Cineradiographic Demonstration of Pharyngeal Area Myotonia in Myotonic Dystrophy Patients

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 ...

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“…Tais alterações são frequentemente relatadas na literatura 6,10,13,14,16,[20][21][22][23][24][25][26] .…”

Section: Discussionunclassified

Disfagia orofaríngea na distrofia miotônica: avaliação fonoaudiológica e análise nasofibrolaringoscópica

Chiappetta

Oda

Zanoteli

et al. 2001

Arq. Neuro-Psiquiatr.

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“…Weakness and myotonia of the striated muscle of the pharynx and upper oesophagus, and disordered peristalsis with involvement of the smooth muscle of the lower oesophagus occur (Goldberg et al, 1972). The dysphagia may be influenced by neck position (Bosma and Brodie, 1969). This was a specific symptom of his sister, and for the patient with a neck injury requiring traction, the neck movement is abolished and the dysphagia exacerbated.…”

Section: )mentioning

confidence: 99%

Myotonic dystrophy and traumatic quadriplegia: case report

1991

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Summ aryA thirty-year-old man with traumatic quadriplegia, was also found to have weakness above the level of the injury. He had facial weakness, difficulty in swallowing, and recurrent respiratory problems. A diagnosis of myotonic dystrophy was supported by examination of his sister. The problems of diagnosis, and the implications of the diagnosis on the management of the patient with myotonic dystrophy and a spinal injury are discussed.

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“…Esophageal motility may be normal if room temperature barium sulfate is administered, whereas aperistalsis may occur if chilled barium is used [18]. In patients with myotonie dystrophy, myotonia may develop in the pharynx only with the ingestion of chilled barium [19]. Ott and Gelfand [18] have advocated the use of chilled barium in double-contrast esophagography because it reduces primary peristalsis and improves distention of the lower esophagus and esophagogastric region.…”

Section: Varied Barium Suspensionsmentioning

confidence: 99%

How I do it: Examination of the patient with dysphagia

Jones

Donner

1989

Dysphagia

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Difficulty in swallowing is not an uncommon symptom. Approximately 10,000 persons choke to death every year in the United States, and at least 50% of patients in nursing homes have some difficulty eating or drinking. Dysphagia will become an increasing problem as the population continues to age, as more intensive resuscitative measures are applied, and as more aggressive head and neck surgery is performed. The practicing radiologist should be familiar with the examination technique and interpretation of swallowing studies.

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Cineradiographic Demonstration of Pharyngeal Area Myotonia in Myotonic Dystrophy Patients

Cited by 38 publications

References 4 publications

Disfagia orofaríngea na distrofia miotônica: avaliação fonoaudiológica e análise nasofibrolaringoscópica

Disfagia orofaríngea na distrofia miotônica: avaliação fonoaudiológica e análise nasofibrolaringoscópica

Myotonic dystrophy and traumatic quadriplegia: case report

How I do it: Examination of the patient with dysphagia

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