Varicella­zoster virus­associated meningitis followed peripheral facial palsy: A case report.

Although central nervous system infection following varicella zoster virus infection is relatively common, subsequent peripheral nervous system infection is comparatively rare. The present case documents a case of meningitis after varicella-zoster virus (VZV) infection, which was then followed by peripheral facial palsy. Specifically, a 54-year-old female patient was first admitted to Shengli Oilfield Central Hospital (Dongying, China) with headache and fever. Physical examination revealed herpes that formed along the intercostal nerve in the left forebreast, armpit and back. Subsequently, neurological examination found cervical resistance in more than three fingers (neck resistance of less than two transverse fingers is not evidence of meningeal irritation; the neck resistance of this patient was approximately three transverse fingers, so the patient was presumed to be positive for meningeal irritation, highly suggestive of meningitis) and Kernig sign was positive. There were no significant abnormalities according to brain MRI and lumbar puncture pressure was 330 mmH2O. In addition, the leukocyte count was 734x106/l, 50% monocyte count, 50% multinucleated cells, chloride levels of 109.1 mmol/l, protein levels of 235 mg/dl and glucose levels of 4.18 mmol/l in the cerebrospinal fluid. DNA and RNA metagenomic detection of pathogenic microorganisms in the cerebrospinal fluid revealed the presence of VZV. The patient was therefore treated with acyclovir, ceftriaxone, mannitol and methylprednisolone, but then developed right peripheral facial palsy at 10 days after treatment. This complication was not found in the literature, and the occurrence of facial neuritis was unexpected. The active period of VZV virus was 21 days, and the patient had herpes 5 days before admission. The active period of the virus was considered to have subsided and the patient was in the recovery period. Moreover, the results of lumbar puncture showed that the white blood cells, the proportion of neutrophils and the protein in cerebrospinal fluid were all decreasing, which also indicated that the patient had entered the recovery period. The patient was discharged 18 days after admission. In conclusion, observations from the present case suggested that the clinical manifestations of VZV infection can be complex and varied, requiring the clinician to have an accurate understanding of its disease progression and treatment.

Masseteric-facial anastomosis and hypoglossal-facial anastomosis after lateral skull base and middle ear surgery.

INTRODUCTION: Lateral skull base (LSB) and middle ear pathologies often involve the facial nerve (FN), and their treatment may require FN sacrifice. Cases with unidentifiable proximal stump or intact FN with complete FN palsy, necessitate FN anastomosis with another motor nerve in order to restore innervation to the mimicking musculature. The results of hypoglossal-to-facial nerve anastomosis (HFA) and masseteric-facial nerve anastomosis in patients with facial paralysis after middle ear and LSB surgeries, are presented and compared. METHODS: Adult patients with total definitive facial paralysis after middle ear or LSB surgery undergoing facial nerve reanimation through hypoglossal or masseteric transfer anastomosis were enrolled. The facial nerve function was graded according to the House Brackmann grading system (HB). The facial function results at 3 months, 6 months, 12 months, 18 months and at the last follow up (more than 18 months) are compared. RESULTS: 153 cases of LSB and middle ear surgery presented postoperative facial palsy and underwent facial nerve reanimation surgery with HF in 85 patients (55.5%) and MF in 68 patients (44.5%). The duration of the FN palsy before reconstructive surgery was inversely associated to better FN results, in particular with having a grade III HB (p = 0.003). Both techniques had significantly lower HB scores when an interval between palsy onset and reanimation surgery was 6 months or less (MF p = 0.0401; HF p = 0.0022). Patients who underwent a MF presented significant improvement of the FN function at 3 months from surgery (p = 0.0078). At the last follow-up, 63.6% recovered to a grade III HB and 22.7% to a grade IV. On the other hand, the first significant results obtained in the HF group were at 6 months from surgery (p < 0.0001). 67.8% of patients had a grade III HB after a HF at the last follow-up, 28.8% a grade IV. FN grading at 6 months from surgery was significantly lower in the MF group compared to the HF (p = 0.0351). The two techniques had statistically similar results at later follow-up evaluations. DISCUSSION/CONCLUTION: MF was associated to initial superior results, presenting significant facial recovery at 3 months, and significantly better functional outcomes at 6 months from surgery compared to HF. Although later results were not significantly different in this study, earlier results have an important role in order to limit the duration of risk of corneal exposure.

Atypical Presentation of Acute Suppurative Otitis Media with Facial Palsy: Extra Medullary Manifestation of AML in Temporal Bone.

Acute Myeloid Leukemia (AML) is the most common leukemia observed in the adult population, accounting for 80% of all leukemia cases. Extramedullary involvement in AML, where leukemic cells are found in organs or tissues outside the blood or bone marrow, is a rare occurrence [1]. The most frequent sites of extramedullary disease include the skin, central nervous system (CNS), and lymph nodes [2, 3]. In this case report, we present an instance of extramedullary AML in the temporal bone, which initially presented with symptoms such as earache, discharge, and facial asymmetry, mimicking acute suppurative otitis media with facial palsy. The patient underwent mastoid exploration and facial nerve decompression. A post-operative bone marrow biopsy confirmed the diagnosis of AML, leading to the initiation of chemotherapy. The patient is currently under follow-up care.

Pediatric Peripheral Facial Palsy Following Varicella Zoster Infection.

Peripheral facial palsy, characterized by sudden weakness or paralysis of the facial muscles, can arise from various etiologies, including viral infections. While Ramsay Hunt syndrome is well-established in clinical practice, Varicella Zoster Virus (VZV) infection leading to facial nerve palsy in pediatric patients remains relatively uncommon.This comprehensive case report documents the clinical presentation, diagnostic evaluation, treatment, and outcomes of a 10-year-old boy who developed left peripheral facial palsy following a primary Varicella infection. The report underscores the importance of timely recognition and tailored management approaches in achieving a complete remission of symptoms in pediatric patients.

Unveiling Unilateral Bell's Palsy: A Case Series.

Bell's palsy is an idiopathic and uncommon peripheral nerve palsy that affects the facial nerve, leading to an inability to control the muscles of facial expression on the affected side. This paper presents two cases of unilateral Bell's palsy in female patients treated with systemic steroids, antiviral drugs, and artificial tear substitutes. The treatment outcomes, clinical course, and recovery timelines are discussed in detail. A review of the current literature on the etiology, diagnosis, and management of Bell's palsy is also provided to contextualize these cases within broader clinical practice.

Aural Manifestations of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis-Diagnosis, Symptoms, Treatment.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of vasculitis sharing a common pathophysiology, which affects small and medium blood vessels. There are three categories of AAV: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). As a systemic disease, AAV can affect basically every organ. The goal of this publication is to sum up and underline the problem of the aural manifestation of AAV; it details the definition of Otitis Media with Antineutrophil Cytoplasmic Antibody Associated Vasculitis (OMAAV) and allows for a better understanding of the specific tasks of medical professionals taking part in the diagnostic and therapeutic process. Among others, this publication is directed to otolaryngologists who may encounter patients with AAV and often are the first specialists who see patients with early symptoms of AAV. This publication presents brief characteristics of AAV, descriptions of aural manifestations and symptoms, differential diagnosis, and both pharmacological and surgical treatment options, based on current recommendations and information found in the literature and clinical databases.

Functional Outcome of single-stage facial reanimation surgery with radical parotidectomy.

Background: Facial nerve sacrifice during radical parotidectomy impairs quality of life. This study assessed the effectiveness of simultaneous single-stage facial reanimation surgery with radical parotidectomy in restoring facial function. Methods: A retrospective analysis was conducted on patients who underwent single-stage facial reanimation with radical parotidectomy. Techniques included selective reinnervation and orthodromic temporalis tendon transfer. Outcomes were measured using modified House-Brackmann and Terzis grades, Emotrics facial assessment, and the Facial Disability Index (FDI). Results.: Among thirteen patients (median age 54, 69% male), ten received selective reinnervation. Nine of these patients showed improved results of House-Brackmann grade III and Terzis grade 4-5. The remaining three underwent tendon transfer, achieving moderate functional outcomes. Emotrics analysis indicated balanced facial symmetry in the selective reinnervation group. FDI scores reflected satisfactory physical and social/well-being functions. Conclusion: Single-stage facial reanimation effectively restores facial function in patients undergoing radical parotidectomy. This approach offers significant benefits in early facial function recovery.

Summary of Japanese clinical practice guidelines for Bell's palsy (idiopathic facial palsy) - 2023 update edited by the Japan Society of Facial Nerve Research.

OBJECTIVE: The "Summary of Japanese clinical practice guidelines for Bell's palsy (idiopathic facial palsy) - 2023 update edited by the Japan Society of Facial Nerve Research" aims to review the latest evidence regarding the treatment of Bell's palsy and to provide appropriate recommendations. METHOD: Regarding the treatment of Bell's palsy, a guideline panel identified key clinical questions using an analytic PICO framework. The panel produced recommendations following the standards for trustworthy guidelines and the GRADE approach. The panel considered the balance of benefits, harm, and preferences when making recommendations. RESULTS: The panel identified nine key clinical questions: systemic (high/standard dose) corticosteroids, intratympanic corticosteroids, systemic antivirals, decompression surgery, acupuncture, physical therapy, botulinum toxin, and reanimation surgery. CONCLUSION: These guidelines strongly recommend systemic standard-dose corticosteroids for the clinical management of Bell's palsy. Other treatments are weakly recommended due to insufficient evidence. The absolute risk reduction of each treatment differed according to the disease severity. Therefore, physicians and patients should decide on treatment based on the disease severity.

Salvaging the transected hypoglossal nerve using descendens hypoglossi in patients undergoing hypoglossal-facial nerve anastomosis for facial palsy: a randomized clinical trial.

OBJECTIVE: Hypoglossal-facial nerve anastomosis (HFA) is the most commonly used surgical treatment for severe facial palsy that does not respond to conservative treatments. A major complication of HFA is the loss of tongue function. The authors aimed to evaluate whether anastomosing the transected hypoglossal nerve using the ramus descendens hypoglossi could prevent tongue deviation and dysfunction in patients undergoing HFA. METHODS: In this randomized trial, adult patients with severe peripheral facial palsy (House-Brackmann grade V or VI) who did not respond to at least 6 months of conservative treatment were randomized at a 1:1 ratio to undergo either HFA alone (control group) or HFA plus anastomosis between the hypoglossal nerve and descendens hypoglossi (intervention group). The primary endpoint was tongue deviation angle at 12 months. Key secondary endpoints included tongue disability (chewing difficulty, swallowing defect, and articulation defect), tongue disability index (TDI; range 1-4, with a higher score indicating more severe disability), and facial nerve function. RESULTS: Twenty patients were enrolled (10 in each group). At 12 months, the tongue deviation angle was significantly lower in the intervention group than in the control group (7.8° ± 5.1° vs 23.6° ± 9.6°, p < 0.001). Although not statistically significant, the intervention group had lower rates of chewing difficulty (1/10 vs 3/10, p = 0.58), swallowing defect (1/10 vs 5/10, p = 0.14), and articulation defect (2/10 vs 6/10, p = 0.17). TDI was significantly lower in the intervention group (1.5 ± 0.6 vs 2.5 ± 0.3, p < 0.001). The percentage of the patients achieving House-Brackmann grade II or III was 80% in each group. CONCLUSIONS: Anastomosis of the descendens hypoglossi to the transected hypoglossal nerve attenuated tongue deviation in patients undergoing HFA for facial palsy, without compromising facial nerve function. Clinical trial registration no: ChiCTR2000034372 (Chinese Clinical Trials Registry).

Association of Coronavirus Disease 2019 Vaccination with Facial-Related Neurological Disorders: A Nationwide Retrospective Cohort Study.

Neurological complications after the coronavirus disease 2019 (COVID-19) vaccine administration have been reported. However, the incidence rates of these complications have not been compared in vaccinated and unvaccinated individuals. This study used a nationwide cohort from South Korea to investigate the incidence and prognostic factors of facial-related neurological disorders, such as facial palsy, trigeminal neuralgia, and hemifacial spasms, after COVID-19 vaccination. A population-based cohort design was used to examine data from a randomly selected 50% of the adult population in Seoul, South Korea. Information on demographics, vaccination status, vaccination type, and medical history was collected. The incidence rates and adjusted hazard ratios (aHRs) for facial-related neurological disorders were calculated. This study included 2,482,481 adults, 85.94% of whom were vaccinated. Vaccinated individuals showed a higher incidence of facial palsy, hemifacial spasm, and trigeminal neuralgia than unvaccinated individuals, with significant aHRs of 1.821, 3.203, and 6.621, respectively. Dyslipidemia, female sex, and young age were identified as risk factors for hemifacial spasms and trigeminal neuralgia. This study demonstrates an increased incidence of facial-related neurological disorders after COVID-19 vaccination, particularly among individuals with dyslipidemia and younger women. These findings underscore the need for further investigations into the mechanisms and management of vaccine-related neurological issues.

[Bilateral facial palsy: an atypical presentation of Lyme disease (a case report)]. / Paralysie faciale bilatérale : une présentation atypique d'une maladie de Lyme (cas clinique).

Lyme neuroborreliosis is a rare zoonosis which can be difficult to diagnose, in particular in low endemic areas. We here report the case of a 35-year-old man presenting with disabling back pain preceded by facial monoplegia, which was wrongly treated as Bell's palsy (paralysis a frigore) and then as post-traumatic lumbosciatica. The onset of facial diplegia allowed for a definitive diagnosis. The patient was treated with ceftriaxone and symptoms gradually improved.

Biopsy of the facial nerve in slow-onset facial palsy.

INTRODUCTION: Primary squamous cell carcinoma of the parotid gland typically presents as a palpable, often painless mass. Peripheral facial palsy as the only sign of malignant neoplasia is rare. In these cases, the diagnosis is regularly confirmed by radiological imaging followed by surgical exploration and biopsy. However, if there is no detection of malignant lesions and no evidence of a tumor, the reluctance to take a biopsy of an unremarkable nerve can lead to misdiagnoses. CASE REPORT: A 40-year-old female patient without medical history presented to our clinic with a complete right-sided peripheral facial palsy that had slowly progressed for 2.5 years. All other otorhinolaryngological examination findings were within normal limits. Magnetic resonance imaging examination of the head and neck and 18-fluorodeoxyglucose positron emission tomography showed unremarkable results. We proceeded with surgical exploration, which revealed no evidence of a tumor and an externally completely unremarkable facial nerve. A biopsy from the main trunk area of the nerve revealed an infiltration by a squamous cell carcinoma. Total parotidectomy with resection and reconstruction of the facial nerve and neck dissection was performed. Considering the absence of a primary tumor and other tumor formations the diagnosis of a completely regressive primary squamous cell carcinoma of the parotid gland was confirmed. CONCLUSION: In conclusion, in the case of slow-onset peripheral facial palsy that persists without signs of recovery, a gadolinium-enhanced MRI should be performed. If imaging is unremarkable and there is no primary tumor detection along the course of the facial nerve, a surgical exploration with biopsy of the facial nerve is necessary.

A case of mixed hereditary gelsolin amyloidosis and hydroxychloroquine induced myopathy.

Hereditary gelsolin amyloidosis is an adult onset autosomal dominant disease with neurologic, ophthalmologic and dermatologic features that may be mistaken for Sjögren syndrome. We describe a case of a 68 year old female presenting with facial numbness and asymmetry, dry eyes, dry mouth and fatigue, originally diagnosed as Sjögren syndrome and treated with hydroxychloroquine. Due to her insidious progression of facial weakness with associated proximal muscle weakness she underwent a muscle biopsy, which demonstrated features of hydroxychloroquine induced myopathy and amyloid deposition. This subsequently led to targeted genetic testing, revealing an autosomal dominant c.640G > A pathogenic variant of the gelsolin gene. Therefore, this is a unique case of complex muscle pathology with features of a rare hereditary systemic amyloidosis an uncommon drug- induced myopathy.

A case of lenticulostriate artery infarction presenting with peripheral type facial palsy.

An 82-year-old woman developed a droopy right eyelid with ipsilateral hemiparesis. Her ocular symptom was caused by weakness of the right frontalis, which is usually seen in patients with peripheral facial nerve palsy. However, head MRI showed acute cerebral infarction of the left lenticulostriate artery, and electroneurography did not detect damage to the right facial nerve. To explain the pathophysiology in this patient, asymmetrical bilateral cortex innervation to the right upper face was hypothesized. This case suggested that patients with some hemispheric strokes could develop upper facial weakness mimicking facial nerve palsy, and clinicians should pay attention to this potential pitfall in the differential diagnosis of facial nerve palsy.

Multidisciplinary Care for Moebius Syndrome and Related Disorders: Building a Management Protocol.

Moebius syndrome is a collection of orofacial anomalies with highly variable features affecting many different systems but characterised by bilateral facial palsy and absent eye abduction. We largely regard Moebius syndrome as a diagnosis of exclusion. Lack of awareness and knowledge means that children often fall between services, leading to treatment delays and difficulty interfacing with social care and schools, with long-term impact on physical health and psychosocial development. We developed a multidisciplinary team comprising core clinicians (lead physician, geneticist, speech and language therapist, psychologist and specialist nurse) and an expanded group to encompass the other affected systems. The interactions between our specialties lead to the development of a treatment protocol, which we present. The protocol harnesses the aspects of care of children with a range of other rare diseases at a specialised paediatric centre and synthesises them into a holistic approach for MBS and related conditions. Management is sequenced on an "ABC-style" basis, with airway, feeding, vision and speech taking priority in the early years. We define management priorities as airway stabilisation with swallow assessment, ocular surface protection and maintenance of nutritional support. Management principles for issues such as speech, reflux, drooling and sleep issues are outlined. In later years, psychological support has a prominent role geared towards monitoring and interventions for low mood, self-esteem and bullying.

Facial Palsy in Post COVID-19 Invasive Fungal Sinusitis.

This study aimed to study the various presentations of post COVID-19 invasive fungal sinusitis with 7th cranial nerve palsy and to correlate topodiagnostic tests and radiological findings to identify the site of lesion. A retrospective study was conducted at a tertiary care hospital where 11 patients with post COVID-19 invasive fungal sinusitis with facial palsy presented to our institute. Detailed history, clinical examination, radiological imaging were done for all patients. A total of 11 patients with post COVID-19 invasive fungal sinusitis with facial palsy were included in this study. The mean age of the patients was 44.8, with a male to female ratio of 4.5:1. Diabetes Mellitus was a major factor contributing to immunosuppression in our study with 9 patients who were known cases of diabetes mellitus and 2 newly diagnosed cases. On MRI, 10 patients had involvement of the Pterygopalatine fossa and 4 patients had involvement of the petrous apex. Post COVID-19 invasive fungal sinusitis can present with facial palsy, and diabetes mellitus is a major contributing factor to immunosuppression in these patients. The involvement of the petrous apex is a possible site of involvement, and MRI can aid in localizing the site of the lesion. Further studies are required to identify the exact mechanism of facial nerve palsy in invasive fungal sinusitis.

Diagnostic Challenges of Lyme Co-infections: Lessons From a Lyme and Herpes Simplex Virus-1 (HSV-1) Cocktail.

Lyme borreliosis (LB) is a complex tick-borne illness with diverse presentations. We report a case of LB meningitis with herpes simplex virus-1 (HSV-1) co-infection in a 55-year-old woman initially presenting with isolated facial nerve palsy. This case illustrates the multifaceted diagnostic challenges associated with Lyme co-infections. It emphasizes the need for thorough testing to identify all potential pathogens and the importance of differentiating between true co-infection and incidental HSV-1 reactivation. Understanding these complexities is crucial for guiding appropriate treatment decisions.

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.

Beyond the Eyes: Clinico-Radiological Correlation of Bilateral Complete Horizontal Gaze Palsy in Moebius Syndrome.

Moebius syndrome is a rare disease characterized by unilateral or bilateral facial nerve palsies with/without other cranial nerve palsy. It manifests clinically with facial muscle weakness and/or ophthalmoplegia and can be associated with other physical anomalies such as various limb deformities and orofacial malformation. Herein, we have described the clinical and radiological features of Moebius syndrome in a 9-year-old female child who presented with left-side facial palsy and bilateral complete horizontal gaze palsy.