Multi-disciplinary clinical protocol for the diagnosis of bulbar amyotrophic lateral sclerosis.

INTRODUCTION AND OBJECTIVES The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time. MATERIALS AND METHODS 22 patients with bulbar ALS were submitted for voice assessment, ENT evaluation, Multi-Dimensional Voice Program (MDVP), spectrogram, electroglottography, fiberoptic endoscopic evaluation of swallowing. RESULTS In the early stage of the disease, the oral tract and velopharyngeal port were involved. Three months after the initial symptoms, most of the patients presented hoarseness, breathy voice, dysarthria, pitch modulation problems and difficulties in pronunciation of explosive, velar and lingual consonants. Values of MDVP were altered. Spectrogram showed an additional formant, due to nasal resonance. Electroglottography showed periodic oscillation of the vocal folds only during short vocal cycle. Swallowing was characterized by weakness and incoordination of oro-pharyngeal muscles with penetration or aspiration. CONCLUSIONS A specific multidisciplinary clinical protocol was designed to report vocal parameters and swallowing disorders that changed more quickly in bulbar ALS patients. Furthermore, the patients were stratified according to involvement of pharyngeal structures, and severity index.

[1]  C. Gaelyn Garrett,et al.  Otolaryngologic presentations of amyotrophic lateral sclerosis , 2005, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery.

[2]  Jordan R. Green,et al.  Spatiotemporal coupling of the tongue in amyotrophic lateral sclerosis. , 2012, Journal of speech, language, and hearing research : JSLHR.

[3]  A. Geurts,et al.  Prognostic value of decreased tongue strength on survival time in patients with amyotrophic lateral sclerosis , 2012, Journal of Neurology.

[4]  W. Mcguirt,et al.  The otolaryngologist's role in the diagnosis and treatment of amyotrophic lateral sclerosis , 1980, The Laryngoscope.

[5]  Jordan R. Green,et al.  Profiling Speech and Pausing in Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD) , 2016, PloS one.

[6]  Gary L. Pattee,et al.  Bulbar and speech motor assessment in ALS: Challenges and future directions , 2013, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[7]  D. Robert,et al.  Évaluation des troubles de la parole et de la déglutition chez les patients atteints de sclérose latérale amyotrophique , 2006 .

[8]  Maciej Wiatr,et al.  The Evaluation of Abnormal Voice Qualities in Patients with Amyotrophic Lateral Sclerosis , 2015, Neurodegenerative Diseases.

[9]  Wieslaw Wszolek,et al.  Acoustic analysis of dysarthria profile in ALS patients , 1999, Journal of the Neurological Sciences.

[10]  R. Higo,et al.  Longitudinal analysis of progression of dysphagia in amyotrophic lateral sclerosis. , 2004, Auris, nasus, larynx.

[11]  B. Jacobson,et al.  Acoustic analysis of voice in individuals with amyotrophic lateral sclerosis and perceptually normal vocal quality. , 1997, Journal of voice : official journal of the Voice Foundation.

[12]  Panying Rong,et al.  Predicting Speech Intelligibility Decline in Amyotrophic Lateral Sclerosis Based on the Deterioration of Individual Speech Subsystems , 2016, PloS one.

[13]  Laura J. Ball,et al.  A protocol for identification of early bulbar signs in amyotrophic lateral sclerosis , 2001, Journal of the Neurological Sciences.

[14]  Yana Yunusova,et al.  Interarticulator coordination in dysarthria: an X-ray microbeam study. , 2003, Journal of speech, language, and hearing research : JSLHR.

[15]  A. Giovanni,et al.  Quantitative voice analysis in the assessment of bulbar involvement in amyotrophic lateral sclerosis. , 1999, Acta oto-laryngologica.

[16]  R. Guiloff,et al.  Dysarthria in amyotrophic lateral sclerosis: A review , 2010, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[17]  A. Cuvelier,et al.  Clinical screening of oropharyngeal dysphagia in patients with ALS. , 2012, Annals of physical and rehabilitation medicine.

[18]  O. Hardiman,et al.  Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: A population-based study. , 2000, Archives of neurology.

[19]  A. Riecker,et al.  Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis , 2008, Nature Clinical Practice Neurology.

[20]  F. cruz-Sánchez,et al.  Amyotrophic lateral sclerosis brain banking: a proposal to standardize protocols and neuropathological diagnostic criteria. , 1993, Journal of neural transmission. Supplementum.

[21]  Y. Kuroiwa,et al.  A Study of the Early Stage of Dysphagia in Amyotrophic Lateral Sclerosis , 2003, Dysphagia.

[22]  Panying Rong,et al.  Predicting Early Bulbar Decline in Amyotrophic Lateral Sclerosis: A Speech Subsystem Approach , 2015, Behavioural neurology.

[23]  Hua-shun Xie,et al.  [Acoustic analysis for 21 patients with amyotrophic lateral sclerosis complaining of dysarthria]. , 2014, Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences.

[24]  M. Turner,et al.  The diagnostic pathway and prognosis in bulbar-onset amyotrophic lateral sclerosis , 2010, Journal of the Neurological Sciences.