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      Manifestações faringo-laríngeas da síndrome pós-poliomielite Translated title: Pharyngolaryngeal manifestations of post-polio syndrome

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          Abstract

          INTRODUÇÃO: A Síndrome pós-poliomielite (SPP) caracteriza-se por um novo episódio de atrofia ou fraqueza muscular em indivíduos acometidos por poliomielite prévia. Os sintomas iniciam-se após um período prolongado de estabilidade clínica que varia de 20 a 40 anos depois do quadro inicial da poliomielite. Dentre os mecanismos fisiopatológicos, o mais provável é que a síndrome represente um processo de desgaste e exaustão metabólica neuronal devido a um processo contínuo de desnervação-reinervação que se inicia após o quadro agudo inicial. OBJETIVO: Revisar as características clínicas e fisiopatológicas da SPP, assim como apresentar a abordagem em casos com manifestações faringo-laríngeas. RELATO DO CASO: Apresentamos o caso clínico de um paciente masculino com 48 anos de idade, com queixas principais disfonia e disfagia e história prévia de poliomielite. O paciente foi submetido à investigação diagnóstica, na qual encontram-se descritos os achados psicoacústicos da voz, laringoscópicos, eletromiográficos e da videoendoscopia da deglutição compatíveis com SPP. O tratamento consistiu em fonoterapia num total de 11 sessões semanais e orientações gerais para deglutição com melhora satisfatória dos sintomas. COMENTÁRIOS FINAIS: As manifestações faringo-laríngeas da SPP são passíveis de tratamento, obtendo-se resultados satisfatórios com melhora na qualidade de vida dos pacientes.

          Translated abstract

          INTRODUCTION: The post-polio syndrome (PPS) is characterized by a new episod of atrophy or muscle weakness in individuals previously affected by poliomyelitis. The symptoms start from an extended period of clinical stability ranging from 20 to 40 years, after the initial chart of polio. Among the pathophysiological mechanisms, the most likely is that the syndrome represents a process of attrition and neuronal metabolic exhaustion due to a continuous process of denervation reinnervation which begins after the initial acute. OBJECTIVE: Review the clinical characteristics and pathophysiological of PPS,as well as present the approach in cases with pharyngolaryngeal manifestations. CASE REPORT: We present the clinical case of a male patient with 48 years old, with main complaints of dysphonia, dysphagia and previous history of polio. The patient underwent a diagnostic investigation, in which the voice psychoacoustic findings are described. Laringoscopic, electromyographic and the videoendoscopy of swallowing compatible with PPS. The treatment consisted in phonotherapy in a total of 11 weekly sessions and guidelines for swallowing, with satisfactory improvement of the symptoms. FINAL COMMENTS: The pharyngolaryngeal manifestations of PPS are susceptible to treatment, obtaining satisfactory results, with improvement of life quality of the patients.

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          Poliomyelitis.

          1996 is polio awareness year. This paper reviews the clinical syndrome of acute paralytic poliomyelitis and its sequelae. We discuss epidemiological studies of the syndrome of late functional deterioration many years after the acute infection and the current hypotheses of the pathophysiology of such disorders. Recent evidence has suggested that potentially treatable factors may be implicated in the majority of such patients and it is therefore important to exclude such disorders before attributing late functional deterioration to progressive post-polio muscular atrophy.
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            Neurological manifestations of the post-polio syndrome.

            Patients with late effects of poliomyelitis, i.e., PPS, are being seen at an ever increasing frequency by general physicians, neurologists, and orthopedists. An appropriate time interval for the onset of late manifestations has elapsed since the major epidemics of poliomyelitis in the 1940s and 1950s. Post-polio neurological manifestations primarily include new weakness, atrophy, muscle pain, and fasciculations. Fortunately, the weakness is of a very slow, progressive nature. Abnormal laboratory studies include routine EMG, demonstrating chronic denervation; SFEMG, demonstrating increased fiber density, increased jitter, and blocking; and muscle biopsy most often revealing fiber-type grouping of chronic denervation and small isolated angular (or angulated) fibers and group atrophy in some series, both suggestive of active denervation. Unfortunately, both EMG and muscle biopsy studies suffer from a lack of specificity as they do not appear to distinguish asymptomatic from symptomatic (new weakness, PPMA) patients with prior poliomyelitis. Although the cause of PPMA is unknown, electrophysiological (SFEMG) and muscle biopsy studies suggest that the process involves a loss or dropout of axon terminals of reinnervated motor units. The axons terminal dropout could be due to dysfunction in the cell soma, the axon, or the terminals themselves. Whether motor neuron exhaustion, a persistent viral infection, or immune-mediated mechanisms play a role in the pathogenesis of the late weakness is unclear at present and will require further investigation. Treatment at this time is of a supportive nature. A major controversy involves the role of strengthening exercises in these patients since experimental animal studies suggest that excessive exercise of denervated muscles leads to increased weakness. Clearly, a better understanding of PPS and PPMA will allow more effective management of these patients' problems and might also provide insight into other motor neuron and neuromuscular junction diseases.
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              Molecular aspects of poliovirus biology with a special focus on the interactions with nerve cells.

              Poliovirus (PV), the pathogenic agent of paralytic poliomyelitis, is the prototype of the picornavirus family. Although paralytic poliomyelitis has been nearly totally eradicated in most industrialized countries, PV continues to be an important public health problem in many developing countries. Moreover, in industrialized countries, two current concerns are the occurrence, albeit at a very low frequency, of vaccine-associated paralytic poliomyelitis, due to the genetic instability of the attenuated oral PV strains in vaccines, and the emergence of a neuro-muscular pathology in many survivors of the acute disease, called the post-polio syndrome. PV has been targeted by the World Health Organization for world-wide eradication in the coming decade and continues to be the subject of intensive research. The advances made in the molecular biology of PV, taken together with the development of new animal and cell models, have permitted a new look at a key step in the pathogenesis of poliomyelitis, i.e. the interactions between PV and nerve cells. These aspects of PV biology are developed in this review according to three themes: (i) the PV host range; (ii) the molecular determinants of PV neurovirulence and attenuation; and (iii) the persistence of PV in nerve cells, which has proven to be an interesting new domain in the field of PV research.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
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                Journal
                aio
                Arquivos Internacionais de Otorrinolaringologia
                Arquivos Int. Otorrinolaringol.
                Fundação Otorrinolaringologia (São Paulo )
                1809-4856
                December 2011
                : 15
                : 4
                : 529-533
                Affiliations
                [1 ] Pontifícia Universidade Católica
                Article
                S1809-48722011000400020
                10.1590/S1809-48722011000400020
                f4e07988-bef8-4071-9963-a61ba2c8e2c9

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

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                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=1809-4872&lng=en
                Categories
                OTORHINOLARYNGOLOGY

                Otolaryngology
                poliomyelitis,post-polio syndrome,neuromuscular diseases,larynx diseases,voice disorders,poliomielite,síndrome pós-poliomielite,doenças neuromusculares,doenças da laringe,distúrbios da voz

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