ABSTRACT
CONTEXT: In Machado-Joseph disease, poor posture, dystonia and peripheral neuropathy are extremely predisposing to oropharyngeal dysphagia, which is more commonly associated with muscular dystrophy. OBJECTIVE: To evaluate the clinical characteristics of oropharyngeal dysphagia in Machado-Joseph disease patients. METHOD: Forty individuals participated in this study, including 20 with no clinical complaints and 20 dysphagic patients with Machado-Joseph disease of clinical type 1, who were all similar in terms of gender distribution, average age, and cognitive function. The medical history of each patient was reviewed and each subject underwent a clinical evaluation of deglutition. At the end, the profile of dysphagia in patients with Machado-Joseph disease was classified according to the Severity Scale of Dysphagia, as described by O'Neil and collaborators. RESULTS: Comparison between dysphagic patients and controls did not reveal many significant differences with respect to the clinical evaluation of the oral phase of deglutition, since afflicted patients only demonstrated deficits related to the protrusion, retraction and tonus of the tongue. However, several significant differences were observed with respect to the pharyngeal phase. Dysphagic patients presented pharyngeal stasis during deglutition of liquids and solids, accompanied by coughing and/or choking as well as penetration and/or aspiration; these signs were absent in the controls. CONCLUSIONS: Oropharyngeal dysphagia is part of the Machado-Joseph disease since the first neurological manifestations. There is greater involvement of the pharyngeal phase, in relation to oral phase of the deglutition. The dysphagia of these patients is classified between mild and moderate.
CONTEXTO: Na doença de Machado-Joseph, a má postura, a distonia e a neuropatia periférica predispõem à disfagia orofaríngea, mais comumente associada à distrofia muscular. OBJETIVO: Avaliar as características clínicas da disfagia orofaríngea em pacientes com doença de Machado-Joseph. MÉTODOS: Quarenta indivíduos participaram do estudo, incluindo 20 sem quaisquer queixas clínicas e 20 disfágicos com doença de Machado-Joseph do tipo clínico 1, grupos similares em termos de sexo, média de idade e função cognitiva. Foi verificada a história clínica de cada paciente e todos os indivíduos passaram por avaliação clínica da deglutição. Ao final, a disfagia dos enfermos com doença de Machado-Joseph foi classificada de acordo com a Escala de Severidade da Disfagia. RESULTADOS: A comparação entre disfágicos e controles não revelou muitas diferenças significativas quanto à avaliação clínica da fase oral da deglutição, visto que os pacientes demonstraram déficits apenas relacionados à protrusão, retração e tônus linguais. Entretanto, em relação à fase faríngea, várias alterações relevantes, ausentes nos controles, foram notadas nos pacientes, tais como estase faríngea à deglutição de líquidos e sólidos, acompanhada de tosse e/ou engasgo, assim como de penetração e/ou aspiração laringotraqueal. CONCLUSÕES: Disfagia orofaríngea faz parte da doença de Machado-Joseph desde as primeiras manifestações neurológicas. Há maior comprometimento da fase faríngea, em relação à fase oral da deglutição. A disfagia desses pacientes é classificada entre leve e moderada.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Young Adult , Deglutition Disorders/diagnosis , Machado-Joseph Disease/complications , Case-Control Studies , Deglutition Disorders/etiology , Deglutition Disorders/physiopathology , Machado-Joseph Disease/physiopathology , Prospective Studies , Severity of Illness IndexABSTRACT
OBJECTIVE: This study investigates resources to provide better conditions for oropharyngeal swallowing for improvement in the quality of life of Parkinson's disease patients. METHOD: Three men and one woman with an average age of 70.25 years had been afflicted with Parkinson's disease for an average of 9.25 years. The patients were submitted to a rehabilitation program for oropharyngeal dysphagia after a clinical evaluation of swallowing. The rehabilitation program consisted of daily sessions for two consecutive weeks during which a biofeedback resource adapted especially for this study was used. The patients were then reevaluated for swallowing ability at follow-up. RESULTS: The patients presenting difficulties with swallowing water displayed no such problems after rehabilitation. Only one patient exhibited slow oral transit of food and other discrete oropharyngeal food remnants when swallowing a biscuit. The sample variance was used to analyze the pressure measurements, demonstrating a numerical similarity of the results obtained with the swallowing of saliva or of biscuits (VAR = 4.41). A statistical difference was observed between the swallowing of saliva and biscuits, showing a significant pressure increase at the end of the rehabilitation program (p < 0.001). CONCLUSION: The effortful swallow maneuver reinforced by using biofeedback appears to be a therapeutic resource in the rehabilitation of oropharyngeal dysphagia in Parkinson's disease patients.