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      Síndrome de Ramsay-Hunt Translated title: Ramsay-Hunt syndrome

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          Abstract

          El síndrome de Ramsay Hunt asocia parálisis facial periférica acompañada de una erupción vesicular en pabellón auricular o cavidad oral, causada por la afectación del ganglio geniculado por el virus varicela-zoster (VVZ). Supone la segunda causa más frecuente de parálisis facial periférica atraumática. Presentamos una revisión de 10 casos con síndrome de Ramsay Hunt que requirieron ingreso en nuestro hospital durante el periodo 2001-2005. Destacamos varios casos atípicos con afectación de otros pares craneales, un caso complicado con cerebelitis y mielopatía, así como la presencia de neumonía en 3/10, que sugiere un estado inmunocomprometido en estos pacientes. La respuesta al tratamiento con aciclovir y prednisona fue satisfactoria en la mayoría de los casos. La afectación facial en el zóster ótico es inicialmente más severa y con peor pronóstico de recuperación que la parálisis de Bell.

          Translated abstract

          Ramsay Hunt syndrome is a peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear or in the mouth; it is caused by varicella zoster virus that affects the geniculate ganglion. The zoster oticus is the second most common cause of atraumatic peripheral facial paralysis. We present a review of zoster oticus identified in our hospital among 2001-2005. we show various atypical cases with multiple cranial nerve involvement; cerebellum and spinal cord was affected in one patient. 3/10 cases were complicated with a pneumonia. So, we think that some grade of immunodeficiency may be present in these cases. Treatment with acyclovir and prednisone has been successfully to improve the outcome in the most of patients. Compared with Bell's palsy, patients with zoster oticus often have more severe paralysis at onset and are less likely to recover completely.

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          Most cited references8

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          Bell's palsy: the spontaneous course of 2,500 peripheral facial nerve palsies of different etiologies.

          The Copenhagen Facial Nerve Study aims to explain the spontaneous course of idiopathic peripheral facial nerve palsy which occurs without any kind of treatment. In this study Bell's palsy and idiopathic palsy are considered to be synonymous and specify an acute, monosymptomatic, unilateral peripheral facial paresis of unknown etiology. The material includes 2,570 cases of peripheral facial nerve palsy studied during a period of 25 years. It includes 1,701 cases of Bell's palsy and 869 of non-Bell's palsy. In the total patient sample, 116 had herpes zoster, 76 were diabetic, 46 were pregnant and 169 were neonates. A total of 38 different etiologies were observed. At the first consultation a standard ENT examination was performed, including a thorough description of the grade and localization of the paresis, taste, stapedius reflex and nasolacrimal reflex tests and acoustic-vestibular examination. Follow-up was done once a week during the first month and subsequently once a month until normal function was restored or for up to 1 year. The initial examination revealed 30% incomplete and 70% complete palsies. Follow-up showed that in 85% of patients function was returned within 3 weeks and in the remaining 15% after 3-5 months. In 71% of patients normal mimical function was obtained. Sequelae were slight in 12% of patients, mild in 13% and severe in 4%. Contracture and associated movements were found in 17% and 16% of patients, respectively. A survey of the literature showed that no kind of treatment, including prednisone, was able to give a better prognosis. The use of prednisone raises a big ethical problem because no evidence of its efficacy exists and the euphoric side-effect induces a false feeling of benefit in the patients.
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            Bell's Palsy and Herpes Zoster Oticus.

            Morrow (2000)
            Normal facial movement is required for chewing, swallowing, speaking, and protecting the eye. Bell's palsy causes most cases of acute, unilateral facial palsy; infection with herpes simplex virus (HSV) type 1 may be its major cause. Varicella zoster virus (VZV) reactivation (Ramsay Hunt syndrome) is less common, but may appear without skin lesions in a form indistinguishable from Bell's palsy. Symptoms improve in nearly all patients with Bell's palsy, and most patients with Ramsay Hunt syndrome, but many are left with functional and cosmetic deficits. Steroids are frequently used to optimize outcomes in Bell's palsy, but proof of their effectiveness is marginal. Oral prednisone has been studied extensively, although some reports have suggested a higher recovery rate with intravenous steroids. Given the existing data, we support the use of oral prednisone in those patients with complete facial palsy, and no contraindications to their use (Fig. 1). In this author's opinion, the greatly increased cost and inconvenience of intravenous steroids cannot be justified by the data available. Antiviral agents may also be effective in treatment of Bell's palsy; HSV is susceptible to acyclovir and related agents. There have been few investigations of acyclovir treatment in Bell's palsy, but one controlled study showed added benefit when the drug was used with prednisone. The risk and cost of acyclovir is low enough that we support its use, with oral steroids, in those patients with complete facial paralysis. Several small studies have implied that oral acyclovir improves the outcome of facial palsy for patients with Ramsay Hunt syndrome. Although these studies do not prove efficacy, evidence for the benefits of antiviral agents in other forms of zoster is strong enough to recommend their use when the facial nerve is involved. VZV is less sensitive to acyclovir than HSV, so higher doses are recommended to treat Ramsay Hunt syndrome. Because some Ramsay Hunt syndrome patients with partial facial palsy do not fully recover, we recommend oral antiviral agents in all patients suspected of having zoster. There is weak evidence to suggest additional benefit of oral steroids in facial zoster, and their use can be supported in immunocompetent individuals. Facial nerve decompression surgery for Bell's palsy and herpes zoster oticus has experienced varying levels of enthusiasm over the years. Recent work implies that early, extensive decompression of the nerve through a middle fossa craniotomy may benefit patients at high risk for persistent deficits. However, until this procedure is subjected to a rigorous, controlled trial comparing it with maximal medical therapy, it is difficult to justify the very high costs and risk.
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              Rapid diagnosis of varicella zoster virus infection in acute facial palsy

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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Journal
                ami
                Anales de Medicina Interna
                An. Med. Interna (Madrid)
                Arán Ediciones, S. L. (Madrid )
                0212-7199
                January 2007
                : 24
                : 1
                : 31-34
                Affiliations
                [1 ] Hospital General Obispo Polanco Spain
                [2 ] Hospital General Obispo Polanco Spain
                [3 ] Hospital General Obispo Polanco Spain
                Article
                S0212-71992007000100008
                10.4321/s0212-71992007000100008
                17373867
                1002475c-d798-4aeb-9114-d1c410faab65

                http://creativecommons.org/licenses/by/4.0/

                History
                Categories
                MEDICINE, GENERAL & INTERNAL

                Internal medicine
                Ramsay-Hunt syndrome,Zoster oticus,Facial paralysis,Varicella zoster virus,Síndrome de Ramsay Hunt,Zóster ótico,Parálisis facial,Virus varicela-zóster

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