Correlação entre funcionalidade bulbar e penetração e/ou aspiração laringotraqueal na doença do neurônio motor / Correlation between bulbar functionality and laryngeal penetration and/or laryngotracheal aspiration on motor neuron disease

RESUMO Objetivo Descrever e correlacionar a funcionalidade bulbar com penetração e aspiração laringotraqueal em distintas consistências de alimento na Doença do Neurônio Motor (DNM). Método Participaram do estudo 18 indivíduos diagnosticados com DNM, independentemente do tipo e tempo da doença. Foi aplicada a escala Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised/BR (ALSFRS-R/BR), sendo analisado apenas o parâmetro bulbar que compreende fala, salivação e deglutição, com pontuação de 0 (incapacidade) a 12 (funcionalidade normal). Realizou-se videofluoroscopia da deglutição com aplicação da Penetration-Aspiration Scale (PAS) descrita por Rosenbek et al. (1996). Realizado Teste de Correlação de Pearson. Resultados Considerando a consistência do alimento, o nível da PAS variou de 1 a 5 na consistência pastosa, de 1 a 4 na líquida espessada e de 1 a 3 na líquida rala, e não houve aspiração laringotraqueal. Para todas as consistências de alimentos, houve correlação negativa entre funcionalidade bulbar e penetração laríngea (pastoso: r=-0,487, p=0,041; líquido espessado: r=-0,442, p=0,076; líquido ralo: r=-0,460, p=0,073), porém somente na consistência pastosa houve diferença estatística significante, ou seja, indivíduos com baixa funcionalidade bulbar apresentaram maior nível de penetração laríngea. Conclusão Houve correlação negativa entre funcionalidade bulbar e penetração laríngea na DNM. Os parâmetros bulbares da escala ALSFRS-R/BR mostraram-se significantes para predizer risco de penetração laringotraqueal na consistência pastosa na DNM.

ABSTRACT Objective Describe and correlate bulbar functionality with laryngeal penetration and/or laryngotracheal aspiration for different food consistencies in Motor Neuron Disease (MND). Methods Study participants were 18 individuals diagnosed with MND regardless of the type and time of onset of disease. The Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised/BR (ALSFRS-R/BR) was applied, and only the bulbar parameter, which includes speech, salivation and swallowing, was analyzed, with scores raging from 0 (disability) to 12 (normal functionality). Swallowing videofluoroscopy was performed using the Penetration-Aspiration Scale (PAS) described by Rosenbek et al. (1996). The Pearson correlation test was used for data analysis. Results According to food consistency, the PAS level ranged from 1 to 5 for puree consistency, 1 to 4 for thickened liquid, and 1 to 3 for liquid, and no laryngotracheal aspiration was observed. Negative correlation between bulbar functionality and laryngeal penetration was observed for all food consistencies (pasty: r=-0.487, p=0.041; thickened liquid: r=-0.442, p=0.076; liquid r=0.460, p=0.073), but statistically significant difference was found only for the puree consistency, that is, individuals with poor bulbar functionality presented higher levels of laryngeal penetration. Conclusion Negative correlation was observed between bulbar functionality and laryngeal penetration in MND. The bulbar parameters of the ALSFRS-R/BR are significant for predicting risk of laryngotracheal aspiration for pasty consistency in MND.

A Korean Case of Juvenile Muscular Atrophy of Distal Upper Extremity (Hirayama Disease) with Dynamic Cervical Cord Compression

We present a Korean case of Hirayama disease with its typical neuroradiological findings of forward displacement of cervical dural sac and compression of the lower cervical cord during neck flexion. A 15-yr-old boy was presented with a one-year history of progressive weakness and atrophy affecting bilateral hands and forearms. The electrodiagnostic findings were compatible with the lesion of the anterior horn cells at the C7, C8, and T1 spinal segments. With neck flexion, cervical magnetic resonance imaging (MRI) showed the anterior shifting of the lower cervical dural sac resulting in the cord compression of those segments. Presumably, this disease might have been prevalent in Korea frequently under the diagnosis of "benign focal amyotrophy". In this regard, we discuss the clinical importance of cervical MRI with neck flexion and anticipate the increasing reports of the case substantiated by its characteristic radiological features.