during training and testing might have elicited the observed whisker paralysis-induced spatial memory retrieval impairment.

Pretreatment Hematologic Findings as Novel Predictive Markers for Facial Palsy Prognosis.

OBJECTIVE: To examine the relationship between prognosis of 2 different facial palsies and pretreatment hematologic laboratory values. STUDY DESIGN: Multicenter case series with chart review. SETTING: Three tertiary care hospitals. SUBJECTS AND METHODS: We examined the clinical records of 468 facial palsy patients who were treated with an antiviral drug in combination with either oral or intravenous corticosteroids in participating hospitals between 2010 and 2014. Patients were divided into a Bell's palsy group or a Hunt's palsy group. We used the Yanagihara facial nerve grading system to grade the severity of facial palsy. "Recovery" from facial palsy was defined as achieving a Yanagihara score ≥36 points within 6 months of onset and having no accompanying facial contracture or synkinesis. We collected information about pretreatment hematologic findings, demographic data, and electrophysiologic test results of the Bell and Hunt group patients who recovered and those who did not. We then compared these data across the 2 palsy groups. RESULTS: In the Bell's palsy group, recovered and unrecovered patients differed significantly in age, sex, electroneuronography score, stapedial muscle reflex, neutrophil rate, lymphocyte rate, neutrophil-to-lymphocyte ratio, and initial Yanagihara score. In the Hunt's palsy group, recovered and unrecovered patients differed in age, electroneuronography score, stapedial muscle reflex, monocyte rate, platelet count, mean corpuscular volume, and initial Yanagihara score. CONCLUSIONS: Pretreatment hematologic findings, which reflect the severity of inflammation and bone marrow dysfunction caused by a virus infection, are useful for predicting the prognosis of facial palsy.

Concurrent idiopathic vestibular syndrome and facial nerve paralysis in a cat.

CASE REPORT: A 4-year-old male neutered Domestic Medium-hair cat was referred for right head tilt and ataxia of 2 weeks duration. On examination it was determined that the cat had right facial nerve paralysis and peripheral vestibular signs. Haematology and serum biochemical testing were performed in addition to magnetic resonance imaging of the brain and ears, and cerebrospinal fluid analysis. An underlying condition was not identified. A diagnosis of idiopathic vestibular syndrome and concurrent idiopathic right facial nerve paralysis was consequently made. The cat was re-evaluated over the following weeks and was determined to have complete resolution of clinical signs within 7 weeks. CONCLUSION: Vestibular dysfunction and concurrent facial nerve paralysis have previously been reported in the cat, but not of an idiopathic nature.

[Moxibustion at Baihui (GV 20) for intractable facial paralysis and its impacts on immunoglobulin].

OBJECTIVE: To observe the efficacy of moxibustion at Baihui (GV 20) on intractable facial paraly sis and the impacts on immune globulin IgA, IgG and IgM. METHODS: One hundred and twenty cases of intractable facial paralysis that was in compliance with the inclusive criteria were randomized into a moxibustion group and an acupuncture group, 60 cases in each one. In the moxibustion group, moxibustion was applied at Baihui (GV 20). In the acupuncture group, the patients were treated with acupuncture, once a day, the treatment of 15 days made one session in two groups. Before treatment and after 2 sessions treatment, the levels of IgA, IgG and IgM were detected respectively for the patients in two groups and compared. RESULTS: The difference in the levels of IgA, IgG and IgM was not significant for the patients between two groups (all P > 0.05) before treatment, but the levels of all three indices were increased significantly as compared with the normal reference values (all P < 0.05). Componed before the treatment, the levels of IgA, IgG and IgM were reduced significantly in two groups after treatment, indicating the significant difference (all P < 0.05). In comparison between two groups after the treatment, the levels of IgA, IgG and IgM in the moxibustion group were lower significantly than those in the acupuncture group, presenting the significant difference after treatment (all P < 0.05). CONCLUSION: The ottack of intractable facial paralysis is relevant with the abnormal increase of immunoglobulin. Moxibustion at Baihui (GV 20) reduces significantly the levels of IgA, IgG and IgM for the patients with intractable facial paraly sis, which is probably one of the mechanisms in the treatment of intractable facial paralysis.

Bilateral facial nerve palsy due to otitis media associated with myeloperoxidase-antineutrophil cytoplasmic antibody.

Otitis media (OM) is well known as a common feature of proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA)-related Wegener granulomatosis, but is a very rare condition in myeloperoxidase ANCA (MPO-ANCA)-related vasculitis. In addition, there have been a few reports showing an association of MPO-ANCA-positive OM with cranial polyneuropathy. In this report, we describe 2 patients with bilateral facial nerve palsy due to MPO-ANCA-related OM. One patient also had bilateral trigeminal neuropathy, pachymeningitis and MPO-ANCA-related glomerulonephritis, whereas the other showed isolated bilateral facial nerve palsy with OM. In both the patients, treatment with prednisolone and immune-suppressant drugs resulted in an improvement of OM and cranial polyneuropathy. Physicians should be aware that MPO-ANCA-positive OM can cause bilateral facial nerve palsy.

Differential diagnosis of recurrent or bilateral peripheral facial palsy.

OBJECTIVE: To describe the differential diagnosis of recurrent or bilateral peripheral facial palsy. METHOD: Case report and literature review. RESULTS: Two patients with recurrent, alternating, peripheral facial palsy are described. In both patients, additional investigation was performed to search for a specific diagnosis. In the first patient, only a positive family history was found, indicating a possible familial susceptibility. In the other patient, diabetes mellitus and hypertension were identified as risk factors. CONCLUSION: There is an important and extensive differential diagnosis of recurrent or bilateral facial palsy. However, in a large proportion of patients the cause remains unknown.

Autoantibodies to human manganese superoxide dismutase (MnSOD) in children with facial palsy due to neuroborreliosis.

AIM: Acute peripheral facial palsy due to neuroborreliosis is associated with a distal neuritis. In patients with Lyme disease the activity of antioxidant enzymes is decreased. With respect to the pathogenesis of neuroborreliosis, sera of children with acute peripheral facial palsy were investigated for autoantibodies against human manganese superoxide dismutase (MnSOD), which were suspected of raising the oxidative injury of infected tissues. METHODS: Sera of 20 children with acute peripheral palsy with neuroborreliosis, sera of 20 children with facial palsy without reference to Lyme disease and sera of 14 blood donors were tested for antibodies against human MnSOD using an ELISA. RESULTS: The concentrations of IgM autoantibodies to MnSOD of the children with neuroborreliosis were significantly increased, compared with the two control groups. CONCLUSIONS: We propose that the antibodies detected block the protective effects of MnSOD resulting in an increased oxidative inflammation.

[An investigation of patients with acute peripheral facial palsy with regard to Lyme disease]. / Akut periferik fasyal paralizili olgularin Lyme hastaligi açisindan incelenmesi.

OBJECTIVES: We investigated the role of Borrelia burgdorferi in the etiology of idiopathic acute peripheral facial palsy. PATIENTS AND METHODS: Nineteen patients (15 females, 4 males; mean age 38 years; range 14 to 61 years) with acute peripheral facial palsy were studied. Following routine otolaryngologic examination, all the patients underwent taste, Schirmer, and stapedial reflex tests to evaluate the level of the palsy. Laboratory examination included routine biochemistry analysis, serum C-reactive protein, rheumatoid factor, and erythrocyte sedimentation rate. Anti-Borrelia burgdorferi IgM and IgG antibodies were sought by ELISA in venous blood samples. RESULTS: Of the patient group, acute facial palsy was localized on the right in 12 patients (63.2%), and on the left in seven patients (36.8%). None of the patients had bilateral involvement. No IgM or IgG seropositivity for Borrelia burgdorferi was detected in the serum samples. CONCLUSION: In highly endemic areas, it may be helpful to detect or even to eliminate Lyme disease through screening of serum in patients with acute peripheral palsy.

Varicella zoster and Borrelia burgdorferi are the main agents associated with facial paresis, especially in children.

BACKGROUND: The etiology of facial paresis (FP) often remains unresolved. Yet, a microbial association is frequently suspected. OBJECTIVE: To evaluate the infectious etiology of FP by using sensitive tests. STUDY DESIGN: We studied the serum and cerebrospinal fluid of 42 patients diagnosed with idiopathic peripheral facial paresis using sensitive serological methods and nucleic acid detection and for reference, 42 patients with other neurological disorders (OND) matched for age, sex, season and geographical area. RESULTS: Varicella zoster virus and Borrelia burgdorferi accounted for 56% of all associated agents in children with FP compared with 11% of OND (P=0.01). In adults, the respective numbers were 29 and 13%. Other treatable etiological agents, Chlamydia pneumoniae and Mycoplasma pneumoniae, accounted for 11% in children and 8% in adults and with the same prevalence between patients with FP and OND. CONCLUSIONS: Microbes, with specific therapy available accounted for 52% of all associated agents in the patients with FP when compared with 26% in controls with OND (P=0.04). Based on this, we conclude that the patients with FP may benefit from antimicrobial therapy.

Lyme borreliosis and facial paralysis--a prospective analysis of risk factors and outcome.

PURPOSE: To evaluate the incidence of Lyme borreliosis in patients with acute idiopathic facial paralysis with special emphasis on the risk factors that explain the poor outcome of facial paralysis and occurrence of Lyme borreliosis. MATERIALS AND METHODS: During a 2-year period, we prospectively studied 503 consecutive patients with acute idiopathic facial paralysis for the presence of Lyme borreliosis. We screened the patients for antibodies to Borrelia burgdorferi and for symptoms or signs related to Lyme borreliosis. Chi-square and logistic regression tests were used for the statistical analysis. Special attention was paid to strict criteria for the diagnosis of Lyme borreliosis. RESULTS: Eleven (2.2%) of the 503 patients with facial paralysis had Lyme borreliosis. Fever, headache, pharyngalgia, enlarged cervical lymph nodes, bilateral paralysis, and arthralgia were more common in patients with Lyme borreliosis than in those without it. In the logistic regression modeling the best combination of explanatory variables for predicting the occurrence of Lyme borreliosis included summer season at the onset of facial paralysis, presence of enlarged cervical lymph nodes, and arthralgia. The best combination of explanatory variables to predict the poor outcome of facial paralysis was total paralysis of facial nerves, recurrent facial paralysis, and hyperacusis. CONCLUSIONS: Lyme borreliosis is an important infectious cause of facial paralysis. In our study, 11 of 503 patients with acute idiopathic facial paralysis had Lyme borreliosis. The screening for serum antibodies in addition to the thorough evaluation of the history of the patient and of the patient's clinical signs or symptoms possibly linked with Lyme borreliosis, are essential when diagnosing Lyme borreliosis.

Herpes virus reactivation and serum tumor necrosis factor-alpha levels in patients with acute peripheral facial palsy.

Recent studies have shown that tumor necrosis factor-alpha (TNF-alpha) plays an important regulatory role in several inflammatory and infectious diseases. In the present study, we evaluated serum TNF-alpha levels of patients with acute peripheral facial palsy using an ELISA method. We examined sera from each group (n = 25 per group) of patients with herpes simplex virus type 1 reactivation (HSV-1). varicella-zoster virus (VZV) reactivation, and with no HSV or VZV reactivation. We also tested the sera of 25 normal controls. No significant difference was found between the serum TNF-alpha levels in facial palsy and controls. No correlation was found between serum TNF-alpha levels in cases with HSV-1 or VZV reactivation and with no HSV-1 or VZV reactivation. These results indicate that serum TNF-alpha levels are not affected by HSV-1 or VZV reactivation in patients with facial palsy.