Dysphagia in Amyotrophic Lateral Sclerosis: Impact on Patient Behavior, Diet Adaptation, and Riluzole Management.

This retrospective study aimed to investigate the clinical features associated with deteriorated swallow in amyotrophic lateral sclerosis (ALS) patients with spinal and bulbar onset, describe the modification of diet and liquid intake, and assess the impact of dysphagia on the use of riluzole. One hundred forty-five patients were observed periodically every 3-6 months. They underwent routinely fiberoptic endoscopic evaluation of swallowing (FEES) and spirometry; dysphagia severity was classified according to the Penetration Aspiration Scale and the Pooling score (P-score) integrated with other parameters such as sensation, collaboration, and age (P-SCA score). During a mean follow-up period of about 2 years, the percentage of ALS patients suffering from dysphagia increased to 85 (rising from 35 to 73% in patients with spinal onset and from 95 to 98% in those with bulbar onset). Also, 8% of patients with dysphagia by FEES did not perceive the disorder. The frequency of normal and semi-solid diets decreased over time, while that of pureed diets and percutaneous endoscopic gastrostomy (PEG) prescription increased. Forty-four percent of dysphagic patients refused thickeners or PEG. A significant difference was observed in the mortality rate between patients untreated with riluzole and patients treated with riluzole oral suspension (p < 0.05). Disease duration mainly impacted on the frequency of dysphagia in spinal onset patients, appearing very early in those with bulbar onset. Riluzole oral suspension would allow the safe administration in dysphagic ALS patients to avoid tablet crushing and consequent dispersion in food, common practices that are inconsistent with the safe and effective use of the drug.

Laryngeal Sensitivity in Patients with Amyotrophic Lateral Sclerosis.

Recent studies have shown the involvement of the sensory nervous system in patients with amyotrophic lateral sclerosis (ALS). The aim of our study was to investigate the correlation between the laryngeal sensitivity deficit and the type of ALS onset (bulbar or spinal) in a large series of 114 consecutive ALS patients. Participants were subdivided into two groups, bulbar and spinal ALS, according to the clinical onset of disease and submitted to a clinical and instrumental evaluation of swallowing, including a fiber-optic endoscopic evaluation of swallowing with sensory testing. Dysphagia severity was scored using the Penetration-Aspiration Scale (PAS) and the Pooling score (P-score). In addition, three patients with laryngeal sensitivity deficit were submitted to a laryngeal biopsy to assess the status of the sensory innervation. All patients showed a normal glottal closure during phonation and volitional cough. Fifty-six subjects (49%), 14 spinal- and 42 bulbar-onset ALS, showed dysphagia at the first clinical observation (PAS score >1; P-score >5). Dysphagia resulted more frequently in bulbar-onset ALS (P < 0.01). Thirty-eight (33%) patients had a sensory deficit of the larynx. The sensory deficit of the larynx was significantly more frequent in bulbar-onset ALS (P < 0.01). The sensory deficit of the larynx among dysphagic patients was also significantly more frequent in bulbar-onset ALS (P = 0.02). Several abnormalities were found in all three subjects who underwent a laryngeal biopsy: in one patient, no intraepidermal fiber was found; in the other two, the fibers showed morphological changes. Our observations are important to consider for assessment and management of dysphagia in patients with ALS.

Afferent nerve ending density in the human laryngeal mucosa: potential implications on endoscopic evaluation of laryngeal sensitivity.

Laryngeal sensitivity is crucial for maintaining safe swallowing, thus avoiding silent aspiration. The sensitivity test, carried out by fiberoptic endoscopic examination of swallowing, plays an important role in the assessment of dysphagic patients. The ventricular folds appear to be more sensitive than the epiglottis during the sensitivity test. Therefore, this study aimed to investigate the mechanical sensitivity of the supraglottic larynx. In seven healthy adults undergoing microlaryngoscopy to remove vocal cord polyps, we excised mucosal samples from the epiglottis and ventricular folds. We measured afferent nerve fiber density by immunoelectron microscopy. All of the subjects underwent an endoscopic sensitivity test based on lightly touching the laryngeal surface of the epiglottis and ventricular folds. The discomfort level was self-rated by the subjects on the visual analog scale. Samples were fixed and stored in cryoprotectant solution at 4 °C. Sections were stained with the protein gene product 9.5, a pan-neuronal selective marker. Nerve fiber density was calculated as the number of fibers per millimeter length of section. The mean nerve fiber density was higher in ventricular samples than in epiglottis samples (2.96 ± 2.05 vs 0.83 ± 0.51; two-sided p = 0.018). The mean visual analog scale scores were significantly higher for touching the ventricular folds than for touching the epiglottis (8.28 ± 1.11 vs 4.14 ± 1.21; two-sided p = 0.017). The higher sensitivity of the ventricular region should be considered for further refining clinical endoscopic evaluation of laryngeal sensitivity.

Cross-cultural adaptation and validation of the Voice Handicap Index into Italian.

OBJECTIVES: To evaluate the internal consistency, reliability, and clinical validity of the Italian version of the Voice Handicap Index (VHI). STUDY DESIGN: Cross-sectional survey study was carried out. METHODS: One hundred and seventy-five patients with voice disorders, divided in four groups according to the etiology of the disease (neurogenic, structural, functional, and inflammatory), and 84 asymptomatic subjects were included in the study. Internal consistency was analyzed through Cronbach α coefficient. For the VHI test-retest reliability analysis, the Italian VHI was filled twice by 56 patients and 56 control subjects. The test-retest reliability was assessed through the Pearson correlation test. For the clinical validity assessment, the scores obtained in the pathological group were compared with those found in asymptomatic individuals through the Kruskal-Wallis test. Also, the correlation between VHI and the grade of voice disorder was assessed. Finally, the effect of age and gender on overall VHI and its three subscales was analyzed. RESULTS: Optimal internal consistency was found (α=0.93); the test-retest reliability in both groups was high (r>0.86). Nonparametric Kruskal-Wallis analysis of variance for the overall VHI score and its three domains revealed a significant main effect for group (P=0.000). The control group scored significantly lower than the four groups of voice-disordered patients. The overall VHI score positively correlated with the grade of voice disorder (r=0.43). In the voice-disorder group, age and gender were not correlated to the overall VHI score and to their three domains. CONCLUSION: The Italian VHI is highly reproducible, and exhibits excellent clinical validity.