Opsoclonus-Myoclonus Syndrome in Children and Adolescents:A Therapeutic Challenge

Opsoclonus-myoclonus syndrome (OMS) is a neurological non-fatal disease that usually responds to immunotherapies. However, the real challenge is to counteract the high frequency of relapses and long-term developmental sequelae. Since the OMS is extremely rare, a common consensus regarding therapeutic guidelines is still lacking. The goals of this study were to test whether ACTH was superior to other immunotherapies and to investigate whether an early treatment could improve the outcome. Sixteen children affected by OMS were retrospectively reviewed. Eight children had a neuroblastic tumor. The other eight patients were affected by non-paraneoplastic OMS. Overall, the most commonly used treatment was corticotherapy (n = 11). However, ACTH (n = 10), rituximab (n = 7), immunoglobulins (n = 4), cyclophosphamide (n = 3), and mycophenolate (n = 2) were also administered. ACTH was associated with a high percentage of patients who healed (80%) and, as a first-line therapy, was associated with a lower incidence of relapses. An early treatment was associated with a favorable long-term outcome. Long-term sequelae occurred in 42% of patients who were treated early and in all of those who were treated late. It is advisable for the affected children to be identified at an early time, as they may benefit from an early treatment. ACTH represents an effective treatment with a high probability of recovery and low rate of relapses

Early alterations of cortical thickness and gyrification in migraine without aura:a retrospective MRI study in pediatric patients

BACKGROUND: Migraine is the most common neurological disease, with high social-economical burden. Although there is growing evidence of brain structural and functional abnormalities in patients with migraine, few studies have been conducted on children and no studies investigating cortical gyrification have been conducted on pediatric patients affected by migraine without aura. METHODS: Seventy-two pediatric patients affected by migraine without aura and eighty-two controls aged between 6 and 18 were retrospectively recruited with the following inclusion criteria: MRI exam showing no morphological or signal abnormalities, no systemic comorbidities, no abnormal neurological examination. Cortical thickness (CT) and local gyrification index (LGI) were obtained through a dedicated algorithm, consisting of a combination of voxel-based and surface-based morphometric techniques. The statistical analysis was performed separately on CT and LGI between: patients and controls; subgroups of controls and subgroups of patients. RESULTS: Patients showed a decreased LGI in the left superior parietal lobule and in the supramarginal gyrus, compared to controls. Female patients presented a decreased LGI in the right superior, middle and transverse temporal gyri, right postcentral gyrus and supramarginal gyrus compared to male patients. Compared to migraine patients younger than 12 years, the ≥ 12-year-old subjects showed a decreased CT in the superior and middle frontal gyri, pre- and post-central cortex, paracentral lobule, superior and transverse temporal gyri, supramarginal gyrus and posterior insula. Migraine patients experiencing nausea and/or vomiting during headache attacks presented an increased CT in the pars opercularis of the left inferior frontal gyrus. CONCLUSIONS: Differences in CT and LGI in patients affected by migraine without aura may suggest the presence of congenital and acquired abnormalities in migraine and that migraine might represent a vast spectrum of different entities. In particular, ≥ 12-year-old pediatric patients showed a decreased CT in areas related to the executive function and nociceptive networks compared to younger patients, while female patients compared to males showed a decreased CT of the auditory cortex compared to males. Therefore, early and tailored therapies are paramount to obtain migraine control, prevent cerebral reduction of cortical thickness and preserve executive function and nociception networks to ensure a high quality of life

Children under 6 years with acute headache in Pediatric Emergency Departments. A 2-year retrospective exploratory multicenter Italian study

Background: Preschool age (i.e. children under six years of age) represents a red flag for requiring neuroimaging to exclude secondary potentially urgent intracranial conditions (PUIC) in patients with acute headache. We investigated the clinical characteristics of preschoolers with headache to identify the features associated with a greater risk of secondary "dangerous" headache. Methods: We performed a multicenter exploratory retrospective study in Italy from January 2017 to December 2018. Preschoolers with new-onset non-traumatic headache admitted to emergency department were included and were subsequently divided into two groups: hospitalized and discharged. Among hospitalized patients, we investigated the characteristics linked to potentially urgent intracranial conditions. Results: We included 1455 preschoolers with acute headache. Vomiting, ocular motility disorders, ataxia, presence of neurological symptoms and signs, torticollis and nocturnal awakening were significantly associated to hospitalization. Among the 95 hospitalized patients, 34 (2.3%) had potentially urgent intracranial conditions and more frequently they had neurological symptoms and signs, papilledema, ataxia, cranial nerves paralysis, nocturnal awakening and vomiting. Nevertheless, on multivariable logistic regression analysis, we found that only ataxia and vomiting were associated with potentially urgent intracranial conditions. Conclusion: Our study identified clinical features that should be carefully evaluated in the emergency department in order to obtain a prompt diagnosis and treatment of potentially urgent intracranial conditions. The prevalence of potentially urgent intracranial conditions was low in the emergency department, which may suggest that age under six should not be considered an important risk factor for malignant causes as previously thought

Arsenic levels in milk and mozzarella cheese from Lazio

"Arsenic is a natural occurring element and its levels in foods generally reflect normal accumulation from the environment. In the past, the main use of arsenic was in the chemical industry and it was widely employed in pharmaceutical and agricultural chemicals, preservatives and related compounds. Arsenic is a persistent poison and it can easily enter the human food chain. Arsenic content in the manufacturing process water seems to be of special importance because it determines if the concentrations in the food may be considerably higher or lower compared to the raw materials. For this reason, it is desirable that all relevant informations is provided about the food to enable a realistic toxicological assessment on dietary exposure to arsenic. In this study, total arsenic concentration was assessed spectrophotometrically in process water, in milk and in cheese samples from Lazio. The data of arsenic analysis in mozzarella are presented and discussed. Total arsenic concentrations measured in certain samples of cheese are quite high and could be of potential hazard for human health, especially in case of high consumption of these products.

The importance of life cycle assessment for environmental sustainability in mozzarella cheese sector: an empirical analysis

"The environmental planning and the current European policy initiatives are aimed at continuous improvement of the productive fabric in a sustainable, heading toward a system in which the "quality" of products will no longer regardless of their "environmental quality". Through a LCA is therefore possible to identify the stages where some of the most critical environmental and information needed to implement the improvement. Aim of this study is to highlight critical points and possible strategies for improvement in the process of production and marketing of the milk product and, specifically, in the production of buffalo mozzarella cheese. The results show the impacts of the production throughout its life cycle but, in particular, the characterization shows a very high Global Warming Potential, and energy consumption attributable to primary production

“Eco -innovation and green economy: the role of sustainability life cycle assessment”

"In recent years, economic growth has been accompanied by growing sensitivity to environmental problems, the problem of energy security and scarcity of nonrenewable natural resources. Innovation has been seen as central to economic performance and social welfare; it is increasingly recognized as a significant driver of economic. This is the context of the life cycle thinking. As we know, Life cycle thinking and Life Cycle Assessment are scientific approaches behind a growing number of environmental policies and business decision support in the context of Sustainability consumption and Production (SPC). The environmental base is supported by Life Cycle Assessment by the international standardized tool (ISO 14040 and ISO 14044) for integrated assessment for goods and services. Sustainability Life Cycle Assessment is a set of tools to analyze the sustainability of a business under different economic, social and environmental. The tools of sustainability can be considered tools of organizational innovation and management to support business decisions. The aims of this work is to give an overview about the current state of sustainability policies implemented in Italian companies emphasizing the prospects for future application. The analysis was carried out comparing the performance data collected through the company accounts of a sample of companies by comparing the performance improvements after the application of LCA and developing forecasts of performance improvement after applying the tools of sustainability.

Arsenic contamination in the agricultural food-chain of mozzarella in Lazio region (Italy)

"\"Arsenic is a naturally occurring element and its levels in foods generally reflect normal accumulation from the environment.. . In the past, the main use of arsenic was in the chemical industry and it was widely employed in pharmaceutical and agricultural chemicals, preservatives and related compounds. Arsenic is a persistent poison and it can easily enter the human food chain .. . The arsenic content in the manufacturing process water seems to be of special importance because it determines if the concentrations in the food may be considerably higher or lower compared to the raw materials. For this reason, it is desirable that all relevant information is provided about the food to enable a realistic toxicological assessment of dietary exposure to arsenic.. . In the present study, total arsenic concentration was assessed spectrophotometrically in process water, in milk and in cheese samples from Lazio. The data of arsenic analysis in mozzarella are presented and discussed. Total arsenic concentrations measured in certain samples of cheese are quite high and could be of potential hazard for human health, especially in case of high consumption of these products.\"

Clinical and neuroimaging characteristics of MOG autoimmunity in children with acquired demyelinating syndromes

: Background Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been recently reevaluated as a biomarker of acquired demyelinating syndromes (ADS) of the central nervous system (CNS). Here, we describe the clinical and neuroimaging features, and the long-term outcome of children with ADS of the CNS associated with MOG-IgG. Methods All patients underwent brain and spinal cord magnetic resonance imaging (MRI), lumbar puncture for cerebrospinal fluid (CSF) analysis and MOG-IgG and aquaporin-4 IgG (AQP4-IgG) testing. Results Forty-eight pediatric patients were recruited. MOG-IgG were detected in 11/48 (25%) patients with the following clinical presentations: encephalomyelitis (EM), 8/11 (73%); optic neuritis (ON), 2/11 (18%); transverse myelitis (TM), 1/11 (9%). Patients negative for MOG-IgG were diagnosed with Multiple Sclerosis (MS) (n=15), EM (n=7), ON (n=7), neuromyelitis optica spectrum disorders (NMOSD) (n=5), TM (n=2) and encephalitis (n=1). MOG-IgG positive patients were younger at disease onset and they more frequently experienced encephalopathy and epileptic seizures compared with negative patients. EM and inflammatory lesions involving optic nerves on MRI imaging were more frequent in MOG-IgG positive patients. None of the patients with MOG-IgG became persistently seronegative during the follow-up, although a decrease in MOG-IgG titer was observed. Patients with MOG-IgG showed a good response to therapy and only two patients presented relapses during follow-up. Conclusion This study supports the distinction of MOG autoimmune oligodendrocytopathy as a unique disease entity, with clinical features different from those of MS and AQP4-IgG-positive NMOSD

Truths and Myths in Pediatric Migraine and Nutrition

The link between migraine and nutrition can be explored from several points of view. Lifestyle and, in particular, aspects of nutrition can have a significant impact on the course of pediatric migraine. In addition, some dietary treatments, such as the ketogenic diet, and some active ingredients present in foods (nutraceuticals) may have a therapeutic effect on migraine. A diet that can control weight gain and obesity has beneficial effects on migraine severity. On the other hand, when we talk about the link between nutrition and headaches, it is also necessary to point out that some public information is actually fake news that has no scientific basis. The purpose of this review is to provide an update on the salient points linking pediatric migraine to nutritional principles, focusing on the relationship between weight and headaches, the therapeutic effect of food for medical purposes, the ketogenic diet as a migraine treatment, and the relationship between migraine and dietary habits

Features and Management of New Daily Persistent Headache in Developmental-Age Patients

Introduction. Our aim was to investigate the clinical features of primary new daily persistent headache (NDPH) in a cohort of paediatric patients. Methods. We reviewed the data of patients with persistent daily headache, attending the Headache Centre of Bambino Gesu Children from the January 2009. The ICHD-III criteria were used for diagnosis. Statistical analysis was conducted to study possible correlations between NDPH and population features (age and sex), NDPH and headache qualitative features, and NDPH and response to pharmacological therapies. Results. We included 46 subjects with NDPH. The features of pain more closely resembled those of migraine than to those of tension-type headache (62 vs. 38%). The NDPH patients showed nausea and vomiting less frequently than migraine ones (28.6 vs. 48.2%, p < 0.01). A total of 75% of NDPH patients experienced an onset of the symptoms in the winter months (November to February) (p < 0.01). NDPH was less common in very young children under 10 years of age. Almost 58% of NDPH patients received pharmacological therapy and the most used drug was amitriptyline. A reduction of attacks by at least 50% in a month was detected in 30.6% of patients. Conclusions. NDPH can be very disabling and correlates with seasonal factors. Although long term pharmacological therapy is recommended, considering the long duration that this headache can have, there are no data supporting the treatment choice