Methodological Quality of Clinical Trials in Amyotrophic Lateral Sclerosis: A Systematic Review.

Background: More than 200 clinical trials have been performed worldwide in ALS so far, but no agents with substantial efficacy on disease progression have been found.

Objective: To describe the methodological quality of all clinical trials performed in ALS and published before December 31, 2022.

Methods: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta Analyses.

Placental Angiodysplasia: A New Sign for Prediction of Fetal Outcome?

The study of the placenta is of great importance, not only in the attempt to understand the etiopathogenesis of various maternal-fetal pathologies, but also in the attempt to understand whether it is possible to find the cause of pathological neonatal outcomes. On the other hand, abnormalities of blood vessel formation, such as angiodysplasias, have been poorly characterised in the literature, and there is a need for more studies investigating the potential impact on the fetus. In this paper, we retrospectively analysed 2063 placentas received at the Department of Pathology of the University of Bari 'Aldo Moro', among which we identified 70 placentas affected by angiodysplasia.

Do changes in SSEP amplitude over time predict the outcome of comatose survivors of cardiac arrest?

Aim: To assess if the amplitude of the N20 wave (N20Amp) of somatosensory evoked potentials (SSEPs) changes between 12-24 h and 72 h from the return of spontaneous circulation (ROSC) after cardiac arrest and if an N20Amp decrease predicts poor neurological outcome (CPC 3-5) at six months.

Setting: Retrospective analysis of the ProNeCA multicentre prognostication study dataset. (NCT03849911).

Trends in survival of ALS from a population-based registry.

To assess survival of ALS patients in general and in selected demographic and clinical subgroups comparing two periods (1998-2000 vs. 2008-2010). Newly diagnosed adults resident of Lombardy, Northern Italy from a population-based registry were included.

ALS incidence and population aging in Northern Italy.

: Data on and increase of the incidence of amyotrophic lateral sclerosis (ALS) are conflicting and reflect the use of differing populations and designs. We investigated the incidence of ALS using data from a large population-based registry (SLALOM) in two three-year periods using the same diagnostic criteria and equal methods of case ascertainment. : The registry is based in Lombardy, a 10 million population area of Northern Italy.

SSEP amplitude accurately predicts both good and poor neurological outcome early after cardiac arrest; a post-hoc analysis of the ProNeCA multicentre study.

Aim: To assess if, in comatose resuscitated patients, the amplitude of the N20 wave (N20amp) of somatosensory evoked potentials (SSEP) can predict 6-months neurological outcome.

Setting: Multicentre study in 13 Italian intensive care units.

Methods: The N20amp in microvolts (μV) was measured at 12 h, 24 h, and 72 h from cardiac arrest, along with pupillary reflex (PLR) and a 30-min EEG classified according to the ACNS terminology.

Neurophysiology for predicting good and poor neurological outcome at 12 and 72 h after cardiac arrest: The ProNeCA multicentre prospective study.

Aims: To assess the accuracy of electroencephalogram (EEG) and somatosensory evoked potentials (SEPs) recorded at 12 and 72 h from resuscitation for predicting six-months neurological outcome in patients who are comatose after cardiac arrest.

Methods: Prospective multicentre prognostication study. EEG was classified according to the American Clinical Neurophysiology Society terminology.

Trauma and amyotrophic lateral sclerosis: a european population-based case-control study from the EURALS consortium.

Objectives: To assess the association between amyotrophic lateral sclerosis (ALS) and previous traumatic events, age of trauma, and site of injury.

Methods: A population-based case-control study was performed in five European countries (Italy, Ireland, France, United Kingdom, Serbia). Newly diagnosed ALS patients and matched controls were interviewed to collect relevant demographic factors and exposures.

Bone Regeneration Mediated by a Bioactive and Biodegradable Extracellular Matrix-Like Hydrogel Based on Elastin-Like Recombinamers.

The morbidity of bone fractures and defects is steadily increasing due to changes in the age pyramid. As such, novel biomaterials that are able to promote the healing and regeneration of injured bones are needed to overcome the limitations of auto-, allo-, and xenografts, while providing a ready-to-use product that may help to minimize surgical invasiveness and duration. In this regard, recombinant biomaterials, such as elastin-like recombinamers (ELRs), are very promising as their design can be tailored by genetic engineering, thus allowing scalable production and batch-to-batch consistency, among others.

Emerging therapy in arthritis: Modulation of markers of the inflammatory process.

The induction of tolerance has been proposed as a therapeutic strategy for arthritis aiming to decrease progression of the pathology, probably by promoting suppressor mechanisms of the autoimmune response. This work aimed to confirm whether the treatment with vitamin D3 could synergize oral tolerance induced by hydrolyzed collagen peptides, in our experimental model of antigen induced arthritis in New Zealand rabbits. Clinical observation of the phenomenon indicates that simultaneous treatment with hydrolyzed collagen peptides and vitamin D3 was beneficial when compared with no treatment, for arthritic animals, and for arthritic animals that received treatment with only hydrolyzed collagen peptides or vitamin D3.

Characterization of Hybrid Bioactive Glass-polyvinyl Alcohol Scaffolds Containing a PTHrP-derived Pentapeptide as Implants for Tissue Engineering Applications.

Hybrid foam (BG-PVA) with 50 % Bioactive glass (BG) and 50 % polyvinyl alcohol (PVA) was prepared by sol-gel process to produce scaffolds for bone tissue engineering. The pore structure of hydrated foams was evaluated by 3-D confocal microscopy, confirming 70% porosity and interconnected macroporous network. In this study, we assessed the putative advantage of coating with osteostatin pentapeptide into BG-PVA hybrid scaffolds to improve their bioactivity.

Physical activity and amyotrophic lateral sclerosis: a European population-based case-control study.

Objective: To assess whether physical activity is a risk factor for amyotrophic lateral sclerosis (ALS).

Methods: From February 2008 to April 2012, 652 patients with ALS from European population-based registries (France, Ireland, Italy, United Kingdom, Serbia) and 1,166 population controls (matched for age, sex, and residency) were assessed. Upon direct interview, data were collected on occupation and history of sport and leisure activities, physical activity, and accidental injuries.

Knowledge and attitudes towards epilepsy in Zambia: a questionnaire survey.

Misconception and stigma towards epilepsy have a profound impact on this disease in Africa. An unselected sample of Zambian people was interviewed to investigate their knowledge and attitudes towards epilepsy. Proper/improper answers were scored, and a composite score was developed with negative values for unsatisfactory awareness and high stigma levels.

Trauma and amyotrophic lateral sclerosis: a case-control study from a population-based registry.

Background And Purpose: Published reports on the association between amyotrophic lateral sclerosis (ALS) and trauma are controversial suggesting the need for a new case-control study done in a large population.

Methods: A case-control study was undertaken in Italy to assess this association. Cases were patients with newly diagnosed ALS from four population-based registries.

Outcome measures and prognostic indicators in patients with amyotrophic lateral sclerosis.

The purpose of the study was to assess frequency and predictors of disability measures in ALS. One hundred and fourteen newly diagnosed patients resident in eight administrative districts of Lombardy, Italy (population 4,947,554), included in a population-based registry, were followed for 2570 person-months (mean 22.5 months).

Incidence of ALS in Lombardy, Italy.

Objective: To assess the incidence and trends of ALS in a large population at risk.

Methods: This study was performed in nine provinces of Lombardy in Northern Italy (population 4,947,554). Patients with newly diagnosed ALS were enrolled during the period 1998 to 2002 through a prospective regional register.

Prevalence and characteristics of peripheral neuropathy in hepatitis C virus population.

Objective: To assess the prevalence of peripheral neuropathy (PN) and its correlation with cryoglobulinemia (CG) in an unselected, untreated referral hepatitis C virus (HCV) population.

Patients And Methods: Two hundred and thirty four patients (120 women and 114 men) with untreated HCV infection were consecutively enrolled by seven Italian centres. Clinical neuropathy was diagnosed when symptoms and signs of peripheral sensory or motor involvement were present.

Survival of patients with amyotrophic lateral sclerosis in a population-based registry.

Objective: To evaluate the survival of patients with amyotrophic lateral sclerosis (ALS) in an Italian population and to assess the effects of selected prognostic indicators on survival.

Background: Median survival of ALS patients has been reported to range between 12 and 23 months from diagnosis and between 23 and 36 months from onset of symptoms. Although several negative prognostic factors have been identified, the overall picture still needs clarification.

Predictors of epilepsy surgery outcome: a meta-analysis.

The potential efficacy of temporal and extratemporal resection in patients with partial epilepsy uncontrolled by anti-epileptic drugs is undisputed. However, there are still uncertainties about which patients will benefit most. A systematic review of the available literature has been undertaken by four pairs of reviewers to assess the overall outcome of epilepsy surgery and to identify factors better correlated to seizure outcome.

Executive dysfunction and avoidant personality trait in myotonic dystrophy type 1 (DM-1) and in proximal myotonic myopathy (PROMM/DM-2).

A previous study in proximal myotonic myopathy (PROMM/DM-2) and myotonic dystrophy type 1 (DM-1) using brain positron emission tomography demonstrated a reduced cerebral blood flow in the frontal and temporal regions associated with cognitive impairment. The objective was to investigate further cognitive and behavioural aspects in a new series of patients with DM-1 and PROMM/DM-2. Nineteen patients with genetically determined PROMM/DM-2 and 21 patients with moderately severe DM-1 underwent neuropsychological testing and neuropsychiatric interviews.

Reliability of the El Escorial diagnostic criteria for amyotrophic lateral sclerosis.

Background: The El Escorial diagnostic criteria are the most commonly used in clinical studies and therapeutic trials in patients with amyotrophic lateral sclerosis (ALS). The accuracy of the El Escorial criteria was tested in clinical practice, but the reliability is unknown when the diagnosis of ALS must be assessed on the basis of medical records.

Objective: To assess the reliability of the El Escorial criteria for the diagnosis of ALS in different settings.

Validity of hospital discharge diagnoses for the assessment of the prevalence and incidence of amyotrophic lateral sclerosis.

Introduction: As amyotrophic lateral sclerosis (ALS) is a rare neurological disorder, many sources must be investigated for complete case ascertainment. Hospital discharge diagnoses (HDDs) are a source of administrative data for patients hospitalized for a given disease. The accuracy of HDDs was tested, to assess whether they could be used to trace patients with amyotrophic lateral sclerosis (ALS).

Proximal myotonic myopathy: report on italian families and literature review.

Despite the more recent genetic classification of the novel myotonic dystrophies into at least two distinct myotome dystrophy (DM) loci (DM1, DM2/PROMM/PDM), the clinical characteristics of these dominant myotonic syndromes often blur. The multisystemic nature of these disorders leads to a spectrum of symptoms and signs th.it emphasize the similarities and differences between these diseases.

[Asymptomatic bilateral occipital calcifications. A possible atypical form of Sturge-Weber-Krabbe syndrome?].

Sturge-Weber-Krabbe syndrome is a rare congenital neuro-cutaneous disease which is characterized, in its full clinical expression, by facial naevus flammeus and epilepsy with mental retardation. Different atypical cases in which one or more symptoms are missing and clinical course is benign, have been reported in the literature in recent years. In the present paper a possible case of this syndrome radiologically diagnosed, without neurological and cutaneous symptoms, is reported; the importance of a more comprehensive study of this complex disorder, with particular attention to the incomplete forms, is stressed.