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.

Diabetes Mellitus and Acute Facial Palsy: A Nationwide Population-Based Study.

INTRODUCTION: Acute facial palsy, characterized by sudden hemifacial weakness, significantly impacts an individual's quality of life. Despite several predisposing factors identified for acute facial palsy, the specific relationship between diabetes mellitus (DM) and acute facial palsy has not been comprehensively explored in recent studies. The aim of the study was to assess the risk of acute facial palsy in patients with DM using a nationwide population sample cohort. METHODS: DM cohort and non-DM cohort were built using the Korean National Health Insurance Service-Sample Cohort which represents the entire population of the Republic of Korea from January 2002 to December 2019. The DM cohort comprised 92,872 patients with a record of medication and a diagnosis of DM. Individuals who had facial palsy before the diagnosis of DM were excluded. A comparison cohort comprised 1,012,021 individuals without DM matched sociodemographically in a 1:4 ratio. The incidence of Bell's palsy (BP) and Ramsay Hunt syndrome (RHS) were evaluated in both cohorts. The risk factors for acute facial palsy were also assessed. RESULTS: Among the 92,868 patients in the DM cohort, the incidence rate (IR) of BP and RHS were 31.42 (confidence interval [CI], 30.24-32.63) and 4.58 per 10,000 person-years (CI, 4.14-5.05), respectively. Among the 371,392 individuals in the non-DM cohort, the IR of BP was 22.11 per 10,000 person-years (CI, 21.62-22.59) and the IR of RHS was 2.85 per 10,000 person-years (CI, 2.68-3.02). IR ratios for BP and RHS were 1.42 (CI, 1.36-1.48) and 1.61 (CI, 1.43-1.80). In multivariate analysis, DM (hazard ratio [HR] 1.428), age (HR 1.008), and high comorbidity score (HR 1.051) were associated with increased risk of BP, and male (HR 0.803) and living in metropolis (HR 0.966) decreased the risk of BP. And DM (HR 1.615), high comorbidity score (HR 1.078), and living in metropolis (HR 1.201) were associated with increased risk for RHS. CONCLUSION: This study suggests that patients with DM had an increased risk of acute facial palsy including BP and RHS.

Ramsay Hunt syndrome and concurrent varicella-zoster virus meningitis in Denmark: A nationwide cohort study.

Ramsay Hunt syndrome (RHS) is a manifestation of reactivated varicella-zoster virus (VZV) from the geniculate ganglion. Data on clinical features and outcomes of patients with RHS and concurrent VZV meningitis (henceforth RHS meningitis) are limited. Thus, we conducted a nationwide population-based cohort study of all adults hospitalized for RHS meningitis at the departments of infectious diseases in Denmark from 2015 to 2020. Patients with VZV meningitis without cranial nerve palsies were included for comparison. In total, 37 patients with RHS meningitis (mean annual incidence: 1.6/1 000 000 adults) and 162 with VZV meningitis without cranial nerve palsies were included. In RHS meningitis, the median age was 52 years (interquartile range: 35-64), and in addition to peripheral facial nerve palsy (100%), dizziness (46%), and hearing loss (35%) were common symptoms. The triad of headache, neck stiffness, and photophobia/hyperacusis was less common in RHS meningitis than in VZV meningitis without cranial nerve palsies (0/27 [0%] vs. 24/143 [17%]; p = 0.02). At 30 days after discharge, 18/36 (50%) patients with RHS meningitis had persistent peripheral facial nerve palsy, with no statistically significant difference between those treated with and without adjuvant glucocorticoids (6/16 [38%] vs. 12/20 [60%]; p = 0.18). Additional sequelae of RHS meningitis included dizziness (29%), neuralgia (14%), tinnitus/hyperacusis (11%), hearing loss (9%), headache (9%), fatigue (6%), and concentration difficulties (3%). In conclusion, clinical features and outcomes of RHS meningitis were primarily related to cranial neuropathies.

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.

Vestibular mapping in Ramsay-Hunt syndrome and idiopathic sudden sensorineural hearing loss.

PURPOSE: To observe vestibular impairment patterns in patients with Ramsay Hunt syndrome with dizziness (RHS_D) and sudden sensorineural hearing loss with dizziness (SSNHL_D) using hierarchical cluster analysis (HCA) to interpret results with possible mechanisms. METHODS: The data of 30 RHS_D and 81 SSNHL_D patients from January 2017 to August 2022 in a single tertiary referral center were retrospectively analyzed. Video head impulse test (vHIT) and vestibular evoked myogenic potential (VEMP) were used for vestibular analysis of peripheral vestibular organs, and the results of vHIT and VEMP were analyzed. HCA was used to analyze vestibular impairment patterns. RESULTS: In RHS_D patients, the lateral semicircular canal (LSCC) was the most impaired semicircular canal (SCC), followed by the anterior semicircular canal (ASCC) and the posterior semicircular canal (PSCC), and the utricle was more impaired than the saccule. In SSNHL_D patients, the PSCC was the most impaired SCC, followed by the LSCC and the ASCC, and the utricle was more impaired than the saccule. In HCA of RHS_D patients, the ASCC and utricle were initially clustered, followed by the LSCC, PSCC and saccule in order. In the HCA of SSNHL_D patients, the PSCC was solely merged and independently clustered. CONCLUSION: There were different patterns of vestibular impairments between RHS_D and SSNHL_D patients. The vestibular analysis and HCA results of SSNHL_D showed tendency of skip lesion, which could be explained by vascular pathophysiology.

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.

Recurrent facial palsy: Characteristics of ipsilateral and alternative palsies of 104 cases.

OBJECTIVE: Recurrent facial palsy is relatively rare and its clinical details of recurrent facial palsy are not well known. We analyzed recurrent facial palsy cases and clarified its characteristics, especially the difference between ipsilateral and alternative palsies. The analysis aimed to obtain information about recurrent facial palsy that would be useful for delivering explanations to patients and help improve recurrent facial palsy treatments based on the etiology. METHODS: We picked up data from the chart and analyzed the clinical characteristics of patients with recurrent facial palsy from 1243 facial palsy patients (Bell's palsy, VZV-related palsy (Ramsay Hunt syndrome and zoster sine herpete [ZSH])) between 2006 and 2020. RESULTS: Recurrent facial palsy was observed in 104 of 1243 patients (8.4%). There were 35 cases (34%) of ipsilateral palsy and 69 cases (66%) of alternative palsy. The mean age at the onset of the first palsy was 38.9 years old in the ipsilateral group and 48.4 years old in the alternative group, and a significant difference was observed between them. The number of recurrences ranged from 1 to 4. Among the ipsilateral group, 6 patients experienced more than second recurrence. In two cases, the condition failed to resolve after the second recurrence. A serological examination confirmed that 4 cases had recurrent VZV-related palsy (both the first and second palsies were VZV-related) and all of them initially had ZSH: no cases had Hunt syndrome as the first palsy. CONCLUSIONS: The VZV-specific immunity obtained with ZSH might be insufficient to suppress VZV reactivation, and VZV vaccination should be recommended for ZSH patients to prevent further recurrence of VZV-related facial palsy. More than 2 ipsilateral recurrent episodes may be a risk factor for incomplete recovery.

Risk for depression and anxiety in patients with facial palsy based on population-based data from Korea.

BACKGROUND: Several studies point out the association of facial palsy with psychiatric distress such as depression and anxiety. However, there have been few studies on this association based on population-based data. OBJECTIVES: To investigate the risk for depression and anxiety disorders in patients with Bell's palsy and in those with Ramsay Hunt syndrome. MATERIALS AND METHODS: This retrospective study included data from the Korean National Health Insurance Service National Sample Cohort. From 2006 to 2015, patients with Bell's palsy and those with Ramsay Hunt syndrome were defined using diagnostic and medication claim codes. Patients with depression or anxiety were defined using diagnostic and medication claim codes. Occurrences of depression and anxiety were included in analyses. RESULTS: There were significantly higher risks for depressive and anxiety disorders in the group of patients with Bell's palsy than in the control group, as well as in the group of patients with Ramsay Hunt syndrome than in the control group. CONCLUSIONS AND SIGNIFICANCE: There were significantly higher risks for depression and anxiety in patients with facial palsy including Bell's palsy and Ramsay Hunt syndrome based on population-based data from Korea.

Fear of falling as a potential complication of Ramsay Hunt syndrome in older adults: a case report.

BACKGROUND: Fear of falling (FOF) is a common and major health concern in older adults. The consequences of FOF include reduced physical performance, social activity, and health-related quality of life. Ramsay Hunt syndrome (RHS) is a herpes zoster-related facial nerve dysfunction accompanied by an erythematous vesicular rash on the ear or mouth that may complicate ipsilateral facial paralysis and otalgia, vertigo, tinnitus, hearing loss, and meningoencephalitis. However, repeated falls and subsequent FOF due to RHS have not been reported in older adults. CASE PRESENTATION: A 65-year-old woman diagnosed with RHS experienced repeated falls during hospital admission and after discharge. Despite recovery of balance and no subsequent falls, the patient presented with persistent FOF at the geriatric outpatient follow-up visit 1 year after the RHS episode. The fear sensation impaired the patient's instrumental daily activities and was confirmed by documentation of serial comprehensive geriatric assessments, especially the Timed Up and Go test scores. CONCLUSIONS: RHS may cause repeated falls and FOF, leading to impairment in daily activities and psychosocial function in older adults. Therefore, clinicians should be mindful of falls and FOF when caring for older patients with RHS and should develop multidimensional strategies for fall prevention and FOF.

Clinical analysis of acute peripheral facial palsy in older adults.

OBJECTIVE: Acute facial palsy is a consequence of various diseases, with the number of patients increasing with advancing age. This study aimed to analyse the clinical characteristics of acute peripheral facial palsy in older adults. METHODS: A total of 30 patients with a mean age of 68.4 ± 9.1 years were included in the study. All patients received a standardised investigation and follow up. The hospital charts of the patients with acute facial palsy were reviewed retrospectively. RESULTS: The predominant causes of acute facial palsy in older adults were: Bell's palsy, Ramsay Hunt syndrome, trauma, otitis media and malignancy. At baseline, complete and incomplete facial palsies were seen in 26.7 per cent and 73.3 per cent of patients, respectively. The overall rates of good recovery, partial recovery and no recovery were 66.7 per cent, 10 per cent and 23.3 per cent, respectively. Increased age led to a significantly lower level of recovery in older adults. CONCLUSION: Bell's palsy and Ramsay Hunt syndrome were the most common aetiologies of acute facial palsy in older adults, and such patients are likely to have incomplete recovery. Active early treatment is necessary for achieving good outcomes in older adults.

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.

Ramsay Hunt syndrome with multiple cranial neuropathy: a literature review.

BACKGROUND: Ramsay Hunt Syndrome (RHS) is a neurotological disorder involving the reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve (Sweeney and Gilden in J Neurol Neurosurg Psychiatry 71:149-154, 2001). The characteristic presentation involves ipsilateral lower motor neuron type facial paresis, auricular pain with or without hearing impairment, and vesicular lesions of the external auditory canal and outer ear. Involvement of the facial and vestibulocochlear nerve is typical in RHS, whilst multiple cranial neuropathies are rare and associated with poorer prognosis and systemic complications (Arya et al. in Am J Case Rep 19:68-71, 2017; Shinha and Krishna in IDCases 2:47-48, 2015; Shim et al. in Acta Otolaryngol 131:210-215, 2011; Coleman et al. in J Voice 26:e27-e28, 2012; Morelli et al. in Neurol Sci 29:497-498, 2008;). Likely mechanisms involved in the pathogenesis of cranial polyneuropathy include direct peri-neural and trans-axonal spread of viral inflammation between contiguous cranial nerves and haematogenous dissemination between nerves with shared blood supply. Impairments in speech, swallowing, hearing, and oculo-protection can contribute to morbidity and requires a multidisciplinary approach to patient care. METHODS: We present a rare case of RHS with multiple cranial neuropathies followed by a comprehensive review of current literature with regard to the pathophysiology, diagnostic workup, and the management strategies employed in these patients. CONCLUSION: RHSs with multiple cranial neuropathies are important to recognise as they are associated with significant morbidity and poor prognosis. A multidisciplinary approach to patient management is required to address the several complications that can arise from cranial nerve deficits, especially in regard to speech and swallow.

A case report of refractory otalgia after Ramsay Hunt syndrome successfully treated by applying pulsed radiofrequency to the great auricular nerve: A CARE-compliant article.

RATIONALE: Ramsay Hunt syndrome is a type of herpes zoster infection involving geniculate ganglion and facial nerve. Unilateral facial palsy, otalgia, and painful vesicular rash on the auricle and external auditory canal are the typical symptoms. Although postherpetic neuralgia (PHN) is a devastating complication of herpes zoster infection, PHN following Ramsay Hunt syndrome has rarely been reported. PATIENT CONCERNS: A 55-year-old immunocompetent female patient visited our pain clinic, for left-sided refractory otalgia (PHN) that persisted for 3 months after she was diagnosed with Ramsay Hunt syndrome. Although facial palsy and tinnitus had recovered within 2 to 4 weeks after symptom onset, the patient had been experiencing a persistent and severe otalgia radiating to mandibular angle, temporal and upper cervical area of neuropathic nature. DIAGNOSES: The patient's pain persisted despite conservative medication and administration of ultrasound-guided stellate ganglion block, facial nerve block, and great auricular nerve block several times. INTERVENTIONS: The patient was treated with the application of ultrasound-guided pulsed radiofrequency (PRF) to the great auricular nerve. OUTCOMES: The patient experienced significant pain reduction more than 50% on a numeric rating scale after 2 weeks of PRF treatment. LESSONS: Chronic otalgia might be a type of PHN after Ramsay Hunt syndrome with cervical nerve involvement. PRF treatment to the great auricular nerve can be a therapeutic option for refractory otalgia following Ramsay Hunt syndrome.