Secular Trends of Amyotrophic Lateral Sclerosis

Chiò, Adriano; Mora, Gabriele; Moglia, Cristina; Manera, Umberto; Canosa, Antonio; Cammarosano, Stefania; Ilardi, Antonio; Bertuzzo, Davide; Bersano, Enrica; Cugnasco, Paolo; Grassano, Maurizio; Pisano, Fabrizio; Mazzini, Letizia; Calvo, Andrea

doi:10.1001/jamaneurol.2017.1387

Cited by 90 publications

(56 citation statements)

References 36 publications

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“…The study population was not significantly different from PARALS population [10] (see Table S2). Compared to patients with lateralized (S ri and S le ) spinal onset, patients with bulbar onset were older (Mann-Whitney-U test p = 0.017) and predominantly female (bulbar 58.7% vs. spinal 34.5%, chi-square test p < 0.001), as expected, while also patients with bilateral spinal onset were more frequently older (bilateral vs. lateralized spinal onset, Mann-Whitney-U test p = 0.020).…”

Section: Resultsmentioning

confidence: 94%

“…The study population included all ALS cases diagnosed between 2010 and 2018 who underwent neuropsychological evaluation at the Turin ALS Center. Site of onset, side of onset, date of onset, sex, hand dominance, and years of formal education were collected using data recorded in the Piemonte and Valle D'Aosta register for ALS (PARALS, [10]). All neuropsychological evaluations were performed at diagnosis (diagnosis-evaluation interval < 3 months).…”

Section: Data Collectionmentioning

confidence: 99%

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The Characteristics of Cognitive Impairment in ALS Patients Depend on the Lateralization of Motor Damage

et al. 2020

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(1) Background: Cognitive features of patients with amyotrophic lateral sclerosis (ALS) have never been specifically analyzed according to the lateralization of motor impairment. In the present study we investigated the cognitive performances of ALS patients to describe the relationship between motor and cognitive dysfunction, according to site and side of disease onset. (2) Methods: Six-hundred and nine ALS patients underwent a comprehensive neuropsychological evaluation at diagnosis in Turin ALS Centre Tests included—mini-mental state examination (MMSE), frontal assessment battery (FAB), trail-making test A/B (TMT A-B), digit span forward and backward (digit span FW/digit span BW), letter fluency test (FAS), category fluency test (CAT), Rey auditory verbal learning test (RAVLT), Babcock story recall test (BSRT), Rey-Osterrieth complex figure test (ROCFT), Wisconsin card sorting test (WCST), Raven’s coloured progressive matrices (CPM47). Cognitive performances of patients, grouped by side and site of onset, were statistically compared using z-scores, as appropriate. (3) Results: Bulbar patients and bilateral spinal onset patients (Sbil) were generally characterized by lower cognitive performances in most neuropsychological tests, when compared to patients with lateralized onset (right-side spinal onset, Sri and left-side spinal onset, Sle). Digit span backward and visual memory task (ROCFT) median z-scores were significantly higher, reflecting a better cognitive performance, in Sri patients when compared to bulbar/Sbil patients, while verbal memory tasks (RAVLT and BRST) resulted in significantly higher scores in Sle patients. Our results are in keeping with hemispheric functional lateralization of language and visuospatial abilities. (4) Conclusions: In ALS patients, as in other neurodegenerative diseases, we found a direct relationship between lateralized motor and cognitive features.

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Section: Resultsmentioning

confidence: 94%

Section: Data Collectionmentioning

confidence: 99%

The Characteristics of Cognitive Impairment in ALS Patients Depend on the Lateralization of Motor Damage

et al. 2020

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“…In terms of geographical distribution, incidence is higher in populations of European than Asian origin [5]. An increase in ALS incidence has been reported in recent decades [6], in Italy as well [7][8][9][10][11]. Despite progress in assessing the role of genetic factors [12], the etiology of ALS remains largely unknown.…”

Section: Introductionmentioning

confidence: 99%

Clinical and Lifestyle Factors and Risk of Amyotrophic Lateral Sclerosis: A Population-Based Case-Control Study

Filippini

Fiore

Tesauro

et al. 2020

IJERPH

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Background: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease of the motor neurons. The etiology of ALS remains largely unknown, particularly with reference to the potential environmental determinants. Methods: We performed a population-based case-control study in four provinces from both Northern and Southern Italy in order to assess non-genetic ALS risk factors by collecting through tailored questionnaires information about clinical and lifestyle factors. We estimated ALS risk by calculating odds ratio (OR) with its 95% confidence interval (CI) using unconditional logistic regression models adjusted for sex, age and educational attainment. Results: We recruited 230 participants (95 cases and 135 controls). We found a possible positive association of ALS risk with trauma, particularly head trauma (OR = 2.61, 95% CI 1.19–5.72), electric shock (OR = 2.09, 95% CI 0.62–7.06), and some sports, although at a competitive level only. In addition, our results suggest an increased risk for subjects reporting use of private wells for drinking water (OR = 1.38, 95% CI 0.73–2.27) and for use of herbicides during gardening (OR = 1.95, 95% CI 0.88–2.27). Conversely, there was a suggestion of an inverse association with overall fish consumption (OR = 0.27, 95% CI 0.12–0.60), but with no dose-response relation. Consumption of some dietary supplements, namely those containing amino acids and, in the Southern Italy population, vitamins and minerals such as selenium, seemed associated with a statistically imprecise increased risk. Conclusions: Our results suggest a potential etiologic role a number of clinical and lifestyle factors with ALS risk. However, caution is needed due to some study limitations. These include the small sample size and the low number of exposed subjects, which affect statistical precision of risk estimates, the potential for exposure misclassification, and the uncertainties about mechanisms underpinning the possible association between these factors and disease risk.

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“…There is no causal therapy for amyotrophic lateral sclerosis (ALS) – a rare, fatal, progressive disease that has been rapidly increasing [ 1 ] and occurs de novo in 90% [ 2 ]. The studies using iPSC-derived motor neurons revealed the complicated molecular nature of this disease related to mitochondrial dysfunction and ER stress, NF aggregate formation, hyperexcitability, and channel deficits [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Umbilical Cord Mesenchymal Stem Cells in Amyotrophic Lateral Sclerosis: an Original Study

Barczewska

Maksymowicz

Zdolińska-Malinowska

et al. 2020

Stem Cell Rev and Rep

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Objective Amyotrophic lateral sclerosis (ALS) is still incurable. Although different therapies can affect the health and survival of patients. Our aim is to evaluate the effect of umbilical mesenchymal stem cells administrated intrathecally to patients with amyotrophic lateral sclerosis on disability development and survival. Methods This case-control study involved 67 patients treated with Wharton’s jelly mesenchymal stem cells (WJ-MSC). The treated patients were paired with 67 reference patients from the PRO-ACT database which contains patient records from 23 ALS clinical studies (phase 2/3). Patients in the treatment and reference groups were fully matched in terms of race, sex, onset of symptoms (bulbar/spinal), FT9 disease stage at the beginning of therapy and concomitant amyotrophic lateral sclerosis medications. Progression rates prior to treatment varied within a range of ± 0.5 points. All patients received three intrathecal injections of Wharton’s jelly-derived mesenchymal stem cells every two months at a dose of 30 × 10 6 cells. Patients were assessed using the ALSFRS-R scale. Survival times were followed-up until March 2020. Results Median survival time increased two-fold in all groups. In terms of progression, three response types measured in ALSFRS-R were observed: decreased progression rate ( n = 21, 31.3%), no change in progression rate ( n = 33, 49.3%) and increased progression rate ( n = 13, 19.4%). Risk-benefit ratios were favorable in all groups. No serious adverse drug reactions were observed. Interpretation Wharton’s jelly-derived mesenchymal stem cells therapy is safe and effective in some ALS patients, regardless of the clinical features and demographic factors excluding sex. The female sex and a good therapeutic response to the first administration are significant predictors of efficacy following further administrations. Graphical Abstract Medical therapeutic experiment with retrospective case-control analyses Electronic supplementary material The online version of this article (10.1007/s12015-020-10016-7) contains supplementary material, which is available to authorized users.

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Secular Trends of Amyotrophic Lateral Sclerosis

Cited by 90 publications

References 36 publications

The Characteristics of Cognitive Impairment in ALS Patients Depend on the Lateralization of Motor Damage

The Characteristics of Cognitive Impairment in ALS Patients Depend on the Lateralization of Motor Damage

Clinical and Lifestyle Factors and Risk of Amyotrophic Lateral Sclerosis: A Population-Based Case-Control Study

Umbilical Cord Mesenchymal Stem Cells in Amyotrophic Lateral Sclerosis: an Original Study

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