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.

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.

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.
CONCLUSIONS: 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.

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.
CONCLUSIONS: 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.

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.
CONCLUSIONS: 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.

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.
CONCLUSIONS: This systematic review first shows the effectiveness of antivirals. Further study is needed to confirm the efficacy of antivirals.

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.

UNASSIGNED: 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.
UNASSIGNED: To investigate the risk for depression and anxiety disorders in patients with Bell\'s palsy and in those with Ramsay Hunt syndrome.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.

(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.

BACKGROUND: 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.
CONCLUSIONS: This study suggests that patients with DM had an increased risk of acute facial palsy including BP and RHS.