Disfagia orofaríngea na distrofia miotônica. Avaliação fonoaudiológica e analise nasofibrolaringoscópica

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Data
1999
Autores
Chiappetta, Ana Lúcia de Magalhães Leal [UNIFESP]
Orientadores
Oliveira, Acary Souza Bulle [UNIFESP]
Tipo
Tese de doutorado
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Apresentamos vinte pacientes com Distrofia Miotonica de Steinert, com o objetivo de avaliar e classificar a degluticao orofaringea pela analise clinica fonoaudiologica e nasofibrolaringoscopica, as funcoes do sistema estomatognatico relacionadas a degluticao e demonstrar achados preditivos. O diagnostico de DMS foi realizado atraves de achados em exames clinicos caracteristicos, historia familiar, encontros eletroneuromiograficos e resultados de analise genetica. O estudo foi realizado no periodo de 1995 a 1999. A idade dos pacientes variou de 12 a 53 anos, com mediana de idade de 35 anos, media de 33,7 anos e desvio padrao de 12,7 anos. O tempo referido de doenca pelo paciente variou de 1 a 24 anos, com mediana de 10 anos, media de 9,6 anos e desvio-padrao de 6,6 anos, sendo 13 pacientes do sexo masculino e 7 pacientes do sexo feminino. Os principais dados observados nas analises foram: (1) relacao estatisticamente significante entre a avaliacao clinica fonoaudiologica e nasofibrolaringoscopica chegando a 1OO por cento nos casos considerados graves; (2) alteracoes do sistema estomatognatico presentes em 1OO por cento dos casos , sendo os musculos mastigatorios o grupo muscular mais comprometido; (3) desordens de degluticao presente em 95 por cento dos pacientes, quando avaliados clinicamente com alimentos liquidos, pastosos e solidos; e em 70 por cento dos pacientes quando avaliados pela nasofibrolaringoscopia com alimentos liquidos e pastosos; (4) maior dificuldade para deglutir alimentos mais consistentes (solidos e pastosos); (5) musculos do sistema estomatognatico muito alterado, presenca de desordem da fase faringeal, presenca de tosse apos degluticao, antecedentes de Pneumonia e queixas referidas de mastigacao e degluticao apresentaram uma correlacao estatisticamente significante com maior gravidade da doenca. As analises foram capazes de avaliar estatica e funcionalmente as estruturas envolvidas na dinamica da degluticao, devendo fazer parte da rotina do atendimento aos pacientes com DMS
Patients with MND/ALS develop oropharyngeal dysphagia, as a result of progressive loss of bulbar and respiratory muscle function. Objective: To identify oropharyngeal dysphagia in patients with MND/ALS and to associate it with nutritional and respiratory aspects in disease progression. Methods: Thirty-eight patients with bulbar impairment - 29 with the classical form or ALS, and 9 with the bulbar form or PBP – were evaluated at admission to regular speech therapy sessions and re-assessed three months later. The oral and pharyngeal phase findings were gathered by clinical speech therapy assessment and swallowing endoscopy. Nutritional and respiratory assessment tests were erformed simultaneously. Results: The results showed oropharyngeal dysphagia in 100% of patients in the PBP group suffering from bulbar impairment or 17 months, and in 65.5% of individuals in the ALS group presenting the condition for 10.7 months. In the latter, dysphagia rate raised to 75.9% within three months. Significant progressive weakness of the anterior muscles of the tongue and velum was observed and caused the first alterations in retrograde food bolus propulsion. The PBP group had more aggravated muscular alterations than the ALS group. Analyzing the endoscopy, we observed reduced tongue base elevation, incomplete glottic closure and reduced laryngeal elevation. The use of BiPAP presented significant variation with worsening in the ALS group. There was progressive impairment of the pharyngeal phase of deglutition regarding to accumulation of food residues after swallowing, laryngeal penetration and tracheal aspiration. Difficulty in swallowing was more frequent in the early stages for liquid and solid food and, in the advanced stages, for liquid and pureed food. As to respiratory assessment, there was a progressive loss of respiratory patterns, and vital capacity (VC) was the most severe, with significantly reduced values in the group with delayed emptying, accumulation of residues, laryngeal penetration, tracheal aspiration, use of percutaneous endoscopic gastrostomy (PEG) and use of non-invasive ventilation (BiPAP). The maximum inspiratory pressure (PImax) and the maximum expiratory pressure (PEmax) also presented significantly reduced values that were related to progressive oropharyngeal changes. Regarding nutritional assessment, there was no significant difference in anthropometrical measures between the PBP and ALS groups. However, when assessing total body weight in the ALS group and considering the presence or not of delayed emptying and laryngeal penetration, there was a significant difference. Conclusions: (1) Dysphagia in MND/ALS is time-dependent and aggravates with progressive muscle impairment in the stomatognatic system. (2) Values below 50% of expected vital capacity and 30% of PEmax and PImax are predictive of worsening of swallowing pharyngeal phase. (3) Changes in nutritional status were independen of oropharyngeal dysphagia progression; nonetheless, dysphagia is an aggravating factor in MND/ALS patients, particularly in those with decreased total body weight.
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Citação
CHIAPPETTA, Ana Lúcia de Magalhães Leal. Disfagia orofaríngea em pacientes com doença do neurônio motor/esclerose lateral amiotrófica. 2005. 142 f. Tese (Doutorado) - Escola Paulista de Medicina, Universidade Federal de São Paulo. São Paulo, 1999.