Ramsay Hunt syndrome with viral meningitis.

Ramsay Hunt syndrome (RHS) is an infection with the Varicella Zoster virus in the geniculate ganglion of the facial nerve. The syndrome consists of a triad of peripheral facial nerve palsy, ear pain and concurrent zoster rash in the ear canal. However, vesicles in the mouth can be seen. A rare complication of RHS is viral meningitis. This is a case report of a patient with orally manifested RHS and concurrent asymptomatic viral meningitis. This case aims to raise awareness of RHS with atypical presentation and concurrent viral meningitis and, thereby, the importance of a thorough neurological examination.

Ramsay Hunt syndrome in atopic dermatitis patient treated with dupilumab.

The Ramsay-Hunt syndrome results from reactivation of the varicella-zoster virus at the geniculate ganglion level. The syndrome is characterized by a combination of symptoms such as ipsilateral facial paralysis, otalgia, and vesicles near the ear and auditory canal. The gold standard in the treatment of Ramsay-Hunt syndrome remains the combination of antiviral therapy with corticosteroids and adequate analgesic therapy. We present a case of a 45-year-old patient with severe form of atopic dermatitis, who developed this syndrome during treatment with dupilumab. The risks and benefits of dupilumab treatment in this patient were considered. Because both bronchial asthma and atopic dermatitis worsened when dupilumab was discontinued, it was indicated to continue this therapy with low-dose of acyclovir.

The Impact of the COVID-19 Pandemic on Bell's Palsy and Ramsay-Hunt Syndrome: A Multicenter Retrospective Study.

BACKGROUND: This article presents a comprehensive review of data on the impact of facial palsy during the coronavirus disease 2019 (COVID-19) pandemic. The possible causes and pathophysiological mechanisms of changes in the epidemiology of facial palsy during the COVID-19 pandemic are also discussed. METHODS: This multicenter retrospective cohort study included 943 patients diagnosed with Bell's palsy or Ramsay Hunt syndrome. This study compared patient demographics, comorbidities, symptoms, and treatments before the COVID-19 pandemic (from 2017 to 2019) and during the COVID-19 pandemic, from 2020 to 2022). RESULTS: Following the COVID-19 outbreak, there has been a significant increase in the number of cases of Bell's palsy, particularly among elderly individuals with diabetes. Bell's palsy increased after the COVID-19 outbreak, rising from 75.3% in the pre-COVID-19 era to 83.6% after the COVID-19 outbreak. The complete recovery rate decreased from 88.2% to 73.9%, and the rate of recurrence increased from 2.9% to 7.5% in patients with Bell's palsy. Ramsay Hunt syndrome showed fewer changes in clinical outcomes. CONCLUSION: This study highlights the impact of the COVID-19 pandemic on the presentation and management of facial palsy, and suggests potential associations with COVID-19. Notably, the observed increase in Bell's palsy cases among elderly individuals with diabetes emphasizes the impact of the pandemic. Identifying the epidemiological changes in facial palsy during the COVID-19 pandemic has important implications for assessing its etiology and pathological mechanisms of facial palsy disease.

Antiviral treatment for Ramsay Hunt syndrome: A systematic review and meta-analysis.

OBJECTIVE: This study aimed to reveal the efficacy and safety of antivirals in patients with Ramsay Hunt syndrome. METHODS: A literature search was conducted in PubMed, Ichushi-Web, and Cochrane Central Register of Controlled Trials. Published randomized controlled trials and observational studies, which compared antivirals versus placebo/no treatment for Ramsay Hunt syndrome, were included in the meta-analysis. The primary outcome was non-recovery at the end of the study follow-up. Data was analyzed using Review Manager Software, and pooled odds ratio (OR) with 95 % CI were calculated. RESULTS: Two randomized controlled trials and 7 cohort studies met the eligible criteria, and 474 individuals were included in the meta-analysis. The OR of antivirals for non-recovery was 0.68 (95 % CI 0.37-1.27, p = 0.22). In subgroup analysis, the OR were 0.48 (95 % CI 0.15-1.61, p = 0.24) in patients with antivirals monotherapy and 0.73 (95 % CI 0.34-1.57, p = 0.42) in patients treated with combination therapy of antivirals and systematic corticosteroid. CONCLUSION: This systematic review first shows the effectiveness of antivirals. Further study is needed to confirm the efficacy of antivirals.

Ramsay Hunt Syndrome Associated with Varicella-Zoster Virus Encephalitis in a Child.

Ramsay Hunt syndrome (RHS) is a triad of peri-auricular pain, ipsilateral facial nerve palsy and vesicular rash around the ear pinna. It is caused by reactivation of varicella-zoster virus (VZV) that lies dormant in the geniculate ganglia. It can be complicated by VZV encephalitis rarely. We report the case of an 8-year-old previously healthy boy who presented to a tertiary care hospital in Muscat, Oman in 2021 with fever, progressive left ear pain, vesicular rash around his ear pinna and left-sided facial nerve palsy. His course was complicated by VZV encephalitis where he was managed with intravenous (IV) acyclovir and IV corticosteroids. He improved significantly and was asymptomatic with a normal neurology examination at the 6-months follow-up.

Sequelae Treatment Needs Following Peripheral Facial Palsy: Retrospective Analysis of 525 Patients.

OBJECTIVE: This study uses retrospective longitudinal data from a large unselected cohort of patients with peripheral facial paralysis to determine the prevalence and patient characteristic predictors of sequelae receiving intervention. STUDY DESIGN: Retrospective case review. SETTING: Karolinska University Hospital in Stockholm Sweden serves as the only tertiary facial palsy center in the region. Here, patients are diagnosed, are followed up, and undergo all major interventions. PATIENTS: All adult patients presenting with peripheral facial palsy due to idiopathic, zoster, or Borrelia origin at Karolinska, January 1, 2010 to December 31, 2011 with follow-up until December 2022. INTERVENTIONS: Patient charts were studied to identify patient characteristics, etiology, initial treatment, severity of palsy, and treatments targeting sequelae. MAIN OUTCOME MEASURES: Types of initial and late treatments were noted. Sunnybrook and/or House-Brackmann scales were used for palsy grading. RESULTS: Five hundred twenty-five patients were included. Thirty-three patients (6.3%) received botulinum toxin injections and/or surgical treatment. In this subgroup, 67% received corticosteroids compared to 85% of all patients ( p = 0.005), cardiovascular disease prevalence was higher (23 and 42%, respectively, p = 0.009). For 81 patients (15%), follow-up was discontinued although the last measurement was Sunnybrook less than 70 or House-Brackmann 3 to 6. CONCLUSIONS: Of patients with peripheral facial palsy, 6.3% underwent injections and/or surgical treatment within 12 years. However, due to a rather large proportion not presenting for follow-up, this might be an underestimation. Patients receiving late injections and/or surgical treatment had more comorbidities and received corticosteroid treatment to a significantly lower extent in the acute phase of disease.

Clinical Analysis of 3D-Fluid Attenuated Inversion Recovery and T1volume interpolated body examination Sequences on Delayed Gadolinium-Enhanced Scanning in Ramsay Hunt Syndrome.

BACKGROUND: Through the clinical analysis of 4 clinically confirmed cases of delayed gadolinium enhancement of Ramsay Hunt syndrome 3D-Fluid Attenated Inversion Recovery'and 'T1volume interpolated body examination (3D-FLAIR and T1VIBE) sequences, the more suitable sequences and pathologically damaged tissue sites of deep tissues of Ramsay Hunt syndrome by magnetic resonance imaging gadolinium enhancement were preliminarily explored. METHODS: From October 2020 to March 2021, 4 clinically confirmed patients with Ramsay Hunt syndrome, 2 males and 2 females, aged 27-63, were continuously collected in the hospital otology clinic. Siemens Avento 1.5T magnetic resonance imaging 3D-FLAIR and T1VIBE sequencedelayed gadolinium enhancement scans and serological laboratory tests were performed, respectively, and corresponding antiviral and antiinflammatory therapy was given. RESULTS: The magnetic resonance imaging gadolinium enhancement of 4 cases of Ramsay Hunt syndrome was as follows: 3D-FLAIR sequence delay of 4.5 hours scanning 4 patients labyrinthine and/or middle ear signal was enhanced at the same time as the healthy side; T1VIBE sequence scanning disease in 3 cases of vestibular nerve development was enhanced than the healthy side, 2 cases of facial nerve development was enhanced than the healthy side, and 2 cases of cochlear nerve development was enhanced than the healthy side. All 4 patients were cured with related treatment. CONCLUSION: Through the comparison of 3D-FAIR and T1VIBE sequence of 4.5 hours delay before intravenous gadolinium injection and 4.5 hours delay after intravenous gadolinium injection in 4 patients with Ramsay Hunt syndrome, it was found that (i) 3D-FLAIR sequence delay of 4.5 hours scan was more likely to show whether the inner ear labyrinth barrier permeability increased and (ii) Ramsay Hunt syndrome deep ear tissue damage can be manifested as labyrinthitis, vestibular cochlear neuritis, facial neuritis, and otitis media.

Multiple cranial neuropathy due to varicella zoster virus reactivation without vesicular rash: a challenging diagnosis.

Ramsay Hunt syndrome is due to reactivation of varicella zoster virus (VZV) dormant in the geniculate ganglion of the facial nerve. The diagnosis is typically based on clinical triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or the auricle. However, Ramsay Hunt syndrome may occur without skin eruption in up to one third of patients. Moreover, the involvement of other cranial nerves in addition to the facial nerve has been also reported. Herein, we reported a case report of a man who developed a multiple cranial neuropathy caused by VZV reactivation without skin vesicular eruption. The present case underlines a possible diagnostic challenge that clinicians may hit when facing a common disorder such as peripheral facial palsy. Indeed, clinicians must be aware that Ramsay Hunt syndrome may develop without skin vesicular eruption as well it may be complicated by multiple cranial nerve involvement. Antiviral therapy is effective in VZV reactivation for recovery of nerve function.

Two cases of Ramsay-Hunt syndrome following varicella zoster viral meningitis in young immunocompetent men: case reports.

BACKGROUND: Ramsay-Hunt syndrome (RHS) due to varicella zoster virus (VZV) infection is commonly reported in individuals aged at least 50 years or immunocompromised individuals. VZV infection may invade the central nervous system (CNS) and cause meningitis or encephalitis, which are more likely to occur in patients with chronic diseases such as diabetes and chronic renal failure. However, cases with VZV-induced concurrent RHS and CNS infections are rare. CASE PRESENTATION: Two young male patients, aged 32 and 43 years, with no underlying disease developed VZV meningitis, followed by RHS involving cranial nerves VII and VIII. Both patients presented with symptoms of peripheral facial palsy, and dizziness accompanied by tinnitus and hearing loss, which appeared several days after the onset of fever and headache. These symptoms were documented as facial neuropathy and sensorineural hearing loss in the electrophysiologic studies. Lymphocyte-dominant pleocytosis and VZV positivity were confirmed from cerebrospinal fluid examination and polymerase chain reaction, respectively. The patients were treated with intravenous acyclovir and oral steroids simultaneously. Following the treatment completion, both patients were relieved of their headaches and fever; however, facial palsy, dizziness, and tinnitus persisted. They were followed up at the outpatient clinic. CONCLUSION: These cases confirmed that RHS and CNS infections can co-exist even in young adults with normal immune function and more importantly, that CNS infection can precede RHS. Since early detection and treatment of RHS improve the prognosis, it is critical to closely monitor patients with VZV meningitis or encephalitis considering the possible superimposition of RHS.

Benefits of High-Dose Corticosteroid and Antiviral Agent Combination Therapy in the Treatment of House-Brackman Grade VI Ramsay Hunt Syndrome.

OBJECTIVE: Few large-scale investigations have been conducted on treatment of House-Brackmann grade VI (HB grade VI) Ramsay Hunt syndrome (RHS) patients. We compared recovery rates among patients receiving a normal-dose corticosteroid (prednisolone [PSL] 60 mg/d) or high-dose corticosteroid (PSL 200 mg/d), both with or without an antiviral agents. Recovery rates were also examined based on the order of presentation of herpetic vesicles versus facial palsy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: A total of 128 patients with HB grade VI RHS were treated in our department between 1995 and 2017. These patients were divided into four treatment groups based on corticosteroid dosage and use of an antiviral agent. METHODS: We assessed treatment outcomes for HB grade VI patients together with logistic regression analysis to investigate factors that can impact treatment outcomes, that is, sex, age, days to start of treatment, PSL dosage, and antiviral agent administration. RESULTS: Recovery rates were best in the high-dose corticosteroid group with an antiviral agent (71.1%) in comparison with the normal-dose corticosteroid group with an antiviral agent (60.0%) or high-dose corticosteroid alone (57.1%). Significant factors for treatment outcomes were high-dose corticosteroid administration and early initiation of treatment. A better recovery rate was also found when the herpetic vesicles appeared before facial palsy. CONCLUSION: We showed that a combination of a high-dose corticosteroid and antiviral agent produced the best outcomes for patients with HB grade VI RHS. However, our results were not statistically significant because of small sample size.

[Clinical and therapeutic features of herpes zoster oticus: a case report]. / Zona otitique, aspects cliniques et thérapeutiques: à propos d'un cas.

Ear shingles, also called herpes zoster oticus, are a viral infection of the outer, middle and/or inner ear due to varicella zoster virus (VZV). Patients with ear involvement associated with facial palsy are clinically diagnosed with Ramsay-Hunt´s syndrome. We here report the case of a 25-year-old patient with herpes zoster oticus associated with peripheral facial palsy, without other associated signs. This study and literature review investigate the various clinical, para-clinical and evolutionary features of herpes zoster oticus and the therapeutic approaches.

Intratympanic corticosteroid for Bell's palsy and Ramsay Hunt syndrome: Systematic review and meta-analysis.

OBJECTIVE: This study aimed to reveal the efficacy and safety of intratympanic steroid therapy (ITST) in patients with Bell's palsy and Ramsay Hunt syndrome. METHODS: A literature search was conducted in PubMed, Embase, Ichushi-Web, and Cochrane Central Register of Controlled Trials. Published randomized controlled trial and observational studies, which compared the combination of intratympanic corticosteroids with systemic corticosteroid versus systemic corticosteroid for Bell's palsy and Ramsay Hunt syndrome, were included for meta-analysis. The primary outcome was non-recovery at the end of the study follow-up. Data was analyzed using Review Manager software, and pooled odds ratio with 95% CI were calculated. RESULTS: Three randomized controlled trial and two cohort studies met the eligible criteria, and 350 individuals included for meta-analysis. The timing of intratympanic corticosteroid was varied from once or twice a week to consecutive 10-days. Patients treated with combination of systemic corticosteroids and intratympanic corticosteroids were likely to achieve recovery of normal facial function (OR = 0.36 [95% CI 0.12-1.14]). The subgroup analysis of the timing revealed that daily ITST reduced non-recovery (OR = 0.14 [95% CI 0.04-0.50). However, non-daily ITST did not reduced non-recovery (OR 0.93 [95% CI 0.37-2.34]). CONCLUSION: Intratympanic corticosteroid have a potential to reduce non-recovery in patients with Bell's palsy and Ramsay Hunt syndrome. The methods of intratympanic corticosteroid varied among included studies. Further well-designed randomized controlled trial is needed to confirm the effectiveness and to identify an adequate method of intratympanic corticosteroid in these patients.

Herpes zoster oticus. / Herpes zoster oticus.

BACKGROUND: Herpes zoster oticus is a rare neurological disease caused by reactivation of varicella-zoster virus in the facial nerve. CASE PRESENTATION: A woman in her 60 s presented with left-sided seventh and eighth cranial neuropathy. A vesicular rash in her left ear and palate appeared one week after symptom onset. Lumbar puncture revealed cerebrospinal fluid (CSF) pleocytosis, mononuclear cells only, with PCR positive for varicella zoster virus. Inflammation of the cochlear and vestibular systems and along the facial nerve was detected by MRI. Antiviral treatment with valacyclovir in combination with prednisolone was initiated. After one month with outpatient rehabilitation, the vertigo had ceased, but hearing loss and facial paralysis persisted. INTERPRETATION: Early diagnosis and treatment of herpes zoster oticus as a cause of facial palsy when accompanied by ear rash, pain or signs of other cranial nerve involvement may improve overall prognosis.