Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30–50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5–9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression. Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation. Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment. Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.

The developmental eye movement (DEM) test is a practical and simple method for assessing and quantifying ocular motor skills in children. In this review, a summary of the literature relevant to the DEM test has been made, its psychometric properties and its pros and cons have also been considered. The DEM test provides clinicians with a simple method of measuring eye movement using a psychometric test. Over the years, many studies have enabled the identification of the strengthens and weaknesses of this test apart from outlining the psychometric properties. The validity of the test has been checked and expanded over time and studies have shown that the DEM test measures an aspect of eye movement related to reading, rather than purely parameters associated with eye-movement. Some reservations have emerged regarding the repeatability of the test because a degree of learning effect emerges over multiple sessions. Being aware of this point allows correct clinical application and interpretation of the test. Normative data in children were available for nine languages and countries. So far, DEM test could be applied clinically in each case when a rapid test of eye movement was required, such as in testing for vision-related visual problems.

The developmental eye movement (DEM) test is a practical and simple method for assessing and quantifying ocular motor skills in children. Different studies have previously assessed the reliability of the DEM test and they have generally found high values for vertical and horizontal time, whereas those for Ratio and Errors were medium and low, respectively. In the second application of test were found an improvement in performance in all subtests. Our aim was to evaluate the reliability of the DEM test using seconds and percentile scoring and looking in depth at the improvement in performance when the test is repeated. We tested the reliability of the DEM test on a group of 115 children from the 2nd to the 5th grade using different statistical methods: correlations, ANOVA, limits of agreement for results expressed in seconds and as percentile scoring and pass-fail diagnostic classification. We found high reliability with excellent values for vertical and adjusted horizontal time, medium-to-high for ratio and medium for errors. We have re-confirmed the presence of a significant improvement of performance on the second session for vertical time, horizontal time and ratio. The stability of binary classification of Pass–Fail criteria appears to be medium. We found high reliability for the DEM test when compared with the published results of other research but the improvement of performance, the learning effect was still present, but at a lower level than previously found. With the awareness of these limitations the DEM test can be used in clinical practice in evaluating performance over time.

The Glare Effect is an illusion in which a white region appears self-luminous when surrounded by linearly decreasing luminance ramps. It has been shown that the magnitude of the luminosity effect can be modulated by manipulating the luminance range of the gradients. In the present study we tested the thresholds for the glare effect on two groups of adults: young (20-30 years old) and elderly (60-75 years old). Purpose of our perspective study was to test the possibility of transforming the glare effect into a test that could easily measure thresholds for luminosity and discomfort glare. The Glare Effect Test (GET) consisted in 101 printed cards that differed from each other for the range of luminance ramps. Participants were assessed with GET and a battery of visual tests: visual acuity, contrast sensitivity, illusion of length perception, and Ishihara test. Specifically in the GET, participants were required to classify cards on the basis of two reference cards (solid black-no gradient; full range black to white gradient). PSEs of the glare effect show no correlation with the other visual tests, revealing a divergent validity. A significant difference between young and elderly group was found: contrary to our original expectations, luminosity thresholds for elderly were higher than those for young, suggesting a non-direct relationship between luminosity perception and discomfort glare.

Introduction: Neuropsychological assessment of cognitive functioning is a crucial part of clinical care: diagnosis, treatment planning, treatment evaluation, research, and prediction of long-term outcomes. The Equivalent Score (ES) method is used to score numerous neuropsychological tests. The ES0 and the ES4 are defined respectively by the outer tolerance limit and the median. The intermediate ESs are commonly calculated using a z-score approach even when the distribution of neuropsychological data is typically non-parametric. To calculate more accurate ESs, we propose that the intermediate ESs need to be calculated based on a non-parametric rank subdivision of the distribution of the adjusted scores. Material and methods: We make three simulations to explain the differences between the classical z-score approach, the rank-based approach, and the direct subdivision of the dependent variable. Results: The results show that the rank procedure permits dividing the region between ES0 and ES4 into three areas with the same density. The z-score procedure is quite similar to the direct subdivision of the dependent variable and different from the rank subdivision. Conclusions: By subdividing intermediate ESs using the rank-subdivision, neuropsychological tests can be scored more accurately, also considering that the two essential points for diagnosis (ES = 0 and ES = 4) remain the same. Future normative data definition should consider the best procedure for scoring with ES.