Clinical and demographical characteristics in a cohort of MND patients treated with riluzole. Differences between tablets and oral suspension.

Objective: To describe the clinical and demographic characteristics of patients with MND treated with riluzole by comparing two dosage forms (oral suspension and tablets), as well as the impact on survival in patients with and without dysphagia according to the form of dosage.Methods: Retrospective and prospective cohort of patients diagnosed with MND at the multidisciplinary functional unit of Motor Neuron Disease in our center in the period between 1 of January 2011 and 31 of December 2020 (n = 742). A descriptive analysis (univariate and bivariate) was carried out and survival curves were estimated.Results: During the follow-up period, 402 males (54.18%) and 340 females (45.82%) were diagnosed with MND. Of these patients, 632 (97.23%) were being treated with 100mg riluzole: 282 (54.55%) patients took this in tablet form and 235 (45.45%) oral suspension. Riluzole in tablet form is taken more frequently by men than women, in younger age ranges, and mostly without dysphagia (78.31%). Also, it is the predominant dosage form for classic spinal ALS and respiratory phenotypes. Dosages via oral suspension are taken by patients in the older age ranges (over 64.8 years), mostly with dysphagia (53.67%) and more frequently with bulbar phenotypes such as classic bulbar ALS and PBP. Because of this, patients using oral suspension (most of them with dysphagia) had a poorer survival rate (at 90% CI) than patients using tablets (most of them without dysphagia).Conclusions: The most appropriate dosage form should be given according to the patient's needs at each stage of the disease and, furthermore, oral suspension could improve adherence to treatment because it avoids having to change from one form (tablet) to the other (suspension) when swallowing disorders appear.

Impact of dysphagia and malnutrition on the survival of hospitalized patients.

INTRODUCTION: Oropharyngeal dysphagia (OD) and malnutrition (MN) are highly prevalent among hospitalized patients, with significant clinical repercussions. OBJECTIVES: To assess the prevalence, survival and factors associated with OD and MN in hospitalized patients with a high risk of OD. METHODS: A cross-sectional observational study with 82 patients aged ≥70 years and with the possibility of oral feeding admitted in 4 services of a third level hospital during 3 months. The Nutritional Risk Screening 2002 test (NRS-2002) was performed to detect nutritional risk and the volume-viscosity screening test (V-VST) for OD evaluation. Data were collected on the clinical suspicion of OD, days of hospital stay, the number of readmissions and other socio-demographic data. RESULTS: 50.6% had OD and 51.9% MN. In 48.8%, there was underdiagnosis of OD. The median number of days of admission was higher among patients with MN (19.5 days vs 13 days, p = 0.02). Of the total readmissions, 70.8% had MN compared to 29.2% that did not (p = 0.03). Survival among patients who did not survive one year after admission was lower when OD was given (Sig. = 0.04). CONCLUSIONS: More than half of the population studied has OD, as well as DN, which increases the rate of readmission and decreases survival at the year of admission. Although there are specific screening methods, their use is not widespread, making it difficult to diagnose OD and its therapeutic intervention.

Evaluation of Dysphagia in Motor Neuron Disease. Review of Available Diagnostic Tools and New Perspectives.

Oropharyngeal dysphagia (OD) is highly prevalent (up to 80%) in patients with motor neuron disease (MND), influencing the prognosis of the disease. The clinical assessment of dysphagia is complex. There are assessment scales and screening questionnaires, but they have not been tested in patients with MND. In a sample of 46 patients with MND, the sensitivity and specificity of the EAT-10 and SwalQoL questionnaires, as well as the ALS-SS and FOIS scales, were tested and compared to the gold standard technique (videofluoroscopy, VFS). The patients were stratified using the DOSSc variable according to the video fluoroscopic examination with (n = 37) or without (n = 8) signs of dysphagia, and the results were compared with the scores obtained in the dysphagia questionnaires. None of the studied questionnaires was more sensitive than the others, but one stood out for its high specificity (= 1): the SwalQoL revised FS. The symptom frequency section of the SwalQoL questionnaire with some modifications, (SwalQoL revised FS) may be a useful tool in the clinical assessment of dysphagia because it's capable to detect the patients that really don't have dysphagia. The ALS-SS showed the greatest validity as a severity scale of dysphagia among the sample studied. A specific questionnaire for screening for dysphagia in MND needs to be developed. Until that time, the proposal is to use a combination of the existing questionnaires for other pathologies (EAT-10 and SwalQoL) and the specific scale for MND, the ALS-SS, to make an accurately clinical assessment of OD in MND patients before to perform a videofluoroscopy.