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.

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.

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.

Seroprevalence of Lyme Disease in Children With Facial Nerve Palsy.

This retrospective chart review examined children with documented Lyme disease serology in New Jersey aged <21 years presenting with facial nerve palsy. The presence of symptoms including tick bite, fever, headache, and arthritis was recorded. Data were categorized based on demographic factors, and multivariate regression was employed. We enrolled 122 children, 54% female (mean age of 11.4 ± 5.1 years); 22.1% had Lyme disease. Fever was a significant predictor of Lyme disease (P = .01), confirmed by multivariate regression (odds ratio [OR] = 16.11, 95% confidence interval [CI] = 2.04, 366.14), as was male gender (P = .01, OR = 3.68, 95% CI = 1.21, 12.89). This association held especially true in Lyme-endemic regions (prevalence ≥ 0.35). The combination of headache with fever was also significantly predictive (P = .01). We found no significant predictive value in the remaining symptoms. These findings suggest that clinical predictors may be useful in diagnosing Lyme disease and initiating early empiric treatment.

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.

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.

Diagnostic value of cerebrospinal fluid examination in children with peripheral facial palsy and suspected Lyme borreliosis.

Our objective was to determine the diagnostic value of CSF examinations in the diagnosis of neuroborreliosis in children with peripheral facial palsy (PFP). Paired serum and CSF samples from 21 children with PFP were investigated for antibody responses to Borrelia burgdorferi antigens using three different ELISA systems and one Western blot assay. Twenty of the children (95%) had detectable immunoglobin (Ig) M or IgG in the acute-phase serum, but discrepancies between serologic assays were noted in 33% for IgM and 22 to 50% for IgG. Intrathecal specific-antibody production was detected in five of the 20 seropositive children (25%). These five patients showed seroconversion in convalescent sera in at least one assay. Similar seroconversion suggesting recent infection with B. burgdorferi was observed in eight of the 10 children (80%) without intrathecal specific-antibody production, from whom convalescent serum samples could be obtained. All patients with intrathecal antibodies or seroconversion had shown lymphocytic pleocytosis in the acute phase of PFP. In the acute phase of PFP the detection of intrathecal production of antibodies to B. burgdorferi allows prompt diagnosis of neuroborreliosis. For patients with lymphocytic pleocytosis but no detectable intrathecal antibodies, analysis of convalescent serum may help to establish this diagnosis.

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.

Adult T-cell leukemia/lymphoma with initial deafness.

In July 1995, a 43-year-old Japanese man presented with deafness in the right ear. On hospital admission, he had deafness in both ears and right facial palsy. Variously sized lymphoid cells with convoluted nuclei were observed in the cerebrospinal fluid. Surface marker analysis revealed monoclonality of T lymphocytes in the spinal fluid. Similar abnormal cells were observed in peripheral blood and bone marrow. Biopsy specimens of the stomach and prostate showed tight proliferation of large lymphoid cells in the interstitium and epithelium. Antibody against human T-lymphotrophic virus type 1 (HTLV-1) was present. The diagnosis of non-Hodgkin's lymphoma, diffuse type, was made. Seven months later, the patient died of sepsis. Autopsy revealed multiple lymphadenopathy in the abdomen and the infiltration of atypical lymphocytes to the pancreas, kidneys, and other organs. A monoclonal band of HTLV-1 provirus was detected by Southern blot analysis. To our knowledge, this is the first report of adult T-cell leukemia/lymphoma with auditory nerve abnormalities as the initial symptom.

Tumor necrosis factor-alpha response and herpesvirus infection in Bell's palsy.

OBJECTIVES: To attempt early diagnosis of patients with Bell's palsy by detection of herpesviral DNA in body fluids, and to investigate whether tumor necrosis factor-alpha (TNF-alpha), a cytokine associated with demyelination, is involved in the inflammatory response in this disease. STUDY DESIGN: Eleven patients with acute facial palsy admitted within 1 week after onset of the disease were followed in a consecutive prospective study. METHODS: Antibodies reactive to herpesviruses were determined by enzyme-linked immunosorbent assay in serum samples from acute and convalescent (> 2-week interval) cases. Intrathecal antibody response was investigated by immunoblotting. Polymerase chain reaction amplification of herpesviral DNA was attempted from samples of serum, cerebrospinal fluid, tear fluid, and saliva TNF-alpha and its soluble receptors (types I and II) were assessed in serum and cerebrospinal fluid samples. RESULTS: Ten of the 11 patients demonstrated serologic evidence of herpesviral primary infection or reactivation, supporting the evidence that herpesviruses are the most prevalent etiologic agents in Bell's palsy. Despite this, DNA amplifications by polymerase chain reaction were negative for herpesviruses in the body fluids tested. TNF-alpha concentrations were significantly elevated in serum, as compared with controls. Only one patient had a remaining facial nerve dysfunction at follow-up after 3 months. CONCLUSION: The absence of herpes DNA in body fluids in the acute stage of serologically confirmed Bell's palsy suggests that viral replication is transient in cases with an early restoration of the facial nerve function. The elevated serum levels of TNF-alpha indicate that this cytokine might be a pathogenetic factor related to the demyelination in this disease.

Plasma endothelin level in the acute stage of Bell palsy.

OBJECTIVE: To determine the plasma endothelin level in the acute stage in patients with Bell palsy (based on the hypothesis that endothelin, which is a potent vasoconstrictor, may play a role in the mechanism of the onset of facial nerve paralysis and in view of the fact that the etiology of Bell palsy is still a maze of unknowns). DESIGN: The study involved 62 patients with the acute stage (tested within 10 days of onset) of Bell palsy (i.e., idiopathic acute peripheral facial paralysis) and an additional 36 healthy persons who served as control subjects. To determine the content of endothelin, 2 ml of plasma samples was collected from each subject. Endothelin-1 was extracted and analyzed by a radioimmunoassay by using anti-endothelin-1 antibody. SETTING: Nihon University Itabashi Hospital, a referral and institutional center in Tokyo, Japan. RESULTS: The patients who were suffering from Bell palsy exhibited a statistically significant (P < .01) increase in the endothelin level compared with that in the 36 normal control subjects. An age-matched comparison (ranges, 20-29 years and 30-39 years) of patients with Bell palsy with normal control subjects revealed a significant difference between the normal group and the group with Bell palsy in the plasma endothelin level for both age groups that were tested (P < .01). The mean value of the endothelin level in patients with Bell palsy was maximal on day 5, and the percentage of patients with abnormally elevated endothelin levels was 100% from days 6 to 9. CONCLUSION: Endothelin, which has potent vasoconstrictive effects, may contribute to the pathogenesis of the microcirculatory impairment that occurs in patients with Bell palsy, mainly by promoting secondary ischemia.

Interferon anti-viral system in Bell's palsy.

Thirty-two consecutive Bell's palsy patients were examined clinically and virologically. All the patients were assayed for serum interferon (IFN) levels at onset of the disease and most of them had repeated IFN assays during the course of the disease. In 15 patients the peripheral blood mononuclear cells (PBMC) were also evaluated for the presence of an anti-viral state (AVS). In viral infections, blood IFN levels are invariably increased, and the PBMC are usually in an AVS. Twenty-three patients (72%) had increased serum IFN values or were in an AVS. The mean serum IFN level was 95 U/ml (normal less than or equal to 16 U/ml) during the first 3 days following onset of the disease, declining later to normal values from the seventh day onwards. No patient had increased antibody titers to herpes simplex and varicella-zoster viruses. The increased blood IFN levels and the AVS of blood cells are compatible with an acute viral infection. It is concluded that the etiology of Bell's palsy is most likely an acute viral infection, possibly due to an unidentified or unusual virus, or to reactivation of a latent virus.

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.

[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.

[Evaluation of the plasma endothelin levels in facial nerve paralysis].

The etiology of so-called Bell's palsy is still unknown. Based on the hypothesis that endothelin, with its potent vasoconstricting action, is in some way involved in the etiological mechanism of facial nerve paralysis, we measured the blood endothelin levels of 62 patients with Bell's palsy in the acute stage (within one week following onset). The measurements were extended to include 10 patients with Ramsay Hunt syndrome and 14 patients with zoster sine herpete. To determine endothelin content, blood samples were drawn into blood collecting tubes, containing EDTA-2Na+aprotinin at any time desired during the day. The samples were immediately chilled and centrifuged to separate the plasma portion, and 2ml of this plasma sample was preserved by freezing. ET-1 was extracted using silica-ODS and analyzed by RIA using anti-ET 1 antibody. An age-matched comparison between the Bell's palsy patients and 36 normal individuals using the Mann-Whitney U test showed a significant difference (p < 0.01). The patients with Ramsay Hunt syndrome and zoster sine herpete also tended to have elevated endothelin levels. This finding suggests that disorders of the microcirculatory system in which endothelin is in some way involved may play a role in the pathogenesis of facial nerve paralysis.