Síndrome de Ramsay Hunt: reporte de un caso clínico / Ramsay Hunt Syndrome: clinical case report

El virus varicela zoster produce cuadros clínicos por reactivación del mismo en años posteriores a la primoinfección, en sujetos con condiciones particulares, como situaciones de estrés, inmunodepresión, radioterapia, estados infecciosos que cursen con fiebre, entre otros. El síndrome de Ramsay Hunt es uno de ellos. Se caracteriza por una tríada de parálisis facial periférica, vesículas herpéticas en oído externo y otalgia. Objetivo: pr esentar un caso típico del síndr ome de Ramsay Hunt, métodos diagnósticos y tratamiento realizado. Presentación de caso clínico: paciente femenina, de 21 años, originaria de Tegucigalpa, Honduras, se presentó al Hospital Escuela Universitario por cuadro de seis días de evolución, de cambios inflamatorios en el oído externo izquierdo, en la región del pabellón auricular y el conducto auditivo externo, con presencia de vesículas herpéticas eritematosas con costras y restos hemáticos, otorrea, hiperemia, prurito y otalgia intermitente. También refirió parálisis de hemicara izquierda de cuatro días de evolución. Examen físico: conducto auditivo externo izquierdo presentaba vesículas con costras. Hubo ptosis palpebral izquierda e incapacidad para realizar las mímicas faciales. Tratamiento intrahospitalario: aciclovir 800 mg vía oral cada 6 horas, dexametasona 8 mg IV cada 8 horas, diclofenaco 75 mg IV cada 12 horas; citidina-5- monofosfato disódico, más uridin-5-trifosfato trisódico, 1 comprimido cada 12 horas; mupirocina ungüento al 1Universidad Nacional Autónoma de Honduras, Facultad de Ciencias Médicas, Tegucigalpa, Honduras. ORCID 0000-0002-2346-4209 2Universidad Nacional Autónoma de Honduras, Facultad de Ciencias Médicas. Departamento de Cirugía. Tegucigalpa, Honduras. 3Hospital Escuela Universitario, Departamento de Cirugía, Tegucigalpa, Honduras. Autor de correspondencia: Paola Andrea Hincapié Gaviria, paolahincapie23@gmail.com Recibido: 10/07/2020 Aceptado: 03/03/2021 al 2%, aplicado en oído externo cada 8 horas y ejercicios de fisioterapia. Conclusión: el síndrome de Ramsay Hunt se diagnóstica, fundamentalmente, mediante hallazgos clínicos. El tratamiento empírico y adecuado, acompañado de una buena terapia posterior, anticipa un pronóstico favorable sin secuelas neurológicas...(AU)

A Case of Recurred Herpes Zoster Oticus Concomitantly Occurred with Zoster Laryngopharyngitis Without Vocal Cord Palsy

Herpes zoster oticus is one of complication of varicella zoster virus (VZV) reactivation in the geniculate ganglion of the facial nerve, which is the most common presentation of herpes zoster in the head and neck region. However, VZV infection of the larynx has rarely been described in the literature compared with Herpes zoster oticus. Moreover, zoster laryngopharyngitis simultaneously occurred with recurred Herpes zoster oticus which has no newly developing motor dysfunction has not been reported yet. Therefore, these diseases are difficult to diagnose due to its rareness. However, distinctive appearances such as unilateral herpetic mucosal eruptions and vesicles are useful and essential in making a quick and accurate diagnosis. Thus, we report a characteristic case of zoster laryngopharyngitis simultaneously occurred with recurred Herpes zoster oticus not accompanied by any newly developing motor palsy.

Ramsay Hunt syndrome

Ramsay Hunt syndrome is a type of acute herpes zoster, which occurs by reactivation of the varicella-zoster virus at the geniculate ganglion. Clinical presentation of Ramsay Hunt syndrome includes a vesicular rash on the ear (herpes zoster oticus) or in the oral mucosa accompanied by acute peripheral facial nerve paralysis. Other cranial nerves such as V, IX, XI, and XII are often involved. Additional variability of the clinical picture of Ramsay Hunt syndrome is produced by varying patterns of skin involvement explained by individual anastomoses between cranial and cervical nerves. Combination treatment containing anti-viral agents and steroids is recommended for the treatment of Ramsay Hunt syndrome. Additionally, early diagnosis of Ramsay Hunt syndrome is a crucial factor to improve damaged nerves in Ramsay Hunt syndrome, which initiates treatment as soon as possible.

Total Transcanal Endoscopic Facial Nerve Decompression for Traumatic Facial Nerve Palsy

A few approaches can be used to decompress traumatic facial nerve paralysis including the middle cranial fossa approach or transmastoid approach depending on the site of injury. In some specific situation of treating traumatic facial nerve palsy whose injured site was confined from the geniculate ganglion to the second genu, transcanal endoscopic approach for facial nerve decompression can be used. We performed two cases of total endoscopic transcanal facial nerve decompression in patients with traumatic facial nerve palsy. After a six month follow-up, both patients showed improvement in facial function by 2 grades according to House-Brackmann grade system. In terms of treatment outcomes, total transcanal endoscopic facial nerve decompression for traumatic facial nerve palsy is an alternative for lesions limited to the tympanic segment I, and has an advantages of being minimally invasive and is cosmetically acceptable without an external scar or bony depression due to drilling.

Herpes Zoster Oticus without Facial Palsy and Hearing Loss Masquerading as Vestibular Neuritis

Herpes zoster oticus, also called Ramsay-Hunt syndrome, involves the geniculate ganglion and presents with facial nerve palsy, sensorineural hearing loss, vestibular dysfunction, and auricular vesicular lesion. In this case, the patient presented with isolated vestibular dysfunctions without facial palsy and hearing impairment, and these findings were confirmed by electrophysiologic studies including video head impulse testing. Clinicians should be aware of this variation, and differentiate it from vestibular neuritis which usually does not require intensive antiviral therapies.

Ramsay Hunt Syndrome in a Patient Infected with Human Immunodeficiency Virus / 대한내과학회지

Ramsay Hunt syndrome is caused by reactivation of the varicella zoster virus in the geniculate ganglion of the sensory branch in the face and ears. It is characterized by peripheral facial palsy, ear pain, and vesicles in the auditory canal and auricle. We report on a first case of Ramsay Hunt syndrome in a patient with human immunodeficiency virus in Korea. The patient, a 40-year-old male, first presented with otalgia and ear fullness. On admission, he had right facial palsy of the peripheral type, otorrhea, headache, limited tongue movement, and right auricle vesicular eruptions. He had positive human immunodeficiency virus antibody and Western blot tests. His CD4 T cell count was 281/microL. The patient was treated with valacyclovir and steroid with highly active antiretroviral therapy. His symptoms and facial palsy improved with treatment.

Temporal Lobe Retraction Provides Better Surgical Exposure of the Peri-Geniculate Ganglion for Facial Nerve Decompression via Transmastoid Approach

PURPOSE: For the exposure of the labyrinthine segment of the facial nerve, transmastoid approach is not usually considered due to being situated behind the superior semicircular canal. To obtain a better view and bigger field for manipulation in the peri-geniculate area during facial nerve decompression, retraction of temporal lobe after bony removal of tegmen mastoideum was designed via transmastoid approach. MATERIALS AND METHODS: Fifteen patients with traumatic facial paralysis [House-Brackmann (HB) grade IV–VI], 3 patients with Bell's palsy (HB grade V–VI), and 2 patients with herpes zoster oticus (HB grade V–VI) underwent facial nerve decompression surgery between January 2008 and July 2014. In all patients, we performed temporal lobe retraction for facial nerve decompression via the transmastoid approach. Patients were examined using pre operative tests including high-resolution computed tomography, temporal magnetic resonance imaging, audiometry, and electroneurography (degenerative ratio >90%). Facial function was evaluated by HB grading scale before and 6 months after the surgery. RESULTS: After the surgery, facial function recovered to HB grade I in 9 patients and to grade II in 11 patients. No problems due to surgical retraction of the temporal lobe were noted. Compared to the standard transmastoid approach, our method helped achieve a wider surgical view for improved manipulation in the peri-geniculate ganglion in all cases. CONCLUSION: Facial nerve decompression via the transmastoid approach with temporal lobe retraction provides better exposure to the key areas around the geniculate ganglion without complications.

Temporal Lobe Retraction Provides Better Surgical Exposure of the Peri-Geniculate Ganglion for Facial Nerve Decompression via Transmastoid Approach

PURPOSE: For the exposure of the labyrinthine segment of the facial nerve, transmastoid approach is not usually considered due to being situated behind the superior semicircular canal. To obtain a better view and bigger field for manipulation in the peri-geniculate area during facial nerve decompression, retraction of temporal lobe after bony removal of tegmen mastoideum was designed via transmastoid approach. MATERIALS AND METHODS: Fifteen patients with traumatic facial paralysis [House-Brackmann (HB) grade IV–VI], 3 patients with Bell's palsy (HB grade V–VI), and 2 patients with herpes zoster oticus (HB grade V–VI) underwent facial nerve decompression surgery between January 2008 and July 2014. In all patients, we performed temporal lobe retraction for facial nerve decompression via the transmastoid approach. Patients were examined using pre operative tests including high-resolution computed tomography, temporal magnetic resonance imaging, audiometry, and electroneurography (degenerative ratio >90%). Facial function was evaluated by HB grading scale before and 6 months after the surgery. RESULTS: After the surgery, facial function recovered to HB grade I in 9 patients and to grade II in 11 patients. No problems due to surgical retraction of the temporal lobe were noted. Compared to the standard transmastoid approach, our method helped achieve a wider surgical view for improved manipulation in the peri-geniculate ganglion in all cases. CONCLUSION: Facial nerve decompression via the transmastoid approach with temporal lobe retraction provides better exposure to the key areas around the geniculate ganglion without complications.

Quantitative Analysis of the Facial Nerve Using Contrast-Enhanced Three Dimensional FLAIR-VISTA Imaging in Pediatric Bell's Palsy

PURPOSE: To evaluate the usefulness of quantitative analysis of the facial nerve using contrast-enhanced three-dimensional (CE 3D) fluid-attenuated inversion recovery-volume isotopic turbo spin echo acquisition (FLAIR-VISTA) for the diagnosis of Bell's palsy in pediatric patients. MATERIALS AND METHODS: Twelve patients (24 nerves) with unilateral acute facial nerve palsy underwent MRI from March 2014 through March 2015. The unaffected sides were included as a control group. First, for quantitative analysis, the signal intensity (SI) and relative SI (RSI) for canalicular, labyrinthine, geniculate ganglion, tympanic, and mastoid segments of the facial nerve on CE 3D FLAIR images were measured using regions of interest (ROI). Second, CE 3D FLAIR and CE T1-SE images were analyzed to compare their diagnostic performance by visual assessment (VA). The sensitivity, specificity, and accuracy of RSI measurement and VA were compared. RESULTS: The absolute SI of canalicular and mastoid segments and the sum of the five mean SI (total SI) were higher in the palsy group than in the control group, but with no significant differences. The RSI of the canalicular segment and the total SI were significantly correlated with the symptomatic side (P = 0.028 and 0.015). In 11/12 (91.6%) patients, the RSI of total SI resulted in accurate detection of the affected side. The sensitivity, specificity, and accuracy for detecting Bell's palsy were higher with RSI measurement than with VA of CE 3D FLAIR images, while those with VA of CE T1-SE images were higher than those with VA of CE 3D FLAIR images. CONCLUSION: Quantitative analysis of the facial nerve using CE 3D FLAIR imaging can be useful for increasing the diagnostic performance in children with Bell's palsy when difficult to diagnose using VA alone. With regard to VA, the diagnostic performance of CE T1-SE imaging is superior to that of CE 3D FLAIR imaging in children. Further studies including larger populations are necessary.

A case of Ramsay Hunt syndrome diagnosed after kidney transplantation

We report the first case of Ramsay Hunt syndrome (RHS) diagnosed after kidney transplantation in Korea. RHS is a disease caused by latent varicella-zoster characterized to involve geniculate ganglion of the seventh cranial nerve. Patients who have undergone kidney transplantation can be easily affected by viral infections because of their immune-compromised status. A 35-year-old man with hypertensive end-stage renal disease underwent kidney transplantation. Two months after surgery, the recipient was diagnosed with RHS and treated with antivirals and steroids. However, after using the antiviral agents for the recommended duration, facial paralysis occurred as a new presentation and he required further treatment. Otalgia and periauricular vesicles improved, but the facial palsy remained.

A Case of Ramsay-Hunt Syndrome with Multiple Cranial Nerve Palsies Preceded by Diffuse Inflammatory Exudates in the Fluid Attenuated Inversion Recovery Image / 대한평형의학회지

Ramsay-Hunt syndrome (RHS) is a well known disease caused by varicella-zoster virus infection in the geniculate ganglion of the facial nerve. Although the otic vesicle and facial palsy are easily recognized clinical signs of RHS, cases of associated multiple cranial nerve palsies present a difficult diagnostic challenge and furthermore, the mechanisms is unclear. We report a case of an 86-year-old man with otic crusted vesicles and peripheral typed facial palsy preceded by severe headache and fever. Several days later, he developed diplopia, dysphagia, hiccup and abdominal myoclonus. On fluid attenuated inversion recovery image of brain, diffuse subdural inflammatory exudates, which disappeared after treatment of acyclovir and corticosteroid, and ipsilateral facial nerve enhancement were observed in follow-up imaging.

A Case of Herpes Zoster Oticus without Facial Nerve Palsy Associated with Vertigo and Hearing Loss / 대한평형의학회지

Herpes zoster oticus (HZO) is characterized by facial nerve palsy, otalgia and auricular vesicles on the affected side and accepted to be caused by the reactivation of varicella zoster virus (VZV) in the geniculate ganglion. Vestibulocochlear deficits are known to be frequently accompanied by HZO. Unusual clinical manifestations such as only vertigo without facial nerve palsy or hearing loss has been reported. We report a case of 27-year-old man presented with vertigo, sensorineural hearing loss and vesicular eruptions on the left auricle without facial nerve palsy. Serologic test revealed that the patient was positive for immunoglobulin G (IgG) and IgM antibodies against VZV.

Auriculotemporal and greater auricular nerve blocks have roles in patients with Ramsay Hunt syndrome with trigeminal nerve involvement: A report of two cases

Ramsay Hunt syndrome (RHS) refers to herpes zoster infection of the geniculate ganglion of the facial nerve. Cases complicated by multicranial nerve involvement in the process of reactivation of the virus, which are known to show virulent clinical course and worse prognosis, are not common in literature as in practice, and there has been only one reported case of suspected co-involvement of the trigeminal nerve in Korean literature. Therefore, in cases of RHS with severe rash over the face and neck, it is pertinent to give consideration to such multiple involvement in their early presentation. Facial nerve palsy and herpes related pain are the two worrisome complication, which could be alleviated by early treatment with neural blockade in addition to oral medication. Especially, nerve blocks are known to decrease the extent of nerve inflammation or damage, thereby facilitating recovery and probably preventing postherpetic neuralgia. We report two rare cases of Ramsay Hunt syndrome with trigeminal nerve involvement, where early implementation of blockade of somatic peripheral nerve branches, in addition to the conventional treatment, promoted early recovery.

Dysphagia in Ramsay Hunt's Syndrome: A Case Report

Ramsay-Hunt syndrome is caused by varicella zoster virus infection in the geniculate ganglion of the facial nerve. It is characterized by facial palsy, otic pain, and herpetic vesicles around the auricle and external auditory canal. Additionally, symptoms may develop related to other cranial nerve involvement, such as dizziness or hearing loss by the vestibulocochlear nerve being invaded. We report a rare case of a Ramsay-Hunt syndrome patient who developed dysphagia due to multiple cranial nerve involvement including the glossopharyngeal nerve and vagus nerve.

A Case of Dumbbell-Shaped Facial Nerve Schwannoma of the Internal Auditory Canal / 대한이비인후과학회지

Schwannoma is a benign neoplasm of the nerve sheath and is the most common neoplasm of the internal auditory canal (IAC) and cerebellopontine angle. A dumbbell-shaped schwannoma is defined as a mass with two bulbous segments, one in the IAC fundus and the other in the membranous labyrinth of the inner ear or the geniculate ganglion of the facial nerve connected to labyrinth segment. This kind of schwannoma should not be missed because it can affect the type of surgery and so the prognosis of the patient. We present one case of dumbbell-shaped facial nerve schwannoma which was completely excised without any complication.

Treatment of Ramsay Hunt Syndrome That is Mistaken for Trigeminal Herpes Zoster: A case report / 대한통증학회지

Ramsay Hunt syndrome is a disorder characterized by herpetic eruptions on the auricle, facial paralysis, and vestibulocochlear dysfunction, and is attributed to varicella zoster virus infection in the geniculate ganglion. Ramsay Hunt syndrome accounts for about 10% cases of facial palsy. We report a 46-year-old healthy man developed left side skin vesicles on the face with severe pain. We thought of the trigeminal herpes zoster. He was treated with intravenous acyclovir, and stellate ganglion block daily. Four days later, brain magnetic resonance imaging revealed small areas of enhancement in the seventh cranial nerve and eighth cranial nerve, not in the fifth cranial nerve. Eight days later, the left facial palsy was come. We confirmed him as Ramsay Hunt syndrome. We started steroid therapy immediately. He recovered completely a month later. The patient was improved through the early antiviral therapy, steroid medication and stellate ganglion block.

A Case of Herpes Zoster Oticus Involving Vestibular Nerve without Facial Nerve Palsy

Herpes zoster oticus (Ramsay Hunt syndrome) is characterized by facial nerve paralysis associated with vesticular eruptions and cochleovestibular symptoms. Many evidences have supported that it is caused by the reactivation of latent varicella-zoster virus in the geniculate ganglion. Recently we experienced a case 49-year-old man presented severe vertigo and a vesicular eruptions of auricle and external ear canal. It is an unusual variant of herpes zoster oticus that involves only vestibular nerve without facial nerve palsy and hearing loss. We believe this case results from reactivation of latent varicella zoster virus in the vestibular ganglion and report with a review of literatures.

Magnetic Resonance Imaging in Facial Nerve Palsy: Comparison between Bell's Palsy and Herpes Zoster Oticus / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Magnetic resonance imaging (MRI) is a valuable and important tool for use in diagnosing and investigating diseases affecting the facial nerve. However, there are few reports investigating the difference in the MRI findings of Bell's palsy and Ramsay Hunt syndrome. in order to evaluate the difference in the clinical values regarding these two groups of facial nerve palsy syndrome. In this study, we observed the MRI findings to investigate the value of MRI and its clinical significance in those two different groups of facial nerve palsy. SUBJECTS AND METHOD: Fourty-eight patients of Bell's palsy or herpes zoster oticus, who were admitted to Kangnam St. Mary's Hospital from January 1998 to December 2003, were selected to assess the results of gadolinium enhanced MRI. The frequency and the site of the facial nerve enhancement and its correlation with electroneuronography (ENoG), the time interval of the palsy to MRI, and initial ESR levels were observed in both groups of facial nerve palsy. RESULTS: On gadolinium enhanced MRI, 16 of 24 patients with Bell's palsy and 22 of 24 patients with herpes zoster oticus had contrast enhancement in the meatal, labyrinthine segments and geniculate ganglion. The number of enhanced segments was significantly larger in the patients with herpes zoster oticus than in the patients with Bell's palsy (p<0.05). There has been significant correlation between the number of enhanced segment of the facial nerve and the result of ENoG in the patients with herpes zoster oticus (p<0.05). CONCLUSION: Gadolinium enhanced MRI (Gd-MRI) studies may predict the extent of lesion within the course of the facial nerve and its clinical value as a prognostic factor could be suggested in patients with herpes zoster oticus

O papel do acesso via fossa média no tratamento da paralisia facial traumática / The role of the middle fossa approach in the management of traumatic facial paralysis

Há vários aspectos controversos no tratamento da paralisia facial traumática. Um destes é a natureza precisa da intervenção cirúrgica a ser usada, uma vez que a decisão de ser realizada tenha sido feita. FORMA DE ESTUDO: Clínico retrospectivo. OBJETIVOS E MÉTODOS: Entre o período de junho de 1984 e junho de 1993, 220 casos de paralisia facial traumática com boa função coclear foram tratados na Universidade de São Paulo pela seguinte técnica cirúrgica: descompressão dos segmentos mastóideo e timpânico através do acesso transmastoídeo e descompressão do gânglio geniculado e dos 50 por cento distais do segmento labiríntico, usando-se o acesso pela fossa média. Apresentamos uma revisão de literatura e a discussão e resultados de nosso trabalho.