Case report: Bilateral facial palsy with paresthesias and positive anti-GT1a antibodies.

Bilateral facial palsy with paresthesia (FDP) is a rare variant of GBS, characterized by simultaneous bilateral facial palsy and paresthesia of the distal limbs. Mounting evidence indicates that the presence of anti-GT1a IgG has a pathogenic role as an effector molecule in the development of cranial nerve palsies in certain patients with GBS, whereas anti-GT1a antibody is rarely presented positive in FDP. Here, we report the case of a 33-year-old male diagnosed with FDP presented with acute onset of bilateral facial palsy and slight paresthesias at the feet as the only neurological manifestation. An antecedent infection with no identifiable reason for the fever or skin eruptions was noted in the patient. He also exhibited cerebrospinal fluid albuminocytologic dissociation and abnormal nerve conduction studies. Notably, the testing of specific serum anti-gangliosides showed positive anti-GT1a IgG/IgM Ab. The patient responded well to intravenous immunoglobulin therapy. This case brings awareness to a rare variant of GBS, and provides the first indication that anti-GT1a antibodies play a causative role in the development of FDP. The case also suggests that prompt management with IVIG should be implemented if FDP is diagnosed.

Role of Biomarkers as Prognostic Factors in Acute Peripheral Facial Palsy.

Acute peripheral facial palsy (APFP), including Bell's palsy and Ramsay Hunt syndrome, is a disease that affects daily life through facial motor dysfunction, causing psychological problems. Various tests to evaluate prognosis have been studied; however, there are no validated predictive biomarkers to guide clinical decision making. Therefore, specific biomarkers that respond to treatment are required to understand prognostic outcomes. In this review, we discuss existing literature regarding the role of APFP biomarkers in prognosis and recovery. We searched the PubMed, EMBASE, and Cochrane Library databases for relevant papers. Our screening identified relevant studies and biomarkers correlating with the identification of predictive biomarkers. Only studies published between January 2000 and October 2021 were included. Our search identified 5835 abstracts, of which 35 were selected. All biomarker samples were obtained from blood and were used in the evaluation of disease severity and prognosis associated with recovery. These biomarkers have been effective prognostic or predictive factors under various conditions. Finally, we classified them into five categories. There is no consensus in the literature on the correlation between outcomes and prognostic factors for APFP. Furthermore, the correlation between hematologic laboratory values and APFP prognosis remains unclear. However, it is important to identify new methods for improving the accuracy of facial paralysis prognosis prediction. Therefore, we systematically evaluated prognostic and potentially predictive APFP biomarkers. Unfortunately, a predictive biomarker validating APFP prognosis remains unknown. More prospective studies are required to reveal and identify promising biomarkers providing accurate prognosis.

'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.

Confirmed Ceylon krait (Bungarus ceylonicus) envenoming in Sri Lanka resulting in neuromuscular paralysis: a case report.

BACKGROUND: Ceylon krait (Bungarus ceylonicus) is a venomous elapid snake endemic to Sri Lanka. It inhabits shaded home gardens and forests in the wet zone of Sri Lanka and might creep into houses in the night. Despite frequent encounters with humans, reports of envenoming are very rare. CASE PRESENTATION: We report a case of a 26-year-old Sri Lankan Sinhalese man with confirmed Ceylon krait envenoming presenting with bilateral partial ptosis, ophthalmoplegia, facial muscle weakness, and dysphagia. Single fiber electromyography and repetitive nerve stimulation confirmed neuromuscular paralysis. He was administered polyvalent anti-venom serum immediately following admission without a prompt clinical response. Complete recovery was observed 3 days following the bite. CONCLUSIONS: Because of the rarity of envenoming, precise and detailed information on the clinical manifestations following envenoming is lacking. However, Ceylon krait bite can be potentially fatal; so, treating physicians should be aware of species identification, habitat, and biting habits and clinical presentation of envenoming of Ceylon krait. This case report adds knowledge to the existing limited literature available on Ceylon krait envenoming; a rare but potentially fatal clinical entity.

Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations.

Does delayed facial involvement implicate a pattern of "descending reversible paralysis" in Fisher syndrome?

OBJECTIVE: Delayed facial palsy (DFP) has often been described during the recovery stage of Fisher syndrome (FS), but the implications of DFP in FS pathophysiology have not been reported previously. The aim of this study was to identify the incidence and clinical course of DFP in FS, and to determine its clinical/pathophysiological implications in FS. METHODS: About 71 FS patients were enrolled from seven university-based hospitals in Korea. DFP was defined with respect to new development of unilateral or bilateral facial palsies with delayed onset after either the nadir or improvement of initial neurological signs of FS. RESULTS: Eleven of the 71 patients (16%) satisfied the definition of DFP. No other cranial palsies developed as a delayed pattern. With the exception of two patients with bulbar involvement, DFP developed after a latent period of upper-cranial neuropathies. Comparison of FS patients without and with DFP revealed no significant clinical, serological, or electrophysiological differences. All except one patient with DFP exhibited a good outcome within 1 month of follow-up. CONCLUSION: DFP was identified as a common and specific phenomenon in FS. Nearly all cases of DFP were developed in a descending manner and were associated with a good outcome, while other cranial neuropathies developed or followed as a sequential pattern. These findings suggest the involvement of so-called "descending reversible paralysis" in the pathophysiology of FS.

Guillain-Barré syndrome variant with facial diplegia and paresthesias associated with IgM anti-GalNAc-GD1a antibodies.

We herein report the case of a 19-year-old woman with facial diplegia and paresthesias (FDP) preceded by flu-like symptoms. We diagnosed the patient with a regional variant of Guillain-Barré syndrome due to decreased tendon reflexes, albuminocytological dissociation in the cerebrospinal fluid and demyelinating features on nerve conduction studies. The patient also had IgM anti-GalNAc-GD1a antibodies, and treatment with glucocorticoids was effective for treating the facial diplegia, but not paresthesia. Therefore, facial palsy may have a different pathophysiology from paresthesia or other symptoms of FDP, which responds to glucocorticoid therapy.

The expression of IL-2 and IL-4 in CD4(+) T cells from mouse lymph nodes and spleen during HSV-1-induced facial palsy.

OBJECTIVE: Herpes simplex virus 1 (HSV-1) is regarded as an important underlying cause of Bell's palsy, but the immunologic mechanism remains unknown. Here, we employed a mouse facial paralysis model to investigate the expressions of CD4(+) T lymphocytes and interleukin (IL)-2 and -4 in the left draining cervical lymph nodes (LCLN) and spleen, as well as the inhibitory effects of glucocorticoids (GCs). METHODS: HSV-1 was inoculated into the surface of the posterior auricle to generate the facial paralysis model. The paralyzed mice were divided into three groups; in one group without any treatment, mice were killed at different time points, and those in the other two groups were injected with methylprednisolone sodium succinate (MPSS) or with a combination of MPSS and GC receptor blocker (RU486). The expression levels of CD4(+) T lymphocytes and CD4(+)-IL-2(+) and CD4(+)-IL-4(+) cells in the LCLN and spleen were detected by fluorescence-activated cell sorting. RESULTS: Expression levels of CD4(+), IL-2, and IL-4 first increased then decreased in LCLN and spleen and peaked 5 and 7 days, respectively, after the manifestation of facial paralysis. All the data at the peak points were significantly different compared with control (p < 0.05), and these effects were inhibited by MPSS. CONCLUSION: Our results suggest that CD4(+), IL-2, and IL-4 participate in the HSV-1-induced facial paralysis immune response. MPSS can effectively attenuate HSV-1-mediated nervous system damage, which is associated with its inhibitory effect on expression of these inflammatory markers.

Recurrent facial nerve palsy associated with anti-GQ1b IgG antibodies.

Recurrent facial nerve palsy (RFNP) in childhood is usually considered to be a benign disorder. We report a 13-year-old female affected with RFNP in the absence of other neurologic signs, in which elevated serum IgG and IgM anti-GQ1b antibodies were detected. To our knowledge, this is the first example in the literature of RFNP, associated with anti-GQ1b IgG antibodies. The possible role of anti-GQ1b antibodies in isolated cranial neuropathy is discussed.

Immune status to polioviruses in the child population of Romania between 2002-2005 as indicated by serological investigation in cases of acute flaccid paralysis (AFP) and facial paralysis (FP) and its use as a "national reference value" to evaluate the vaccination coverage in particular groups of healthy children.

Although the European region is polio free since 2002, the risk of importation from endemic regions remains present and a high level of population immunity must be maintained. In Romania during the period 2002-2005, 101 FP cases, 91 AFP cases and 29 healthy contacts (living in groups with low social and sanitary status, relatively low vaccination coverage named "at risk") could have been investigated serologically. Antibody prevalences for poliovirus types 1, 2, and 3 were: 97.2%, 98% and 81.2% for FP cases; 96.7%, 94.5% and 85.7% for AFP cases, and 85.7%, 82.1% and 53% for the group of healthy children at risk. The risk of the emergence and spread of cVDPVs remains present especially in "at risk" groups with the gaps in immunity, even in countries where indigenous wild polioviruses have already been eradicated.

Human Lyme arthritis and the immunoglobulin G antibody response to the 37-kilodalton arthritis-related protein of Borrelia burgdorferi.

In Borrelia burgdorferi-infected C3H-scid mice, antiserum to a differentially expressed, 37-kDa spirochetal outer-surface protein, termed arthritis-related protein (Arp), has been shown to prevent or reduce the severity of arthritis. In this study, we determined the immunoglobulin G (IgG) antibody responses to this spirochetal protein in single serum samples from 124 antibiotic-treated human patients with early or late manifestations of Lyme disease and in serial serum samples from 20 historic, untreated patients who were followed longitudinally from early infection through the period of arthritis. These 20 patients were representative of the spectrum of the severity and duration of Lyme arthritis. Among the 124 antibiotic-treated patients, 53% with culture-proven erythema migrans (EM) had IgG responses to recombinant glutathione S-transferase (GST)-Arp, as did 59% of the patients with facial palsy and 68% of those with Lyme arthritis. In addition, 75 to 80% of the 20 past, untreated patients had reactivity with this protein when EM was present, during initial episodes of joint pain, or during the maximal period of arthritis. There was no association at any of these three time points between GST-Arp antibody levels and the severity of the maximal attack of arthritis or the total duration of arthritis. Thus, after the first several weeks of infection, 60 to 80% of patients had IgG antibody responses to GST-Arp, but this response did not correlate with the severity or duration of Lyme arthritis.

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.

The role of viruses in idiopathic peripheral facial palsy and cellular immune response.

PURPOSE: The purpose of this study is to investigate the role of viruses on the idiopathic peripheral facial palsy and show the interaction of immune system. MATERIALS AND METHODS: The levels of immunoglobulin (Ig) G and IgM antibodies against to varicella-zoster virus (VZV), herpes simplex virus (HSV), cytomegalovirus (CMV), Ebstein-Barr virus (EBV), and mumps virus in venous blood taken from patients in the amount of 10 mL have been investigated by enzyme-linked immunosorbent assay method. Tumor necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma), and transforming growth factor-beta (TGF-beta) were also examined. Of lymphocyte subpopulation, antibodies of CD3(+), CD4(+), CD8(+), CD19(+), and CD16(+) plus 56(+) were analyzed. RESULTS: Ten of the patients had HSV-1 IgG; 1 of the patients IgM, 5 of the patients EBV IgG, 6 of the patients VZV IgG, 1 of the patients IgM, 9 of the patients mumps IgG, 1 of the patients IgM, and finally in 7 of the patients CMV IgG antibodies were obtained. It was found that CD4(+) cell and ratio of CD4(+)/CD8(+) lower and the percentage of the CD8(+) and CD16(+) plus 56(+) cells higher compared with the control group (P < .05). The levels of TNF-alpha were lower, whereas IFN-gamma and TGF-beta1 were higher. CONCLUSION: It may be concluded from these results that VZV, HSV-1, CMV, EBV, and mumps virus play a significant role in the etiology of idiopathic peripheral facial palsy and activate the cellular immunity.

The VlsE (IR6) peptide ELISA in the serodiagnosis of lyme facial paralysis.

OBJECTIVE: Facial paralysis is a manifestation of early disseminated Lyme neuroborreliosis. In the current study, we compared the immunoglobulin G (IgG) VlsE (sixth invariant region) peptide enzyme-linked immunosorbent assay (ELISA) with the current two-tier approach of sonicate ELISA and Western blot in the serodiagnosis of Lyme facial paralysis. STUDY DESIGN: Retrospective. SETTING: Tertiary referral center. PATIENTS: Serum samples from 47 Lyme patients with facial paralysis and 86 control subjects were analyzed for IgG antibodies to VlsE peptide of Borrelia burgdorferi and for immunoglobulin M (IgM) and IgG antibodies to sonicate antigens of B. burgdorferi using the two-tier approach. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: Serum IgG antibody responses to VlsE (IR6) peptide. RESULTS: All 47 (100%) patients with facial paralysis and 4 (5%) of 86 controls had positive antibody responses to the VlsE peptide. In the two-tier test, 41 (87%) patients had positive IgM, 31 (66%) had positive IgG, and all 47 patients had positive IgM or IgG responses. Of the 86 control subjects, 2 (2%) had positive results with the two-tier test. Thus, the sensitivities of the VlsE and the two-tier tests were 100%; the specificity of the VlsE ELISA was 95% and the specificity of the two-tier test was 98%. CONCLUSIONS: The VlsE peptide ELISA showed a high sensitivity and specificity in the serological diagnosis of Lyme facial paralysis, similar to the two-tier test. The principal advantage of the VlsE peptide ELISA is that it requires only one test rather than four tests. However, the specificity of the VlsE test may not be as high as that of the two-tier test.

Immunologic abnormalities and surgical experiences in recurrent facial nerve paralysis.

OBJECTIVE: To document immunologic findings in patients with recurrent facial paralysis (RFP) and to compare the results of the surgery with the results of medical treatment. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care referral center. PATIENTS: Nine patients with RFP were reviewed. INTERVENTION: Patients underwent nonspecific antibody detection, protein electrophoresis (in blood and cerebrospinal fluid [CSF]) and oligoclonal band determination for immunoglobulin G, A, and M (in CSF). The extended subtotal facial nerve decompression via the transmastoid and transattic route was performed in four patients. Five patients received medical treatment only (steroids, vitamin B). RESULTS: Two patients had the complete and four patients had the oligosymptomatic form of Melkersson-Rosenthal syndrome. The other three patients were diagnosed with idiopathic RFP. Serum immunoglobulin G was high in seven of nine patients (77%). CSF protein electrophoresis demonstrated an elevated albumin fraction in six of nine patients (66%). CSF immunoglobulin G was high in four of nine patients (44%). The oligoclonal band in CSF was negative in all patients. Mean follow-up time was 5.2 +/- 2.6 years and 3 +/- 1.5 years for surgically treated patients and medically treated patients, respectively. None of the patients who underwent the surgery demonstrated recurrence. Although marked recovery was observed in patients who had received medical treatment, three of them had recurrence during the follow-up period. CONCLUSION: Serologic test results have demonstrated immune system involvement in cases of idiopathic RFP and in cases of Melkersson-Rosenthal syndrome, providing no distinction between the two. There was no sign substantiating local antibody production in CSF, which implies that the elevated antibodies in CSF were peripheral in origin. Although the serologic test results were not conclusive for a specific diagnosis, they support an immune-mediated pathogenesis. Despite the small number of patients who underwent the extended transmastoid facial nerve decompression, our follow-up data were suggestive for the prevention of recurrences.

[Repair of facial nerve gaps with cryopreserved allografts in rats immunosuppressed with cyclosporin A]. / Reparación de lesiones del nervio facial mediante aloinjertos criopreservados en ratas inmunodeprimidas con ciclosporina A.

UNLABELLED: The aim of this study is to assess the feasibility of peripheral nerve allografts pretreated utilizing cold storage and cyclosporin A to improve the facial nerve regeneration in Wistar rat. Three groups were designed: Normal Wistar rats. 4 millimeters nerve gaps repaired with cryopreserved nerve allografts. 4 millimeters nerve gaps repaired with cryopreserved nerve grafts and treated with cyclosporin A. At 16 weeks post-engraftment the animals were evaluated: Facial palsy grading system. Electrophysiologic latency. Axonal counting. Nervous fiber area immediately distal to the graft. The facial function of the experimental groups was similar to the control rats while the latency and morphometric parameters was poor than the normal rats. CONCLUSIONS: 1. Cryopreserved nerve allografts in facial nerve repair in rats are useful in the aquisition of a facial functional nerve recovery as much in immunosuppressed animal as not. 2. Cryopreservation of nerve allografts results a good method of storage of nerve grafts. 3. Cyclosporin A immunosuppression improved not much the grading facial palsy in this model but the electrophysiologic and morphometry is significantly better.