The natural history of progressive myoclonus ataxia.

Progressive myoclonus ataxia (PMA) is a rare clinical syndrome characterized by the presence of progressive myoclonus and ataxia, and can be accompanied by mild cognitive impairment and infrequent epileptic seizures. This is the first study to describe the natural history of PMA and identify clinical, electrophysiological, and genetic features explaining the variability in disease progression. A Dutch cohort of consecutive patients meeting the criteria of the refined definition of PMA was included. The current phenotype was assessed during in-person consultation by movement disorders experts, and retrospective data was collected to describe disease presentation and progression, including brain imaging and therapy efficacy. Extensive genetic and electrophysiological tests were performed. The presence of cortical hyperexcitability was determined, by either the identification of a cortical correlate of myoclonic jerks with simultaneous electromyography-electroencephalography or a giant somatosensory evoked potential. We included 34 patients with PMA with a median disease duration of 15 years and a clear progressive course in most patients (76%). A molecular etiology was identified in 82% patients: ATM, CAMTA1, DHDDS, EBF3, GOSR2, ITPR1, KCNC3, NUS1, POLR1A, PRKCG, SEMA6B, SPTBN2, TPP1, ZMYND11, and a 12p13.32 deletion. The natural history is a rather homogenous onset of ataxia in the first two years of life followed by myoclonus in the first 5 years of life. Main accompanying neurological dysfunctions included cognitive impairment (62%), epilepsy (38%), autism spectrum disorder (27%), and behavioral problems (18%). Disease progression showed large variability ranging from an epilepsy free PMA phenotype (62%) to evolution towards a progressive myoclonus epilepsy (PME) phenotype (18%): the existence of a PMA-PME spectrum. Cortical hyperexcitability could be tested in 17 patients, and was present in 11 patients and supported cortical myoclonus. Interestingly, post-hoc analysis showed that an absence of cortical hyperexcitability, suggesting non-cortical myoclonus, was associated with the PMA-end of the spectrum with no epilepsy and milder myoclonus, independent of disease duration. An association between the underlying genetic defects and progression on the PMA-PME spectrum was observed. By describing the natural history of the largest cohort of published patients with PMA so far, we see a homogeneous onset with variable disease progression, in which phenotypic evolution to PME occurs in the minority. Genetic and electrophysiological features may be of prognostic value, especially the determination of cortical hyperexcitability. Furthermore, the identification of cortical and non-cortical myoclonus in PMA helps us gain insight in the underlying pathophysiology of myoclonus.

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.

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.

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.

Otologic disease trends in Japan post-COVID-19 outbreak: A retrospective time-series analysis.

OBJECTIVE: To evaluate the potential impact of coronavirus disease 2019 (COVID-19) and vaccinations on otologic diseases, including facial nerve paralysis (including Ramsay Hunt syndrome), vestibular neuritis, sudden sensorineural hearing loss, and Meniere's disease. METHODS: In this retrospective study, we conducted a time-series analysis employing a causal impact algorithm on a large-scale inpatient database in Japan. We compared the actual number of hospitalized patients with otologic diseases to two predictions: one without any covariates and another with a covariate accounting for the reduction in the number of hospitalized patients due to lockdown measures. Additionally, we performed Granger causality tests to ensure the robustness of our findings. RESULTS: No significant increase was noted in the number of hospitalized patients with otologic diseases following the onset of the COVID-19 pandemic in the causal impact analysis. Similarly, no notable surge was observed in hospitalizations for these diseases following the introduction of the COVID-19 vaccine. The Granger causality tests results aligned with the causal impact analysis findings. CONCLUSION: Our findings indicate that COVID-19 and vaccinations had minimal discernible effects on hospitalization of patients with otologic diseases, suggesting that otologic diseases may not be significantly impacted by COVID-19 and vaccinations, which could have implications for public health policies and the allocation of healthcare resources during a pandemic. Further research and monitoring of long-term effects are warranted to validate these findings and guide healthcare decision-making.

Prognostic value of electroneurography using the midline method for predicting the development of synkinesis after peripheral facial palsy.

OBJECTIVE: The prognostic value of electroneurography (ENoG) for predicting the incidence of synkinesis is reportedly about 40 % using the formal standard method (ENoG-SM). However, the prognostic value of ENoG using the newly developed midline method (ENoG-MM) has not been determined. The aim of this study was to demonstrate the optimal prognostic value and advantages of ENoG-MM for predicting the incidence of synkinesis. METHODS: Participants were 573 patients treated for peripheral facial palsy including Bell's palsy or Ramsay Hunt syndrome. We investigated the clinical presence of any oral-ocular or ocular-oral synkinesis from the medical records. ENoG-MM and ENoG-SM were performed 10-14 days after symptom onset. In ENoG-MM, compound muscle action potentials were recorded by placing the anode on the mental protuberance and the cathode on the philtrum. In ENoG-SM, electrodes were placed on the nasolabial fold. Synkinesis was clinically assessed at the end of follow-up or at >1 year after onset. The sensitivity and specificity of ENoG values for predicting the incidence of synkinesis were compared between ENoG-MM and ENoG-SM at every 5 % around 40 % (range, 30-50 %). RESULTS: At every 5 % of ENoG values around 40 %, ENoG-MM provided higher sensitivity and lower specificity for predicting the incidence of synkinesis compared with ENoG-SM. In particular, when the cut-off value was set at 45 %, sensitivity was 100 % and 95.3 % with ENoG-MM and ENoG-SM, respectively. CONCLUSION: In peripheral facial palsy, ENoG-MM offered higher sensitivity than ENoG-SM for predicting synkinesis. ENoG-MM is useful for screening patients at risk of developing synkinesis. In clinical practice, an ENoG-MM cut-off value of 45 % must be the optimal prognostic value because of the 100 % sensitivity.

Physical therapy for peripheral facial palsy: A systematic review and meta-analysis.

OBJECTIVE: This study aimed to reveal the efficacy of physical therapy for patients with peripheral facial palsy. METHODS: A literature search was conducted using PubMed, Ichushi-Web, and Cochrane Central Register of Controlled Trials. Published randomized controlled trials comparing the physical therapy versus placebo/non-treatment for peripheral facial palsy such as Bell's palsy, Ramsay Hunt syndrome, and traumatic facial palsy were included for meta-analysis. The primary outcome was non-recovery at the end of the follow-up. Non-recovery was defined according to the authors' definition. The secondary outcomes were the composite score of the Sunnybrook facial grading system and sequelae (presence of synkinesis or hemifacial spasm) at the end of the follow-up. Data was analyzed using Review Manager software and pooled risk ratio (RR) or mean difference (MD) with 95% confidence intervals (CI) were calculated. RESULTS: Seven randomized controlled trials met the eligible criteria. The data on non-recovery from four studies was obtained and included 418 participants in the meta-analysis. Physical therapy might reduce non-recovery (RR = 0.51 [95% CI = 0.31-0.83], low quality). Pooling the data of composite score of the Sunnybrook facial grading system from three studies (166 participants) revealed that physical therapy might increase the composite scores (MD = 12.1 [95% CI = 3.11-21.0], low quality). Moreover, we obtained data on sequelae from two articles (179 participants). The evidence was very uncertain about the effect of physical therapy on reduction of sequelae (RR = 0.64 [95% CI = 0.07-5.95], very low quality). CONCLUSION: The evidence suggested that physical therapy reduces non-recovery in patients with peripheral facial palsy and improves the composite score of the Sunnybrook facial grading system, whereas the efficacy of physical therapy in reducing sequelae remained uncertain. The included studies had high risk of bias, imprecision, or inconsistency; therefore, the certainty of evidence was low or very low. Further well-designed randomized controlled trials are needed to confirm its efficacy.

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 in Asymptomatic COVID-19 Infection: A Case Report and a Literature Review.

(1) Background: COVID-19 infection has affected almost 6 million people worldwide. Geniculate Ganglion Zoster resulting in Ramsay Hunt Syndrome (RHS) has been rarely described in this context. (2) Methods: Here, a case of RHS in the context of asymptomatic COVID-19 infection is reported followed by a literature review of the previously published cases (PubMed research combining "COVID-19" and "Ramsay Hunt Syndrome" or their abbreviations/synonyms, searching for data published at any time till October 2023). (3) Results: Five cases have been previously published (age range: 25-67 years; n = 3 males). Three patients were known to be immunocompetent prior to infection, one was receiving corticotherapy for lung disease, and one had an unspecified immune status. RHS predominantly involved both facial and vestibulocochlear nerves, with one case exclusively involving the facial nerve as the presented case. Regarding facial nerve palsy, three were right-sided (like the current report) and two were left-sided. Two cases were asymptomatic to COVID-19 (like the present patient), one had mild fatigue, and two had classical COVID-19 symptoms preceding RHS symptoms. Workup included serological testing against Varicella Zoster Virus and PCR assays that can detect the viral DNA in saliva, blood, tears, exudates, and cerebrospinal fluid. The treatment combined antiviral and corticosteroid therapies which yielded heterogeneous outcomes that might be related to some demographic and clinical data. (4) Conclusions: RHS rarely occurs in the context of COVID-19. Early recognition is important. Management seems to be similar to the classical condition. Some data may help predict facial nerve recovery.

Predicting synkinesis caused by Bell's palsy or Ramsay Hunt syndrome using machine learning-based logistic regression.

Objective: To investigate whether machine learning (ML)-based algorithms, namely logistic regression (LR), random forest (RF), k-nearest neighbor (k-NN), and gradient-boosting decision tree (GBDT), utilizing early post-onset parameters can predict facial synkinesis resulting from Bell's palsy or Ramsay Hunt syndrome more accurately than the conventional statistics-based LR. Methods: This retrospective study included 362 patients who presented to a facial palsy outpatient clinic. Median follow-up of synkinesis-positive and -negative patients was 388 (range, 177-1922) and 198 (range, 190-3021) days, respectively. Electrophysiological examinations were performed, and the rate of synkinesis in Bell's palsy and Ramsay Hunt syndrome was evaluated. Sensitivity and specificity were assessed using statistics-based LR; and electroneurography (ENoG) value, the difference in the nerve excitability test (NET), and scores of the subjective Yanagihara scaling system were evaluated using early post-onset parameters with ML-based LR, RF, k-NN, and GBDT. Results: Synkinesis rate in Bell's palsy and Ramsay Hunt syndrome was 20.2% (53/262) and 40.0% (40/100), respectively. Sensitivity and specificity obtained with statistics-based LR were 0.796 and 0.806, respectively, and the area under the receiver operating characteristic curve (AUC) was 0.87. AUCs measured using ML-based LR of "ENoG," "difference in NET," "Yanagihara," and all three components ("all") were 0.910, 0.834, 0.711, and 0.901, respectively. Conclusion: ML-based LR model shows potential in predicting facial synkinesis probability resulting from Bell's palsy or Ramsay Hunt syndrome and has comparable reliability to the conventional statistics-based LR. Level of Evidence: 3.

Cochleo-Vestibular Disorders in Herpes Zoster Oticus: A Literature Review and a Case of Bilateral Vestibular Hypofunction in Unilateral HZO.

The varicella-zoster virus (VZV), a member of the Herpesviridae family, causes both the initial varicella infection and subsequent zoster episodes. Disorders of the eighth cranial nerve are common in people with herpes zoster oticus (HZO). We performed a review of the literature on different databases including PubMed and SCOPUS, focusing on cochlear and vestibular symptoms; 38 studies were considered in our review. A high percentage of cases of HZO provokes cochlear and vestibular symptoms, hearing loss and vertigo, whose onset is normally preceded by vesicles on the external ear. It is still under debate if the sites of damage are the inferior/superior vestibular nerves and cochlear nerves or a direct localization of the infection in the inner ear. The involvement of other contiguous cranial nerves has also been reported in a few cases. We report the case of a patient with single-side HZO presenting clinical manifestations of cochleo-vestibular damage without neurological and meningeal signs; after 15 days, the patient developed a new episode of vertigo with clinical findings of acute contralateral vestibular loss. To our knowledge, only three other such cases have been published. An autoimmune etiology may be considered to explain these findings.

Efficacy of 1064 nm Photobiomodulation Dosimetry Delivered with a Collimated Flat-Top Handpiece in the Management of Peripheral Facial Paralysis in Patients Unresponsive to Standard Treatment Care: A Case Series.

Peripheral facial paralysis (PFP) is a common condition where oxidative stress (OS) is involved in the pathophysiology of facial paralysis, inhibiting peripheral nerve regeneration, which can be featured in Bell's palsy, Ramsay Hunt syndrome and Lyme disease. The current standard care treatments lack consensus and clear guidelines. Hence, the utilization of the antioxidant immunomodulator photobiomodulation (PBM) can optimize clinical outcomes in patients who are unresponsive to standard care treatments. Our study describes three unique cases of chronic PFP of various origins that were unresponsive to standard care treatments, but achieved a significant and complete recovery of facial paralysis following PBM therapy. Case presentations: Case #1: a 30-year-old male who presented with a history of 12 years of left-side facial paralysis and tingling as a result of Bell's palsy, where all the standard care treatments failed to restore the facial muscles' paralysis. Eleven trigger and affected points were irradiated with 1064 nm with an irradiance of ~0.5 W/cm2 delivered with a collimated prototype flat-top (6 cm2) in a pulsed mode, with a 100 µs pulse duration at a frequency of 10 Hz for 60 s (s) per point. Each point received a fluence of 30 J/cm2 according to the following treatment protocol: three times a week for the first three months, then twice a week for another three weeks, and finally once a week for the following three months. The results showed an improvement in facial muscles' functionality (FMF) by week two, whereas significant improvement was observed after 11 weeks of PBM, after which the House-Brackmann grading scale (HBGS) of facial nerve palsy dropped to 8 from 13 prior to the treatment. Six months after PBM commencement, electromyography (EMG) showed sustainability of the FMF. Case #2: A five-year-old female who presented with a 6-month history of severe facial paralysis due to Lyme disease. The same PBM parameters were utilized, but the treatment protocol was as follows: three times a week for one month (12 consecutive treatment sessions), then the patient received seven more sessions twice a week. During the same time period, the physiotherapy of the face muscles was also delivered intensively twice a week (10 consecutive treatments in five weeks). Significant improvements in FMF and sustainability over a 6-month follow-up were observed. Case #3: A 52-year-old male who presented with severe facial palsy (Grade 6 on HBGS) and was diagnosed with Ramsay Hunt syndrome. The same laser parameters were employed, but the treatment protocol was as follows: three times a week for three weeks, then reduced to twice a week for another three weeks, then weekly for the next three months. By week 12, the patient showed a significant FMF improvement, and by week 20, complete FMF had been restored. Our results, for the first time, showed pulsed 1064 nm PBM delivered with a flat-top handpiece protocol is a valid and its treatment protocol modified, depending on the origin and severity of the condition, which is fundamental in optimizing facial paralysis recovery and alleviating neurological symptoms. Further extensive studies with large data are warranted to validate our PBM dosimetry and treatment protocols.

Homeopathic Treatment of Ramsay Hunt Syndrome: A Case Report.

INTRODUCTION: Ramsay Hunt syndrome (RHS) is an uncommon neurological complication resulting from the reactivation of latent herpes zoster virus. The condition often presents with facial paralysis, palatal ulcers, dysphagia, and altered taste sensation, leading to reduced quality of life. Standard therapeutic options for RHS have limitations, prompting the exploration of alternative treatments with improved prognostic outcomes. This case report aims to present a noteworthy clinical observation of RHS managed with individualized homeopathic treatment, emphasizing its potential therapeutic effect. CASE DESCRIPTION: A 24-year-old female patient exhibited left-sided facial weakness, along with palatal ulcers, dysphagia, and ageusia, prompting the diagnosis of RHS. Following the principles of homeopathy, a personalized therapeutic regimen was formulated, consisting tailored administration of Rhus toxicodendron, Spigelia anthelmia, and Sulfur. The House-Brackmann scale was employed to objectively assess the severity of facial palsy, while photographic documentation tracked the progression of palatal ulcers and facial paralysis. Over a carefully monitored observation period of 14 days, the patient demonstrated notable therapeutic response. There was a significant reduction in the extent of palatal ulceration and left-sided facial palsy exhibited marked improvement. Subsequent days of follow-up witnessed a consistent amelioration of the patient's condition, substantiating the effect of the individualized homeopathic treatment. CONCLUSION: This case report highlights an exceptional instance of RHS recovery within a relatively short timeframe, achieved through the administration of individualized homeopathic therapy. The favorable outcomes observed in this case underscore the potential of homeopathy as a promising intervention for RHS management. Nevertheless, further systematic investigations are imperative to comprehensively evaluate the scope and applicability of homeopathy in the treatment of RHS.Das Ramsay­Hunt­Syndrom (RHS) ist eine seltene neurologische Komplikation, die durch die Reaktivierung einer latenten Herpes­Zoster­Virusinfektion verursacht wird. Die Krankheit manifestiert sich häufig mit Gesichtslähmung, Ulcerationen am Gaumen, Dysphagie und verändertem Geschmacksempfinden und ist mit einer Einschränkung der Lebensqualität verbunden. Die Standardtherapieoptionen für RHS sind begrenzt, weshalb nach alternativen Behandlungsmöglichkeiten mit besseren prognostischen Ergebnissen gesucht wird. Im vorliegenden Fallbericht wird eine interessante klinische Beobachtung bei RHS vorgestellt, das mit individualisierter Homöopathie behandelt wurde, und deren potenzielle therapeutische Wirksamkeit wird hervorgehoben.Eine 24-jährige Patientin zeigte eine linksseitige Gesichtsschwäche in Verbindung mit Ulcerationen am Gaumen, Dysphagie und Ageusie, so dass die Diagnose RHS gestellt wurde. Gemäß den Prinzipien der Homöopathie wurde ein personalisiertes Therapieschema formuliert, das die individuell zugeschnittene Gabe von Rhus toxicodendron, Spigelia anthelmia, und Sulphur umfasste. Die objektive Bewertung des Schweregrads der Gesichtslähmung erfolgte mithilfe der House-Brackmann-Skala, wohingegen das Fortschreiten der Gaumenulcerationen und der Gesichtslähmung fotografisch dokumentiert wurde. Während eines sorgfältig überwachten Beobachtungszeitraums von 14 Tagen zeigte die Patientin ein deutliches therapeutisches Ansprechen. Das Ausmaß der Gaumenulcerationen ging signifikant zurück, und die linksseitige Gesichtslähmung besserte sich deutlich. In den folgenden Tagen besserte sich der Zustand der Patientin kontinuierlich, was die Wirkung der individualisierten homöopathischen Behandlung untermauert.Dieser Fallbericht beleuchtet einen ungewöhnlichen Fall von Genesung nach einem RHS innerhalb relativ kurzer Zeit, die durch Verabreichung einer individualisierten homöopathischen Therapie erreicht wurde. Die im vorliegenden Fall beobachteten günstigen Ergebnisse unterstreichen das Potenzial der Homöopathie als vielversprechende Intervention zur Behandlung von RHS. Allerdings sind weitere systematische Untersuchungen unabdingbar, um den Umfang und die Anwendbarkeit der Homöopathie bei der Behandlung von RHS umfassend zu beurteilen.

Ramsay Hunt Syndrome with Pharyngolaryngeal Involvement Mimicking Acute Stroke: A Case Report.

Ramsay Hunt syndrome is a clinical manifestation of the reactivation of latent varicella zoster virus in the geniculate ganglion after acute infection. It is commonly associated with an acute peripheral facial nerve paralysis, auricular vesicular eruption and acute sensorineural hearing loss. However, in some rare cases the involvement of other cranial nerves, especially the glossopharyngeal and vagal is described. We present a 56-year-old male patient who was diagnosed with acute pharyngitis. Two days later, the patient developed dysarthria, hoarseness, and left side facial weakness. Considering a patient's history of cardiovascular events, the acute stroke was suspected. Magnetic resonance imaging ruled out an acute cerebrovascular accident. The patient's otalgia aggravated and he reported hearing loss. Clinical examination revealed facial paralysis affecting the left side of the face, erythematous rash on the left auricle, multiple vesicles present on patient's left tympanic membrane, ulcerous lesion on the left side of the soft palate and ipsilateral velopharyngeal palsy. Indirect laryngoscopy revealed ulcerous lesions in the left side of the epiglottis and left vocal cord paralysis. The patient was diagnosed with Ramsay Hunt syndrome with cranial polyneuropathy. Oral acyclovir and oral prednisolone were administered. Seven months after discharge, facial paralysis improved to lower grade and flexible laryngoscopy showed that the left vocal cord had resumed normal movement. Ramsay Hunt syndrome can be accompanied by involvement of other cranial nerves. Previous history of hypertensive crises and myocardial infarction may influence a diagnosis of Ramsay Hunt syndrome, especially when it has atypical presentation. However, this diagnosis should be considered in patients with unilateral multiple cranial nerve palsies.

Association between Serum Varicella-Zoster Virus IgM and IgG and Prognosis of Ramsay Hunt Syndrome.

Ramsay Hunt syndrome (RHS) has a poor prognosis because of varicella-zoster virus (VZV) infection. This is most closely related to severe inflammation in the geniculate ganglion of the facial nerve due to VZV infection or reactivation. This study investigated whether there were differences in the prognosis and accompanying symptoms of facial paralysis based on the presence or absence of VZV IgM and IgG antibodies. This study was conducted as a retrospective chart analysis of 105 patients with RHS who were admitted to our hospital between 2015 and 2021. The House-Brackmann (HB) grade and electroneurography (ENoG) was used to evaluate the degree of facial paralysis. Patients' subjective symptoms were evaluated by dividing them into dizziness, tinnitus, hyperacusis, and hearing loss. No difference was observed in the initial HB grade with or without IgM; however, the final HB grade was significantly higher in IgM-positive patients than in IgM-negative patients (p < 0.05). Further, when IgM was positive, the value of the orbicularis oculi muscle in the ENoG test results was significantly higher (p < 0.05), and symptoms of tinnitus and hyperacusis occurred more frequently (p < 0.05). The initial and final HB grades were significantly higher in IgG-positive patients than in IgG-negative patients (p < 0.05). When IgG was positive, the values of nasalis and oris muscles in the ENoG test results were significantly higher (p < 0.05), and symptoms of dizziness occurred more frequently (p < 0.05). This study confirmed that the more active the immunological action of the VZV in the body, the greater the damage to the facial and vestibulocochlear nerves, which are associated with the degree of facial paralysis and the accompanying otologic symptoms.

Peripheral Nerve Stimulation of the Lesser Occipital and Greater Auricular Nerve for Post Herpetic Neuralgia in a Case of Ramsay Hunt Syndrome: Case Report.

Background: Post-herpetic neuralgia (PHN) is a painful condition that presents after herpes zoster reactivation in the peripheral and central nervous system. When medical treatment fails, options are limited, and patients may suffer with chronic pain indefinitely. Case Presentation: We present the case of a gentleman with a 3-year history of PHN in the distribution of the right lesser occipital and greater auricular nerves that failed to respond to medical treatment. He underwent a trial of neuromodulation, and post-operative pain scores improved by 80%, and at 60 days post-operatively his average pain score was 0 out of 10. Conclusions: PNS is an effective and safe option for the treatment of chronic pain, and we present a report of successful treatment of PHN in a particularly difficult anatomic distribution. PNS of the lesser occipital and greater auricular nerves is a novel treatment for PHN and shows promise as an effective, safe therapy when other treatment fails.

Síndrome de Ramsay Hunt en paciente epiléptico en Santander, Colombia: Reporte de caso / Ramsay Hunt Syndrome in an epileptic patient in Santander, Colombia: Case report

El Síndrome de Ramsay Hunt es una entidad infrecuente, con una incidencia de 5 por cada 100.000 personas por año. Esta condición se caracteriza por una reactivación del virus de la varicela-zoster en el nervio facial. Su diagnóstico implica un reto para el médico puesto que suele ser netamente clínico, con la aparición de una triada consistente en: otalgia, parálisis facial ipsilateral y vesículas en el canal auditivo. El objetivo del artículo es presentar el caso de una mujer de 49 años de edad, con antecedente de epilepsia en tratamiento anticonvulsivante, quien ingresa con la triada clínica antes descrita, asociada a visión borrosa derecha y vértigo. La paciente fue tratada con antivirales y corticoides orales, presentando una resolución clínica favorable dado una reducción de más del 50% de las lesiones cutáneas. No se identificaron diferencias respecto a la presentación clínica de este síndrome al compararse con pacientes no epilépticos.

Ramsay Hunt Syndrome is a rare entity, with an incidence of 5 per 100,000 people per year. This condition is characterized by a reactivation of the varicella-zoster virus in the facial nerve. Its diagnosis implies a challenge for the physician since it is usually a clinical diagnosis, with the appearance of a clinical triad consisting of: otalgia, ipsilateral facial paralysis and vesicles in the ear canal. The objective of the article is to present the case of a 49-year-old woman, with a history of epilepsy receiving anticonvulsant treatment, who was admitted with the aforementioned clinical triad, associated with blurred right vision and vertigo. The patient was treated with oral antiviral management and oral corticosteroids, presenting a favorable clinical resolution given a reduction of more than 50% of the skin lesions. No differences were identified regarding the clinical presentation of this syndrome when compared with non-epileptic patients.

A Case Report of Refractory Postherpetic Neuralgia After Ramsay Hunt Syndrome Treated With Tympanic Nerve Neurectomy.

Ramsay Hunt syndrome is a facial nerve palsy that arises from herpes zoster infection. In rare cases, postherpetic neuralgia is a complication following Ramsay Hunt syndrome. Pain management to address postherpetic neuralgia includes facial nerve blocks, medications such as gabapentin, carbamazepine and botulinum toxin injections, and pulsed radiofrequency. Despite the reported benefits for patients with glossopharyngeal nerve pain, neurectomy as a treatment has rarely been described. A 45-year-old patient visited our ENT clinic for chronic right-sided facial, ear, and jaw pain that persisted for 9 years following the development of Ramsay Hunt syndrome. She trialed multiple medications including gabapentin, carbamazepine, and botulinum toxin injections with minimal relief to her symptoms. The patient underwent a diagnostic myringotomy with topical application of lidocaine to the tympanic nerve. This resulted in temporary relief of her pain until the effects of the lidocaine subsided. The patient was subsequently offered lysis of the right tympanic nerve for more definitive management. The patient experienced significant pain reduction after the right tympanic neurectomy procedure. Chronic postherpetic neuralgia following Ramsay Hunt syndrome can cause significant impairment in a patient's quality of life. For patients with ear pain refractory to conservative management, a tympanic neurectomy can be considered.

Impact on online research on celebrities' uncommon diseases: the curious case of Justin Bieber and Ramsay Hunt syndrome.

Aim: We investigated how to use Internet user searches to gauge the impact of a celebrity illness on global public interest. Methods: The study design is cross-sectional. Data on Internet searches were obtained from Google Trends (GT) for the period between 2017-2022 using the search words "Ramsay Hunt syndrome" (RHS), "Ramsay Hunt syndrome type 2," "Herpes zoster," and "Justin Bieber." The frequency of specific page views for "Ramsay Hunt syndrome," "Ramsay Hunt syndrome type 1," Ramsay Hunt syndrome type 2," Ramsay Hunt syndrome type 3," "Herpes zoster," and "Justin Bieber" were collected via a Wikipedia analysis tool that shows the number of times a specific page is viewed. Statistical analyses were performed using the Pearson (r) and Spearman's rank correlation coefficient (rho). Results: GT data, in 2022, show a strong correlation for Justin Bieber and RHS or RHS type 2 (r = 0.75); similarly, Wikipedia data show a strong correlation for Justin Bieber and the others explored terms (r > 0.75). Furthermore, the correlation was strong between GT and Wikipedia for RHS (rho = 0.89) and RHS type 2 (rho = 0.88). Conclusions: The peak search times for the GT and Wikipedia pages were during the same period. Useful new tools and analyses of Internet traffic data may be effective in assessing the impact of announced celebrity uncommon illnesses on global public interest.