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.

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.

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.

Should acupuncture therapy be used for acute facial paralysis? A protocol for systematic review.

BACKGROUND: Peripheral facial paralysis (PFP) results in functional disorder and social dysfunction, when it is under a severe condition at onset, long-term poor outcomes do occur. Different acupuncture methods have been reported to be potentially effective for shortening the disease course and reducing the occurrence of sequelae when they are applied at an early stage. Neuro edema is a common pathological feature in the acute phase, and many clinical studies have suggested its effect of reducing facial nerve edema. It is of value to estimate the effectiveness and safety of acupuncture treatment at the onset, and to assess the most suitable acupuncture method for the acute period. METHODS AND ANALYSIS: All the RCTs and quasi-RCTs on acupuncture therapy for patients who is during acute stage of PFP will be included. The recovery rate of facial function, the time it takes to restore facial function and the odds of sequelae occurring will be the key parts we focus on. Psychological well-being and quality of life will also be evaluated. Literature searching will be conducted until December 31th, 2022 from eight databases systematically. Two reviewers will screen the literature and extract the data independently. RevMan software will be used for data analysis, and the version 2 of the Cochrane risk-of-bias tool (RoB 2) will be used to assess the certainty of evidence. Forest plots and summary findings will be generated. If data permits, a meta-analysis will be conducted. ETHICS AND DISSEMINATION: Since this study will not involve clinical treatment of patients, ethics approval is not required. The result of this study will be submitted to a peer-reviewed journal for publication and as a proposal for clinical practice and further study on acupuncture treatment at the early stage of PFP. DISCUSSION: This review will summarize the evidence on the different type of acupuncture therapy for acute Bell's palsy and Ramsay-Hunt syndrome. We anticipate that it would be safe and effective when applied to the acute phase of PFP, and some specific suitable acupuncture methods would be found resulting from this review. SYSTEMATIC REVIEW REGISTRATION: International Prospective Register for Systematic Reviews (PROSPERO) number CRD42020205127.

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.

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.

Varicella zoster virus associated-polyradiculoneuritis in an elderly woman: A new subtype of varicella zoster virus neuropathy.

An 82-year-old Japanese woman without underlying disease was admitted to our hospital 3 days after she noticed lower-limb weakness. At presentation, she had lower-leg motor paralysis with mild upper-limb paresis and left Ramsay Hunt syndrome. Cerebrospinal fluid (CSF) findings revealed moderate pleocytosis. A polymerase chain reaction for varicella zoster virus (VZV) DNA in CSF was positive. MRI using 3D Nerve-VIEW (Philips) and contrast T1 images showed high-intensity lesions on the L2-5 and S1-2 spinal roots. A new subtype of VZV-associated polyradiculoneuritis was diagnosed in this patient. We provide the case details and compare three similar reported cases.

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.

[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.

Distribution of Medical Service Use for Facial Palsy Between Medicine and Traditional Korean Medicine Based on Population-Based Data of Korea.

We investigated the distribution of medical service uses for Bell's palsy and Ramsay Hunt syndrome between medicine and traditional Korean medicine using the National Health Insurance Service National Sample Cohort data of Korea from 2006 to 2015. Patients were identified with diagnostic codes and medication or treatment claim codes. For Bell's palsy, there were 5,970 (68.8%) patients who used traditional Korean medical service only, whereas for Ramsay Hunt syndrome, there were 749 (93.6%) patients who used medical service only. The proportion of traditional Korean medical service use was higher than that of medical service use in patients with Bell's palsy, while the opposite was found in patients with Ramsay Hunt syndrome.