Continuous hemicrania as the initial manifestation of Ramsay Hunt syndrome: a case report / Hemicrania contínua como manifestação inicial da síndrome de Ramsay Hunt: relato de caso

Introduction:Ramsay-Hunt syndrome, also called otic zoster, is a rare complication of herpes zoster. The syndrome is characterized by peripheral facial nerve palsy and an erythematous vesicular eruption in the ear or mouth. Preceding the appearance of the vesicles, unilateral otalgia or neck pain may occur more commonly. However, persistent hemicrania is infrequent in the preeruptive phase. Objective:To present an atypical case of Ramsay Hunt syndrome with continuous unilateral headache preceding the onset of other symptoms and signs of the syndrome. Case report:Report of a 69-year-old woman who presented subacute onset of moderate to severe left hemicrania with no autonomic signs. Eight days after the start and continuous headache maintenance, she presented with peripheral facial paralysis. After four days, she noticed the presence of vesicles in the left ear and odynophagia. She developed nausea with several episodes of vomiting and severe imbalance that made it impossible for her to walk unassisted. On physical examination, she presented vesicles in the left ear and oropharynx, left peripheral facial palsy (House Brackmann grade IV), left hypoacusis, nystagmus, and vestibular gait. Diagnostic tests for screening several metabolic diseases and diagnosis of infection (including HIV) were unremarkable. Brain computed tomography and cerebrospinal fluid analysis showed no abnormalities. Conclusion:Ramsay-Hunt syndrome mainly involves the facial and vestibulocochlear nerves, causing peripheral facial palsy, otalgia, hypoacusis, and, less frequently, imbalance. Although pain is a frequent manifestation of the preeruptive phase of RHS, unilateral headache is not common in this scenario. On the other hand, it is a prevalent complaint in the emergency department and has several different etiologies. Hence, diagnosing RHS when patients present exclusively unilateral headaches is challenging for clinicians. Physicians must consider RHS a vital differential diagnosis of sided-locked headaches, avoiding diagnostic errors and treatment delays.

Introdução: A síndrome de Ramsay-Hunt, também chamada de ótico-zóster, é uma complicação rara do herpes-zóster. A síndrome é caracterizada por paralisia do nervo facial periférico e erupção vesicular eritematosa no ouvido ou na boca. Precedendo o aparecimento das vesículas, pode ocorrer mais comumente otalgia unilateral ou dor cervical. No entanto, a hemicrania persistente é pouco frequente na fase pré-eruptiva. Objetivo: Apresentar um caso atípico de síndrome de Ramsay Hunt com cefaleia unilateral contínua precedendo o aparecimento de outros sintomas e sinais da síndrome. Relato de caso: Relato de uma mulher de 69 anos que apresentou hemicrania esquerda de início subagudo, moderada a grave, sem sinais autonômicos. Oito dias após o início e manutenção contínua da cefaleia, apresentou paralisia facial periférica. Após quatro dias, notou presença de vesículas em orelha esquerda e odinofagia. Ela desenvolveu náuseas com vários episódios de vômitos e desequilíbrio grave que a impossibilitou de andar sem ajuda. Ao exame físico apresentava vesículas em orelha esquerda e orofaringe, paralisia facial periférica esquerda (grau IV de House Brackmann), hipoacusia esquerda, nistagmo e marcha vestibular. Os testes de diagnóstico para rastreio de diversas doenças metabólicas e diagnóstico de infeções (incluindo VIH) não apresentaram resultados dignos de nota. A tomografia computadorizada de cérebro e a análise do líquido cefalorraquidiano não mostraram alterações. Conclusão: A síndrome de Ramsay-Hunt envolve principalmente os nervos facial e vestibulococlear, causando paralisia facial periférica, otalgia, hipoacusia e, menos frequentemente, desequilíbrio. Embora a dor seja uma manifestação frequente da fase pré-eruptiva da SHI, a cefaleia unilateral não é comum neste cenário. Por outro lado, é uma queixa prevalente no serviço de urgência e tem diversas etiologias. Portanto, diagnosticar SHI quando os pacientes apresentam dores de cabeça exclusivamente unilaterais é um desafio para os médicos. Os médicos devem considerar a SHI um diagnóstico diferencial vital de cefaleias laterais, evitando erros de diagnóstico e atrasos no tratamento.

Síndrome de Ramsay Hunt: revisión narrativa / Ramsay Hunt Syndrome: Narrative Review

El síndrome de Ramsay Hunt (SRH) corresponde a la asociación de la parálisis facial periférica con una erupción vesicular localizada en el pabellón auricular, causada por el compromiso del ganglio geniculado secundario a una infección por el virus de la varicela-zóster (VVZ). Este síndrome es la segunda causa más común de parálisis facial atraumática y representa aproximadamente el 10 %-12 % de las parálisis faciales agudas, con una incidencia anual de 5 por cada 100 000 habitantes en Estados Unidos. El diagnóstico es principalmente clínico y entre las manifestaciones más destacadas se encuentran síntomas neurológicos como otalgia, tinnitus, hipoacusia asociada con parálisis facial junto a lesiones herpéticas características. Dentro de las complicaciones que se pueden presentar en esta entidad se encuentra, principalmente, la neuralgia posherpética, seguida de otras menos frecuentes como la encefalitis, el herpes zóster oftálmico y la mielitis. El manejo actual del SRH se basa en la aplicación de terapias duales con corticosteroides asociados a terapia antiviral, lo cual ha demostrado que el inicio temprano del tratamiento mejora el pronóstico y disminuye la aparición de complicaciones. El pronóstico de esta patología es inferior en comparación a patologías menos severas que comprometen el nervio facial (como la parálisis de Bell) y se ve impactado por varios factores como el inicio oportuno de tratamiento, el grupo etario y la presencia de comorbilidades.

Ramsay Hunt syndrome corresponds to the association of peripheral facial paralysis with a vesicular eruption located in the pinna, caused by the involvement of the geniculate ganglion secondary to infection by the varicella zoster virus. This syndrome is the second causes of atraumatic facial paralysis, representing approximately 10 %-12 % of acute facial paralysis, with an annual incidence of 5 per 100,000 inhabitants. The diagnosis is mainly clinical and among the most prominent manifestations are neurological symptoms such as otalgia, tinnitus, hypoacusis associated with facial paralysis together with characteristic herpetic lesions. Among the complications that may occur in this entity is mainly postherpetic neuralgia, followed by less frequent ones such as encephalitis, ophthalmic herpes zoster and myelitis. Current management of Ramsay Hunt syndrome is based on the application of dual therapies consisting of corticosteroids associated with antiviral therapy, showing that early initiation of treatment improves prognosis and reduces the appearance of complications. The prognosis of this pathology is inferior compared to less severe pathologies that compromise the facial nerve (Bell's palsy) and is impacted by several factors such as the timely initiation of treatment, the age group, and the presence of comorbidities.

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.

Facial paralysis due to Ramsay Hunt syndrome - A rare condition / Paralisia facial secundária à síndrome de Ramsay Hunt - Uma condição rara

Summary Ramsay Hunt syndrome (or herpes zoster oticus) is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion occurs. Usually, there are auricular vesicles and symptoms and signs such otalgia and peripheral facial paralysis. In addition, rarely, a rash around the mouth can be seen. Immunodeficient patients are more susceptible to this condition. Diagnosis is essentially based on symptoms. We report the case of a diabetic female patient who sought the emergency department with a complaint of this rare entity.

Resumo A síndrome de Ramsay Hunt (ou zóster auricular) é uma complicação rara do herpes-zóster em que ocorre reativação de uma infecção latente pelo vírus varicela-zóster no gânglio geniculado. Geralmente, estão presentes vesículas auriculares e sintomas como otalgia e paralisia facial periférica. Além disso, mais raramente pode haver rash ao redor da boca. Pacientes com imunodeficiência apresentam maior susceptibilidade para essa condição. O diagnóstico é essencialmente pelo quadro clínico. É apresentado o caso de uma paciente diabética que compareceu ao setor de emergência com essa manifestação rara.

Cranial Polyneuropathy in Ramsay Hunt Syndrome Manifesting Severe Pharyngeal Dysphagia: a Case Report and Literature Review

Ramsay Hunt syndrome (RHS) is characterized by herpes zoster oticus, facial nerve palsy, and vestibulocochlear symptoms. Dysphagia caused by the involvement of multiple cranial nerves (CNs) in RHS is very rare. We encountered a rare case presented with severe dysphagia due to cranial polyneuropathy involving trigeminal nerve (CN V), facial nerve (CN VII), vestibulocochlear nerve (CN VIII), glossopharyngeal nerve (CN IX), vagus nerve (CN X), and hypoglossal nerve (CN XII) in RHS. This case report suggested that the prognosis for dysphagia in RHS seems favorable, and swallowing rehabilitation therapy could be beneficial in expediting tube removal.

Ramsay Hunt Syndrome in a Living-donor Kidney Transplantation Recipient: Unusual Clinical Course Case / 대한이식학회지

Varicella zoster virus (VZV) infection is due to VZV reactivation in most cases. The infection rate ranges from 4% to 12% in renal allograft recipients. Ramsay Hunt syndrome (RHS) is a rare manifestation of VZV infection. RHS typically presents as severe ear pain, small vesicles, and facial palsy. We reported a case of a 60-year-old man with an unusual clinical course who underwent living donor renal transplantation. He complained of severe ear pain but did not show vesicles or facial palsy. He also presented lesions indicating a fungal infection. Diagnosis of RHS was delayed since facial palsy did not develop until some days later. Although the denervation rate was high, he showed recovery of nearly all symptoms after antiviral treatment. Solid organ recipients may not typically show presentation of viral infection, and therefore clinical suspicion is important. Even though the final diagnosis is delayed, we must treat patients since they may recover well in contrast with the average population.

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.

A Case of Herpes Zoster Oticus Involving Multiple Cranial Nerves without Facial Palsy / 대한이비인후과학회지

Varicella zoster virus (VZV) infection of the head and neck region may present with various symptoms. We present a case of VZV infection of the external ear (cranial nerve V), pharynx and larynx with neuropathy of three ipsilateral lower cranial nerves (IX, X, XII). The patient's paresthesia was reduced after antiviral therapy and oral steroids. By the end of one month follow-up, the patient showed normal movement of vocal cord, soft palate and tongue.

Herpes Zoster Oticus / 대한이비인후과학회지

Herpes zoster oticus, traditionally known as Ramsay Hunt syndrome, is nowadays described also as acute peripheral facial neuropathy by varicella zoster virus infection. Besides facial palsy, auricular vesicular eruptions accompanied by neuralgic pain, adjacent cranial nerve structures are involved and various audiovestibular symptoms are frequently combined. Early proper treatment strategies are required to get the favorable outcome and reduce the morbidity of neurocranial deficits of this syndrome.

Effects of integrative medicine treatment of ear herpes zoster / 中国基层医药

Objective To observe the clinical effect of western medicine combined with traditional reatme medicine in the treatment of ear herpes zoster.Methods 60 herpes zoster oticus patients were selected,randomly divided into observation group and control group,30 cases in each group.The control group received conventional western medicine treatment,while the observation group treated with integrated traditional Chinese and western medicine treatment.Two groups of clinical curative effects,and the average healing time of clinical symptoms were observed.Results The total effective rate in observation group was 93.3 %,which was 73.3 % in the control group,the difference was statistically significant(x2 =4.32,P ＜0.05),the clinical symptoms of two groups of cure time average of the differences were statistically significant (t =5.24,7.72,7.70,6.21,all P ＜ 0.05).Conclusion The western medicine combined with traditional reatme medicine in the treatment of ear herpes zoster has better effect than that of western medicine.

Comparison of Transcranial Magnetic Stimulation and Electroneuronography Between Bell's Palsy and Ramsay Hunt Syndrome in Their Acute Stages

OBJECTIVE: To examine the neurophysiologic status in patients with idiopathic facial nerve palsy (Bell's palsy) and Ramsay Hunt syndrome (herpes zoster oticus) within 7 days from onset of symptoms, by comparing the amplitude of compound muscle action potentials (CMAP) of facial muscles in electroneuronography (ENoG) and transcranial magnetic stimulation (TMS). METHODS: The facial nerve conduction study using ENoG and TMS was performed in 42 patients with Bell's palsy and 14 patients with Ramsay Hunt syndrome within 7 days from onset of symptoms. Denervation ratio was calculated as CMAP amplitude evoked by ENoG or TMS on the affected side as percentage of the amplitudes on the healthy side. The severity of the facial palsy was graded according to House-Brackmann facial grading scale (H-B FGS). RESULTS: In all subjects, the denervation ratio in TMS (71.53+/-18.38%) was significantly greater than the denervation ratio in ENoG (41.95+/-21.59%). The difference of denervation ratio between ENoG and TMS was significantly smaller in patients with Ramsay Hunt syndrome than in patients with Bell's palsy. The denervation ratio of ENoG or TMS did not correlated significantly with the H-B FGS. CONCLUSION: In the electrophysiologic study for evaluation in patients with facial palsy within 7 days from onset of symptoms, ENoG and TMS are useful in gaining additional information about the neurophysiologic status of the facial nerve and may help to evaluate prognosis and set management plan.

A Case of Herpes Zoster Oticus with Intractable Hiccups / 대한이비인후과학회지

Herpes zoster oticus is a common inflammatory disease caused by reactivation of varicella zoster virus in the dorsal root ganglia. Its symtpoms include unilateral distribution of the vesicular eruptions, neuralgia and various type of cranial nerve palsy depending on the involving site of head and neck region. Most commonly affected cranial nerves are facial nerve (VII) and vestibulocochelar nerve (VIII), but on rare occasions cranial nerves V, IX, X, XI are affected as well. Although neurologic symptom of herpes zoster was commonly reported, zoster associated hiccups is very rare. We experienced one case of herpes zoster oticus involving multiple cranial nerve palsy with intractable hiccups. We report our case with a review of literature.

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.

A Case of Herpes Zoster Oticus Involving Glossopharyngeal Nerve without Facial Nerve Palsy / 대한피부과학회지

No abstract available.

Acute Inflammatory Facial Nerve Paralysis / 대한이비인후과학회지

Bell's palsy is the most common form of acute facial nerve disorder, which presents as an acute peripheral unilateral facial palsy of unknown cause. Ramsay-Hunt syndrome is the second most common cause of acute facial palsy, and is known to be caused by reactivation of latent varicella zoster virus. The main goal of treatment for acute inflammatory facial nerve paralysis is to speed up recovery, to facilitate the recovery more completely and to prevent other sequelae. However, some patients may have a poor recovery with permanent, disfiguring facial asymmetry despite of many kinds of treatments. Regarding the diagnostic and therapeutic issues of the two common disorders, there still exist some controversies. This article reviewed recent evidences on several important issues in evaluation and management of acute inflammatory facial nerve paralysis, and intended to provide an evidence-based framework for decision-making in the clinic.

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.

Herpes Zoster Oticus with Cranial Polyneuropathy without Involvement of Facial Nerve

Herpes zoster oticus is caused by herpetic viruses including varicella zoster and most commonly affects cranial nerves (CN) VII and VIII. With a review of literature, we report a case of herpes zoster oticus with selective involvement of CN VIII, IX and X. Interestingly, the motor fibers of CN VII were spared while ipsilateral recurrent laryngeal nerve involvement was evident. The patient was treated with antiviral medication and systemic steroids and symptoms were improved.

Delayed Facial Paralysis After Middle Ear and Mastoid Surgery due to Reactivation of Herpes-Zoster Virus / 대한이비인후과학회지

Herpes viral reactivation is thought to be an important etiologic factor in the development of post-operative delayed onset of facial paralysis. The authors present a case of herpes zoster oticus that occurred as a consequence of middle ear and mastoid surgery. The patient was initially misdiagnosed as iatrogenic facial paralysis and treated with oral steroid only. The final diagnosis of herpes zoster oticus was made by the development of herpetic auricular eruption with severe otalgia and the results of positive serological tests. Clinical features, diagnosis and management of this rare case are presented and discussed.

Bilateral Vestibular Hypofunction Induced by Unilateral Herpes Zoster Oticus

Herpes zoster oticus usually accompanies vestibulopathy on the ipsilateral ear. However we have encountered two herpes zoster oticus patients with bilateral vestibulopathies. Bilateral vestibulopathy was detected on the 2nd month and 19th day of herpes zoster oticus, respectively. While the contralateral vestibulopathy was detected 2 month after the ipsilateral vestibulopathy in the first patient, the vestibular function deteriorated simultaneously on the 19th day in the second patient. It seems that the bilateral vestibulopathy was caused by an autoimmune process in both cases, but the initiating event is different. The ipsilateral vestibular damage may have sensitized the immune system in the first patient resulting in sympathetic vestibulopathy. But in the second patient, the ipsilateral cochlear damage may have sensitized the immune system resulting in simultaneous bilateral vestibulopathy.