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.

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.

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.

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.

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.

Can Google Trends analysis confirm the public's need for information about the rare association of facial nerve paralysis with COVID-19 or the COVID-19 vaccination?

Facial nerve paralysis or Bell's palsy have been suggested as possible consequences of SARS-CoV-2 infections, as well as possible side effects of COVID-19 vaccinations. Google Trends data have been used to evaluate worldwide levels of public awareness for these topics for pre- and post-pandemic years. The results demonstrate a relatively low public interest in facial nerve paralysis in comparison to other more common COVID-19 related topics. Some peaks of interest in Bell's palsy can most likely be explained as triggered by the media. Therefore, Google Trends has shown public's relatively low awareness of this rare neurological phenomenon during the pandemic.

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.

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.

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.

Thermographic follow-up of postherpetic neuralgia (PHN) subsequent to Ramsay Hunt syndrome with multicranial nerve (V, VII, VIII and IX) involvement: a case report.

BACKGROUND: Ramsay Hunt syndrome (RHS) is caused by a reactivation of varicella-zoster virus (VZV) infection, and it is characterized by the symptoms of facial paralysis, otalgia, auricular rash, and/or an oral lesion. Elderly patients or immunocompromised patients, deep pain at the initial visit and no prompt treatment are significant predictors of postherpetic neuralgia (PHN). When PHN occurs, especially involved cranial polyneuropathy, multiple modalities should be administered for patients with the intractable PHN. The use of thermography in the follow-up of PHN secondary to RHS with multicranial nerve involvement has not yet been described yet in the literature. CASE PRESENTATION: The patient was a 78-year-old man with the chief complaint of a 3-month history of PHN secondary to RHS with polycranial nerve (V, VII, VIII, and IX) involvement. Multimodality therapy with oral gabapentin, pulsed radiofrequency (PRF) application to the Gasserian ganglion for pain in the trigeminal nerve region, linear-polarized near-infrared light irradiation for pain in the facial nerve region, and 2% lidocaine spray for pain in the glossopharyngeal nerve region was used to the treat patient, and follow-up evaluations included thermography. This comprehensive treatment obviously improved the quality of life, resulting in considerable pain relief, as indicated by a decrease in the numerical rating scale (NRS) score from 9 to 3 and a decrease in thermal imaging temperature from higher to average temperature on the ipsilateral side compared with the contralateral side. Lidocaine spray on the tonsillar branches of the glossopharyngeal nerve resulted in an improvement in odynophagia, and the NRS score decreased from 9 to 0 for glossopharyngeal neuralgia after three applications. CONCLUSION: Although the use of thermography in the follow-up of RHS with multiple cranial nerve (V, VII, VIII, and IX) involvement is very rare, in this patient, thermal imaging showed the efficacy of combination therapy (oral gabapentin, 2% lidocaine sprayed, PRF application and linear-polarized near-infrared light irradiation) and that is a good option for treatment.

Unusual presentation of Ramsay-Hunt Syndrome in kidney transplant patient.

Herpes Zoster (HZ) is caused by reactivation of latent varicella zoster virus (VZV) in craniospinal sensory neurons and is characterized by a painful erythematous rash in the affected dermatome. Although kidney transplant recipients who are chronically maintained on immunosuppressive regimens are considered at risk, there are only a few cases described. We report a well-documented case of a 50-year-old male kidney transplant recipient who presented Ramsay-Hunt syndrome with atypical neurological finds.

Nystagmus in Ramsay Hunt syndrome with or without dizziness.

BACKGROUND: The presence of dizziness has been reported as a negative prognostic factor for recovery of facial palsy in Ramsay Hunt syndrome (RHS). The aim of this study was to investigate the incidence and patterns of nystagmus in RHS patients without dizziness, and discuss possible mechanisms. We also compared the severity and prognosis of facial palsy between RHS patients with and without dizziness. METHODS: From January 2014 to January 2019, 36 patients diagnosed with RHS (27 with dizziness and 9 without dizziness) were included. Patterns of nystagmus were examined and categorized using video-nystagmography. House-Brackmann(HB) grade of facial palsy was compared between RHS patients with and without dizziness. RESULTS: Not only RHS patients with dizziness exhibited nystagmus in most cases (96%, 26 of 27) but also as many as 67% (6 of 9) of RHS patients without dizziness exhibited nystagmus, though the intensity was remarkably weak. In both groups of RHS with and without dizziness, direction-fixed nystagmus and direction-changing positional nystagmus were observed. Initial HB grade and recovery of facial palsy after treatment were not significantly different between RHS with and without dizziness. CONCLUSION: Various patterns of nystagmus including direction-fixed and positional direction-changing nystagmus were observed in RHS patients, and inflammation of the vestibular nerve and inner ear end organs may be responsible for the production of nystagmus in these patients. The results support that the evaluation of vestibular function may be necessary even in RHS patients who do not complain of dizziness or vertigo.

Cranial polyneuropathy caused by herpes zoster infection: a retrospective single-center analysis.

PURPOSE: Cranial polyneuropathy (CP) is a rare complication of herpes zoster (HZ) infection. This entity often produces situations of a diagnostic dilemma, as can be seen in a wide spectrum of clinical presentations. The aim of this study was to report the clinical characteristics, treatment, and outcomes of 11 patients from a single-institution experience. METHODS: A retrospective analysis of patients treated for HZ CP over a 12-year period was performed. RESULTS: The present study included 11 patients with CP caused by HZ infection-7 (63.63%) females, and 4 (36.36%) males. The mean age at presentation was 63 years (range, 38-85 years). Cranial nerve VII was affected in nine (81.82%) cases, CN VIII in six (54.55%) cases, CN V in five (45.45%) cases, CN III and IX in two (18.18%) cases, and CN VI and X in one (9.09%) case. The treatment of choice was acyclovir in all patients, while corticosteroids were administered in six (54.55%) patients. Complete CN recovery was observed in seven (63.63%) patients, while four (36.36%) patients suffered from permanent CN damage-two (18.18%) CN VII, one (9.09%) CN VII and VIII, and one (9.09%) CN VI. CONCLUSION: Herpes zoster CP presents an interesting diagnostic and therapeutic challenge. Successful management of these patients depends on a thorough knowledge of the anatomy and topodiagnostic of CNs. Early administration of antiviral agents is crucial in terms of responsiveness to treatment and expedite recovery.

Role of Biomarkers as Prognostic Factors in Acute Peripheral Facial Palsy.

Acute peripheral facial palsy (APFP), including Bell's palsy and Ramsay Hunt syndrome, is a disease that affects daily life through facial motor dysfunction, causing psychological problems. Various tests to evaluate prognosis have been studied; however, there are no validated predictive biomarkers to guide clinical decision making. Therefore, specific biomarkers that respond to treatment are required to understand prognostic outcomes. In this review, we discuss existing literature regarding the role of APFP biomarkers in prognosis and recovery. We searched the PubMed, EMBASE, and Cochrane Library databases for relevant papers. Our screening identified relevant studies and biomarkers correlating with the identification of predictive biomarkers. Only studies published between January 2000 and October 2021 were included. Our search identified 5835 abstracts, of which 35 were selected. All biomarker samples were obtained from blood and were used in the evaluation of disease severity and prognosis associated with recovery. These biomarkers have been effective prognostic or predictive factors under various conditions. Finally, we classified them into five categories. There is no consensus in the literature on the correlation between outcomes and prognostic factors for APFP. Furthermore, the correlation between hematologic laboratory values and APFP prognosis remains unclear. However, it is important to identify new methods for improving the accuracy of facial paralysis prognosis prediction. Therefore, we systematically evaluated prognostic and potentially predictive APFP biomarkers. Unfortunately, a predictive biomarker validating APFP prognosis remains unknown. More prospective studies are required to reveal and identify promising biomarkers providing accurate prognosis.

Bilateral asymmetrical herpes-zoster with Ramsay hunt syndrome in an immunocompetent adult.

BACKGROUND: Bilateral herpes zoster (BHZ) is an atypical presentation of herpes zoster (HZ), with few cases reported before. Ramsay Hunt syndrome (RHS) is an uncommon complication of VZV infection. Cases of BHZ with RHS in immunocompetent adults have been reported rarely. CASE PRESENTATION: We described an immunocompetent adult who suffered from left-sided thoracic herpes zoster and contralateral RHS simultaneously, and summarizes the characteristics of BHZ. CONCLUSIONS: Cases of BHZ with RHS in immunocompetent adults have not been reported previously. Antivirus - glucocorticoid combination therapy showed a good effect in this case.

Clinical characteristics of herpes zoster laryngitis.

INTRODUCTION: Herpes zoster laryngitis (HZL) is a recently recognized rare disease, easily mistaken for common viral laryngopharyngitis. There are only a few case reports in the English literature. No study has evaluated the clinical characteristics of HZL. In this study, we analyzed the clinical characteristics of HZL and compared them to those of Ramsay Hunt syndrome (RHS). MATERIALS AND METHODS: Seventy-three patients who were initially diagnosed with HZL or RHS were enrolled in this study. Their medical records, including laryngoscopic findings, were analyzed retrospectively. The demographic factors, cranial nerve involvement, and recovery rate of both groups were evaluated. RESULTS: Sixty patients in the non-HZL group and 13 patients in the HZL group were analyzed. Five more patients in the non-HZL group were newly identified with HZL during the retrospective chart review. The mean age of the patients in the HZL group was higher than that of the non-HZL group (p = 0.016). The prevalence of hypertension was higher in the HZL group (p = 0.012). Patients with multiple cranial nerve involvement were more common in the HZL group (p < 0.001). In addition, the prognosis of facial weakness (p = 0.002) and multisensory dizziness (p = 0.006) was poor in HZL group. CONCLUSION: This study showed that a considerable proportion of HZL cases were misdiagnosed or overlooked if not suspected. Considering the poor prognosis of HZL patients with facial paralysis and dizziness, HZL should be diagnosed earlier and treated properly.

A CASE OF MRI-NEGATIVE HERPES VIRUS ENCEPHALITIS PRESENTED BY RAMSAY HUNT SYNDROME.

Ramsay Hunt syndrome (RHS) occurs due to reactivation of latent Varicella Zoster Virus (VZV) infection in the geniculate ganglion of the facial nerve. Major clinical symptoms include ipsilateral facial paralysis, otic pain, and herpetic vesicles (rashes) along the nerve with accompanying ear pain. Rarely clinical findings include retrograde transaxonal spread of the virus from the ganglion into the brain parenchyma with developing the encephalitis or multiple cranial nerve involvement. We describe here a patient with both RHS along with complicating brainstem encephalitis developed due to the coinfection of VZV and EBV.

[Manifestations of herpes zoster ophthalmicus]. / Glaznye proyavleniya opoyasyvayushchego gerpesa.

The article reviews the main ocular features of Herpes Zoster Ophthalmicus (HZO) including Ramsay-Hunt and Tolosa-Hunt syndromes that involve III, IV and VI pairs of cranial nerves and I branch of the trigeminal nerve, and describes treatment methods of its active manifestations and postherpetic neuralgia, as well as herpes zoster vaccination practices.

Atypical clinical manifestations of herpes zoster oticus: diagnostic usefulness of magnetic resonance imaging.

Typical symptoms of Ramsay Hunt syndrome (RHS) consist of painful vesicular eruptions in the external ear, unilateral facial palsy, and/or vestibulocochlear deficit. When RHS patients show atypical clinical manifestations, correct diagnosis can be delayed, and ideal treatment timing for antiviral therapy may be missed. The aim of this study is to describe RHS patients with atypical clinical manifestations and evaluate the usefulness of magnetic resonance imaging (MRI) for early differential diagnosis. We retrospectively reviewed the clinical data and investigated the findings of internal auditory canal (IAC) MRI of seven patients diagnosed with RHS presenting "atypical" clinical manifestations between January 2013 and December 2016. "Typical" symptoms of RHS consist of herpetic vesicular eruption and facial palsy with or without vestibulocochlear deficit. Regardless of symptomatic presentations, IAC MRI demonstrated post-contrast enhancement of cranial nerve (CN) VII, CN VIII, and IAC dura in patients with atypical clinical manifestations. In cases with multiple lower CN palsy, enhancement along the involved nerve was observed on IAC MRI. When RHS was complicated by acute parotiditis, diffuse enhancement of the parotid gland was demonstrated. The present study shows that in IAC MRI of RHS patients with atypical clinical manifestations, post-contrast enhancement was not confined to the facial nerve but also observed in CN VIII and IAC dura regardless of the symptoms, which may facilitate early diagnosis of RHS.