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Objective: The Ljubljana ALS Centre, established in 2002, is the only tertiary center for amyotrophic lateral sclerosis (ALS) in Slovenia. The aim of our study was to evaluate the impact of therapeutic interventions and improvements in the multidisciplinary care on the survival of our patients. Methods: All patients diagnosed with ALS at our center during years 2003–2005 (early group) and 2011–2012 (late group) were included in this retrospective cohort study (n = 124). Kaplan-Meier survival analysis and multiple regression analysis with Cox proportional hazards model were performed to compare survival and to evaluate the differences between the two cohorts. Results: Median survival from the time of diagnosis was 13.0 (95% CI 10.2–15.8) months in the early group and 21.8 (95% CI 17.2–26.4) months in the late group (p = 0.005). In the Cox proportional hazards analysis, the late group of patients was associated with better survival independently of all other prognostic factors (hazard ratio (HR)=0.51, 95% CI = 0.32–0.81, p = 0.004). Survival was also associated with patients' age, use of noninvasive ventilation (NIV) and gastrostomy. The model fit significantly improved when the interaction between the NIV use and the observed time period was added to the model (HR = 0.34, 95% CI = 0.12–0.96, p = 0.041). Conclusions: Our findings suggest that improvements in the multidisciplinary care were beneficial for survival of our patients with ALS. The survival benefit in the late group of our patients could be partially explained by the improvements in the NIV use at our center. [ABSTRACT FROM AUTHOR]

6 Eisen A, Kuwabara S. J Neurol Neurosurg Psychiatry. J Neurol Neurosurg Psychiatry. 47 Desai J, Swash M. J Neurol Neurosurg Psychiatry. 9 Eisen A, Kuwabara S. J Neurol Neurosurg Psychiatry. [Extracted from the article]

Our aim was to investigate changes in movement-related cortical potentials (MRCPs) in ALS patients with different degrees of upper motor neuron (UMN) involvement. Since respiratory failure is the main cause of death in ALS, changes in inspiratory-related (sniffing) potentials were studied in addition to finger-flexion-related potentials. Subjects (21 ALS, 19 controls) performed two self-paced motor tasks while their EEGs were recorded. The first task required flexions of the right index finger and the second, brisk nasal inspirations. The early (BP1), late (BP2) and motor potential (MP) components of MRCPs were evaluated. Results showed that patients generated higher MRCPs than controls. However, this effect was most significant in the subgroup of patients with low UMN burden (LUB). The high UMN burden (HUB) subgroup did not differ from controls, but had significantly lower MP amplitudes than the LUB subgroup. Progressive UMN deterioration was associated with an initial increase, followed by a later decrease, in MP amplitudes in ALS. In conclusion, the increased MRCPs in LUB compared to HUB patients indicate different processes of ALS pathophysiology that force opposing changes in MRCP amplitudes. [ABSTRACT FROM AUTHOR]

The study aimed to evaluate the nature and extent of executive deficits in nondemented amyotrophic lateral sclerosis (ALS) patients. A total of 22 ALS patients and 21 matched controls were compared on standard neuropsychological tests of executive functions with appropriate control for motor impairment and on an ecologically valid motor-free test of executive functions, the Medication Scheduling Task (MST). Our results show that motor dysfunction can present a significant confound when using standard neuropsychological measures; however, even when accounting for motor disabilities, ALS patients show a robust pattern of cognitive dysfunctions. Additionally, MST was shown to be a sensitive measure of cognitive impairment, providing an important insight into cognitive processes relevant for patients' daily living. [ABSTRACT FROM AUTHOR]