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.

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.

Estudio de líquido cerebroespinal en el síndrome de Ramsay-Hunt: controversia a raíz de un caso clínico / Study of cerebrospinal fluid in Ramsay-Hunt syndrome: controversy because of a clinical case

El Síndrome de Ramsay-Hunt (SRH), es la segunda causa de parálisis facial periférica (PFP). Causado por el virus Varicella zoster (VVZ), ipsilateral a la PFP,presenta unaerupción herpetiforme y cefalea en distribución del nervio facial. Presentamos el caso de una mujer, 54 años, con SRH y cefalea persistente cuyo líquido cerebroespinal (LCE) fue compatible con meningitis. Se indicó aciclovir endovenoso (EV). La literatura no recomienda estudio de LCE en PFP; y en SRH se sugiere en inmunosuprimidos y complicaciones del SRH como queratopatía, accidentes-cerebrovasculares, y meningitis. Un LCE alterado en SRH, indica modificar la conducta terapéutica.

Ramsay-Hunt Syndrome (RHS) is the second leading cause of peripheral facial palsy (PFP). Caused by the Varicella zoster virus (VZV), ipsilateral to the PFP, it presents a herpetiform rash and headache in the facial nerve distribution. We present the case of a 54-year-old woman with RHS and persistent headache whose cerebrospinal fluid (CSF) was compatible with meningitis. Intravenous acyclovir was indicated. The literature does not recommend an CSF study in PFP; in RHS it is suggested in immunosuppressed patients and complications of RHS such as keratopathy, cerebrovascular accidents, and meningitis. An altered CSF in RHS indicates modifying the therapeutic conduct.

Ramsey hunt syndrome after antimonial treatment for American Cutaneous Leishmaniasis

Abstract Ramsay Hunt Syndrome (RHS), also known as herpes zoster oticus, is caused by the reactivation of varicella zoster virus (VZV) in the geniculate ganglion of the facial nerve. Herein, we report a case of Ramsey Hunt Syndrome in a patient after antimonial treatment for Cutaneous Leishmaniasis. The patient presented with microvesicles grouped on an erythematous base, starting in the neck and ascending towards the scalp margin on the right side of the head. The patient also developed grade V peripheral facial palsy the day after initiating the herpes zoster treatment, this outcome corroborated the assumption of Ramsey Hunt Syndrome.

Síndrome de Ramsay Hunt. Informe de un caso y revisión de la literatura / Ramsay Hunt syndrome. Case report and literature review

RESUMEN Se presentó paciente masculino de 68 años de edad, con antecedentes de síndrome de Parkinson posinfarto cerebral isquémico antiguo e hipertensión arterial sistémica, con la intensión de describir la experiencia del diagnóstico y tratamiento del síndrome de Ramsay Hunt. El paciente acudió a consulta de Medicina Interna del Policlínico Universitario "Omar Ranedo Pubillones", Guantánamo, por presentar dolor en hemicara izquierda y otalgia homolateral, cefalea y febrícula. Luego, presentó lesiones vesiculosas de aspecto herpético. Se concluyó el diagnóstico definitivo de síndrome de Ramsay Hunt tipo II y se remitió a consulta de Otorrinolaringología, donde se confirmó el diagnóstico. Luego del tratamiento evolucionó satisfactoriamente. Aunque el síndrome de Ramsay Hunt no es un problema de salud, se revela la necesidad de que el médico del nivel primario de atención a la salud se familiarice con las bases teóricas para su diagnóstico y se connota la importancia de la evaluación interdisciplinaria para esta finalidad.

ABSTRACT It was reported a 68 year old male patient with a personal pathological history of post- ischemic stroke Parkinson's syndrome and systemic arterial hypertension and with the intention of describing the experience of diagnosis and treatment of Ramsay Hunt syndrome. Patient presented to his primary health physician at the Univirsity Clinic "Omar Ranedo Pubillones" in Guantanamo, presenting pain in right side of the face and earache, headache and fever. Then, he presented vesicular lesions with a herpetic appearance. It was concluded the definitive diagnosis of Ramsay Hunt syndrome type II and it was referred to Otolaryngology consultation, where the diagnosis was confirmed. After treatment patient's improvement was good. Although Ramsay Hunt syndrome is not a health problem, shows the need for primary care physicians to become familiar with the theoretical basis for its diagnosis and the importance of interdisciplinary evaluation for this purpose.

Facial Herpes Zoster: A 15-year Retrospective Single-Center Study / 대한피부과학회지

BACKGROUND: Many studies about herpes zoster exist in the Korean dermatologic literature. However, few studies have reported about facial herpes zoster and its complications. OBJECTIVE: The purpose of this study was to investigate the epidemiology, clinical features, and complications of facial herpes zoster. METHODS: We analyzed the medical records of 977 patients who visited Sanggye Paik Hospital between January 2002 and December 2017. We categorized patients into 8 groups based on the dermatomal distribution of herpetic lesions. We investigated the correlations between clinical features, prognosis, and complications. RESULTS: We analyzed the medical records of 977 patients who visited Sanggye Paik Hospital between January 2002 and December 2017. We categorized patients into 8 groups based on the dermatomal distribution of herpetic lesions. We investigated the correlations between clinical features, prognosis, and complications. Results: The most common site of involvement of facial herpes zoster was the ophthalmic branch of the trigeminal nerve (59.2%). Herpes zoster involving the ophthalmic and the maxillary branches of the trigeminal nerves showed a significantly higher incidence rate of herpes zoster ophthalmicus than that observed with herpes zoster involving only the ophthalmic branch (odds ratio 7.373). Age and periorbital swelling were significantly correlated with herpes zoster ophthalmicus (p<0.001). Facial palsy was significantly correlated with otalgia and cutaneous ear involvement (p<0.001). Postherpetic neuralgia (PHN) occurred in 41% of patients with facial herpes zoster. Patients who received antiviral treatment within 3 days showed lower rates of PHN (35.8%) than patients who received treatment after 4 days (45.4%) (p=0.002). CONCLUSION: Facial herpes zoster is associated with several neurological complications. Variables including age, periorbital swelling, otalgia, and cutaneous ear involvement are useful predictors of complications and prognosis. Early institution of antiviral treatment and appropriate interdepartmental consultations are required for better treatment outcomes.

Ramsay Hunt Syndrome Complicated by Meningoencephalitis and Radiologic findings: a Rare Case Report

Ramsay Hunt syndrome with the complication of encephalitis or meningoencephalitis is rarely reported and uncommon in immunocompetent patients. The radiological manifestations of such cases usually involve the cerebellum and brainstem or exhibit the absence of any abnormality. We report a case of a 78-year-old immunocompetent man hospitalized with Ramsay Hunt syndrome, who later developed meningoencephalitis. The cerebrospinal fluid-study excluded other causes of meningoencephalitis, and the clinical diagnosis indicated varicella zoster virus meningoencephalitis. Magnetic resonance imaging revealed increased signal intensities in the bilateral temporal lobe, midbrain, and pons on T2-weighted imaging, and T2 fluid attenuated inversion recovery and contralateral asymmetric pachymeningeal enhancement. Contrast-enhanced T1-weighted imaging revealed ipsilateral facial nerve enhancement.

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.

Clinical Characteristics of Dizziness Associated with Acute Peripheral Facial Palsy

BACKGROUND AND OBJECTIVES: We aimed to assess the clinical significance of dizziness associated with acute peripheral facial palsy (APFP). SUBJECTS AND METHODS: Medical records of patients who visited an otorhinolaryngology clinic at a university hospital and were admitted for treatment of APFP between 2014 and 2016 were thoroughly reviewed. RESULTS: In total, 15.3% (n=15) of patients had dizziness. Continuous, rotatory dizziness without exacerbating factors was most common and frequently accompanied by nausea/vomiting. Dizziness disappeared within 1 week during the hospitalization period. Patients with Ramsay Hunt syndrome (31.0%) had dizziness more frequently than those with Bell’s palsy (8.7%). In addition, higher hearing thresholds and pain around the ear was reported more often in dizzy patients (p < 0.05). Logistic regression analysis revealed that the initial House-Brackmann grade of facial paralysis was solely associated with final recovery, but dizziness was not associated with prognosis. CONCLUSIONS: Patients with APFP may have transient dizziness in the early stage, which may be more frequently accompanied by worse hearing thresholds and/or pain around the ear. However, these symptoms including dizziness seem to be unrelated to final prognosis.

Abducens Nerve Palsy Associated with Ramsay-Hunt Syndrome / 대한평형의학회지

Ramsay-Hunt syndrome is an infectious disease caused by the varicella zoster virus. It is usually associated with facial and vestibulocochlear nerve palsy, but other cranial nerve dysfunction can be accompanied. We present a 68-year-old woman with abducens nerve palsy associated with Ramsay-Hunt syndrome. She showed abduction limitation of left eye with peripheral facial palsy and vestibulopathy of the left side. Varicella zoster virus polymerase chain reaction of cerebrospinal fluid was positive and internal auditory canal magnetic resonance imaging was revealed enhancement of labyrinthine segment of left facial nerve. Although abducens nerve palsy is uncommon feature of Ramsay-Hunt syndrome, but it can be developed by several different mechanisms.

Ramsay Hunt syndrome presenting with abducens nerve palsy followed by V, VII, VIII, IX, and X nerve palsies

@#Ramsay Hunt syndrome, associated with varicella zoster virus infection is characterized by herpes zoster oticus, facial nerve palsy, and cochleovestibular symptoms. Ramsay Hunt syndrome associated cranial polyneuropathy occasionally occurs with involvement beyond VII and VIII. We represent a patient of Ramsay Hunt syndrome who presented with only VI involvement at the first visit followed by cranial polyneuropathy. Varicella zoster virus infection was confirmed by the detection of varicella zoster virus-DNA in cerebrospinal fluid.

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.

Treatment and Prognosis of Facial Palsy on Ramsay Hunt Syndrome: Results Based on a Review of the Literature

Abstract Introduction Ramsay Hunt syndrome is the second most common cause of facial palsy. Early and correct treatment should be performed to avoid complications, such as permanent facial nerve dysfunction. Objective The objective of this study is to review the prognosis of the facial palsy on Ramsay Hunt syndrome, considering the different treatments proposed in the literature. Data Synthesis We read the abstract of 78 studies; we selected 31 studies and read them in full. We selected 19 studies for appraisal. Among the 882 selected patients, 621 (70.4%) achieved a House-Brackmann score of I or II; 68% of the patients treated only with steroids achieved HB I or II, versus 70.5% when treated with steroids plus antiviral agents. Among patients with complete facial palsy (grades V or VI), 51.4% recovered to grades I or II. The rate of complete recovery varied considering the steroid associated with acyclovir: 81.3% for methylprednisolone, 69.2% for prednisone; 61.4% for prednisolone; and 76.3% for hydrocortisone. Conclusions Patients with Ramsay-hunt syndrome, when early diagnosed and treated, achieve high rates of complete recovery. The association of steroids and acyclovir is better than steroids used in monotherapy.

Acute Vestibular Neuritis and Ramsay-Hunt Syndrome / 대한평형의학회지

Acute vestibular neuritis is the disorder characterized by acute, spontaneous vertigo with the unilateral vestibular loss. Reactivation of herpes simplex virus is considered as its cause. Its management consists of symptomatic therapy in the acute phase and following rehabilitation exercise to improve central compensation. The differential diagnosis should include central vestibular disorders mimicking peripheral vertigo. Ramsay-Hunt syndrome, which defined as a herpes zoster oticus with facial paresis, is also a disorder frequently accompanied with vestibular deficit. Combination therapy of acyclovir and corticosteroid is recommended for the treatment. In this review, diagnosis and management of the two disorders are described.

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.

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.

Vagus Nerve Palsy in Ramsay-Hunt Syndrome / 대한임상신경생리학회지

No abstract available.

Disseminated Herpes Zoster in an Immunocompetent Elderly Patient

Herpes zoster is a cutaneous infection that is characterized by an acute vesicobullous rash with ipsilateral one or two dermatomal distribution and painful allodynia, while predominantly being found in the elderly. Extensive cutaneous dissemination has been reported in immune-compromised patients, such as those who suffer from HIV infections, cancer, chemotherapy, and corticosteroid therapy patients. However, we report a case of disseminated herpes zoster infection in an immuno-competent elderly individual.

A Case of Ramsay Hunt Syndrome with Multicranial Nerve Palsy / 대한임상신경생리학회지

No abstract available.

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.