Disfagia orofaríngea na distrofia miotônica: avaliação fonoaudiológica e análise nasofibrolaringoscópica

Abstract

RESUMO -Apresentamos vinte pacientes com distrofia miotônica de Steinert, avaliados entre 1995 e 1999, pela análise fonoaudiológica e nasofibrolaringoscópica, com o objetivo de analisar e classificar as alterações da deglutição orofaríngea e funções do sistema estomatognático e considerar os fatores preditivos. A idade dos pacientes variou de 12 a 53 anos; 13 eram do sexo masculino e 7 do feminino. Os principais achados foram: (1) há relação estatisticamente significante entre avaliação fonoaudiológica e nasofibrolaringoscópica; (2) foram observadas alterações do sistema estomatognático, em 100% dos casos; (3) foram registradas alterações de deglutição em 95% dos pacientes, à avaliação fonoaudiológica, e em 70%, à nasofibrolaringoscopia; (4) os pacientes têm maior dificuldade para deglutir alimentos consistentes; (5) os músculos estomatognáticos muito alterados, desordem da fase faringeal, tosse após deglutição, antecedentes de pneumonia e queixas de mastigação/ deglutição apresentaram correlação estatisticamente significante com maior gravidade da doença. Foram avaliadas, estática e funcionalmente, estruturas envolvidas na dinâmica da deglutição. Esta avaliação deve constar da rotina do atendimento aos pacientes com distrofia miotônica de Steinert.PALAVRAS-CHAVE: deglutição, endoscopia, doenças musculares, distrofia miotônica de Steinert. Oropharyngeal dysphagia in the myotonic dystrophy: phonoaudiological evaluation and nasofibrolaryngoscopical analysis.ABSTRACT -We herein present twenty myotonic dystrophy of Steinert patients with the main objective to evaluate and classify the oropharyngeal swallowing by the phonoaudiological clinical and nasofibrolaryngoscopical analysis. The age of the patients varied from 12 to 53 years, being 13 male and 7 female. The mean data: (1) statistically significant relation between the phonoaudiological clinical evaluation and nasofibrolaryngoscopical one; (2) stomatognatical system disorders present in 100%; (3) swallowing disorders present in 95%, when clinically evaluated, and in 70% when evaluated by the nasofibrolaryngoscopy; (4) higher difficulty to swallow consistent feed; (5) stomatognatical muscles very altered, pharyngeal phase disorders, cough after swallowing, antecedents of pneumonia and complaints of chewing/swallowing presented statistically significant correlation with severity of the sickness. The analysis were able to evaluate statical and functionally the involved structures in the swallowing, having got to take part of the rotine of attendance to the patients with myotonic dystrophy of Steinert.KEY WORDS: swallowing, endoscopy, muscle diseases, myotonic dystrophy of Steinert. A distrofia miotônica de Steinert (DMS) é a forma mais frequente das distrofias musculares, de iní-cio na idade adulta, merecendo uma atenção multiprofissional especial. A prevalência é de aproximadamente 4/100.000 1 . Trata-se de doença multissistêmica de herança autossômica dominante, decorrente de expansão do triplo CGT no braço longo do cromossomo 19. Observa-se ainda o fenômeno da ...

Desempenho longitudinal da deglutição orofaríngea na distrofia miotônica tipo 1

“…In the present longitudinal study of oropharyngeal swallowing in DM1 we did not detect changes in laryngeal sensitiviy as reported in the literature for this population (6) . We also wish to point out that no changes in laryngeal sensitivity were detected on the occasion of the initial evaluation of swallowing, or four years after the neurological diagnosis or at the final evaluation after one year and four months of follow-up.…”

“…In DM1, the presence of pharyngeal residues, the need for multiple swallowings, and laryngeal penetration and/or laryngotracheal aspiration are frequent findings reported in the literature, signs that suggest impaired and reduced efficiency and safety of swallowing (3)(4)(5)(6) . However, we do not know at what time during the progression of the disease the dysphagic symptoms will become a risk for the efficiency and/or the safety of swallowing.…”

“…We also wish to point out that no changes in laryngeal sensitivity were detected on the occasion of the initial evaluation of swallowing, or four years after the neurological diagnosis or at the final evaluation after one year and four months of follow-up. The presence of laryngeal sensitivity in the diagnosis of DM1 suggests that one of the mechanisms of airway protection, i.e., the cough reflex, is preserved and thus contributes to the expulsion of part or all the material that may be aspirated by the patient (6) . Although there are many mechanisms of lower airway protection, the preservation of laryngeal sensitivity in DM1 even by five years after the initial diagnosis is a finding that may contribute to the definition of the conduct regarding the fed orally.…”

“…This result is expected for patients with oropharyngeal dysphagia and muscle weakness since the increased viscosity of the food bolus plus the progression of muscle weakness interfere with both the oral food propulsion and pharyngeal cleaning mechanisms. Oropharyngeal muscle weakness impacts the oral phase of swallowing and consequently also the pharyngeal phase since the reduced pressure wave reduces the propulsion of the food bolus, increases the oral transit time and reduces the swallowing response as well as the pharyngeal peristalsis, thus compromising the elevation of the larynx (4)(5)(6) .…”

“…Although few studies have been conducted on oropharyngeal swallowing in DM1, there are reports of changes in swallowing with the presence of laryngeal penetration and laryngotracheal aspiration, pharyngeal residues, and multiple swallowing. The progressive muscle weakness affecting this population may have an impact on the oral and pharyngeal phases of swallowing, negatively affecting the efficiency and safety of swallowing (3)(4)(5)(6) .…”