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Background and Purpose: The prediction of disease course is one of the main targets of amyotrophic lateral sclerosis (ALS) research, particularly considering its wide phenotypic heterogeneity. Despite many attempts to classify patients into prognostic categories according to the different spreading patterns at diagnosis, a precise regional progression rate and the time of involvement of each region has yet to be clarified. The aim of our study was to evaluate the functional decline in different body regions according to their time of involvement during disease course., Methods: In a population-based dataset of ALS patients, we analysed the functional decline in different body regions according to time and order of regional involvement. We calculated the regional progression intervals (RPIs) between initial involvement and severe functional impairment using the ALS Functional Rating Scale revised (ALSFRS-r) subscores for the bulbar, upper limb, lower limb and respiratory/thoracic regions. Time-to-event analyses, adjusted for age, sex, ALSFRS-r pre-slope (ΔALSFRS-R), cognitive status, and mutational status were performed., Results: The duration of RPI differed significantly among ALS phenotypes, with the RPI of the first region involved being significantly longer than the RPIs of regions involved later. Cox proportional hazard models showed that in fact a longer time between disease onset and initial regional involvement was related to a reduced duration of the RPI duration in each different body region (bulbar region: hazard ratio [HR] 1.11, 95% confidence interval [CI] 1.06-1.16, p < 0.001; upper limb region: HR 1.16, 95% CI 1.06-1.28, p = 0.002; lower limb region: HR 1.11, 95% CI 1.03-1.19, p = 0.009; respiratory/thoracic region: HR 1.10, 95% CI 1.06-1.14, p = 0.005)., Conclusions: We found that the progression of functional decline accelerates in regions involved later during disease course. Our findings can be useful in patient management and prognosis prediction., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background and Purpose: Oropharyngeal dysphagia is generally recognized to increase the risk of malnutrition; however, its role in patients with neurodegenerative disease has yet to be determined. This cross-sectional study aimed to investigate the impact of swallowing function on malnutrition risk in patients with neurodegenerative diseases., Methods: Patients with oral nutrition and diagnosis of Huntington disease (HD), Parkinson disease (PD), or amyotrophic lateral sclerosis (ALS) were recruited. Demographic and clinical data were collected. The swallowing assessment included a fiberoptic endoscopic evaluation of swallowing, an oral phase assessment, and a meal observation scored with the Mealtime Assessment Scale (MAS). Malnutrition risk was assessed with the Mini Nutritional Assessment., Results: Overall, 148 patients were recruited (54 HD, 33 PD, and 61 ALS). One hundred (67.6%) patients were considered at risk of malnutrition. In the multivariate analysis, age ≥ 65 years (odds ratio [OR] = 3.16, p = 0.014), disease severity (moderate vs mild OR = 3.89, severe vs mild OR = 9.71, p = 0.003), number of masticatory cycles (OR = 1.03, p = 0.044), and MAS safety (OR = 1.44, p = 0.016) were significantly associated with malnutrition risk., Conclusions: Prolonged oral phase and signs of impaired swallowing safety during meals, together with older age and disease severity, are independent predictors of malnutrition risk in neurodegenerative diseases. This study broadens the focus on dysphagia, stressing the importance of early detection not only of pharyngeal signs, but also of oral phase impairment and meal difficulties through a multidimensional swallowing assessment., (© 2022 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

The coefficient of thermal expansion of core and veneer porcelains for all ceramic crowns must be compatible to prevent formation of stresses during the firing cycles. The purpose of this study was to use a thermal shock test to evaluate the crazing resistance of six all-ceramic crown systems. The systems tested were: Vita In-Ceram, Vita Hi-Ceram, Dicor veneered with Vitadur N, magnesia core veneered with Ceramco II, magnesia core veneered with Silhouette, and magnesia core veneered with Vita VMK 68. A maxillary central incisor was prepared with 1.0 mm axial and 1.5 mm incisal reduction. Refractory dies were prepared and crowns were fabricated using the manufacturers' recommendations. Six specimens of each system were placed inside a beaker in a furnace at 90 degrees C. After 15 min heat soak, ice water was poured into the beaker through a funnel. The samples were dried in air, reheated, and inspected for crazing using light microscopy. If crazing was observed, this would constitute a failure at a temperature difference (delta T) of 90 degrees C. If no failure occurred, the test was repeated at increasing temperature increments of 10 degrees C until failure. The crazing resistance of each system, expressed as the mean delta T, was: > 200 (Hi-Ceram), 163 +/- 14 (In-Ceram), 152 +/- 19 (Dicor/Vitadur N), 143 +/- 5 (magnesia core/Vita VMK 68), 122 +/- 21 (magnesia core/Silhouette), and 118 +/- 10 (magnesia core/Ceramco II) degrees C. All systems tested resisted crazing due to quenching in ice cold water from 100 degrees C. Systems with lower coefficients of expansion showed significantly greater resistance to thermal shock using ANOVA.