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Salt crystallization is one of the harshest deterioration mechanisms affecting heritage materials, causing impressive decay patterns and the loss of a high thickness of original materials. Although salt damage has been widely investigated in the literature from the theoretical and experimental points of view, the solutions to mitigate this problem are still extremely limited. In the present paper, a new biopolymeric treatment based on chitosan was tested on two kinds of porous limestones widely used in historic architecture, aiming at inhibiting the crystallization of sodium sulphate inside the stone and promoting the formation of salt efflorescence over the surface, rather than harmful subflorescence inside the pore network. The treatment was applied to the bare stone and also after an inorganic pre-treatment based on the formation of hydroxyapatite in the stone. Hydroxyapatite was recently proposed for the consolidation and protection of carbonate stones and here it is expected to provide an effective anchoring layer for the chitosan coating on the pores surface, and also to prevent the calcite washout from the stone and hence the removal of chitosan. The effect of hydroxyapatite alone was also tested, for comparison’s sake. Treated and untreated stone specimens were subjected to two different accelerated salt crystallization tests, one based on crystallization cycles (wetting-drying cycles) and the other one based on continuous capillary absorption of a saline solution (“wick effect”), evaluating the results in terms of weight loss, efflorescence formation, and changes in porosity and mechanical properties. The results showed that all the treatments are compatible with the stones, and the combined treatment (hydroxyapatite + chitosan) is extremely promising for the prevention of salt damage.

Purpose: Pervasive refusal syndrome (PRS) is a rare psychiatric disease that affects children. It was first described by Lask in 1991 (Arch Dis Child 66:866–869, 1991). Recently, Otasowie and Collaborators reported a systematic review about PRS. Despite this, PRS has not yet been classified in DSM-5 and ICD-11 and the lack of evidence-based treatment makes this syndrome a real challenge for clinicians. The aim of this paper is to present our experience through the description of a case report and its treatment. Methods and results: The case reported is a girl aged 11years that fits the clinical picture described in the literature of PRS. In previous reports, behavioural treatment was not used or appreciated; our case adds new knowledge regarding the PRS diagnosis and the successful behavioural treatment during hospitalization, which we describe in all its phases. Conclusion: PRS is a rare, life-threatening syndrome; it would be extremely important to have an official and evidence-based treatment guide. Level of evidence: Level V, case report.

This study aimed at evaluating the effectiveness and the compatibility of two alternative treatments, in view of their possible use for conservation of prehistoric paintings in the Magura cave (Bulgaria). The paintings are made of bat guano applied over limestone, therefore, two sets of laboratory specimens were considered: stone specimens and stone specimens covered with a layer of sterilized bat guano. The two investigated treatments were a commercial product based on ethyl silicate (&ldquo, ES&rdquo, ) and a solution of diammonium hydrogen phosphate (&ldquo, DAP&rdquo, ), aimed at forming calcium phosphates. The results of the study indicated that both treatments were able to increase the mechanical properties of stone, the increase being higher for &ldquo, Both consolidants caused acceptable color changes, but the &ldquo, treatment significantly decreased stone wettability, water absorption, and water vapor permeability, while the &ldquo, treatment slightly affected those properties. In the stone + guano specimens, the presence of the guano layer affected the penetration of the consolidants, thus partly reducing their effectiveness. Compared to the stone samples, the guano layer experienced a more intense color change, alongside visible cracking. However, the adopted methodology to replicate the cave paintings was not completely successful, as the so-deposited guano layer was very prone to detachment when dry, unlike cave paintings. Future work will be dedicated to assessing the consolidant performance onto samples that resemble even more closely the conditions of the cave paintings, by improving the methodology for the guano layer deposition and by contaminating specimens with soluble salts before consolidant application.

Purpose: The aim of this study is to describe demographic data, semiology and etiology in a pediatric population with status epilepticus (SE) and refractory SE (RSE). Method: We retrospectively reviewed patients with the following inclusion criteria: i) age between two months and eighteen years; ii) SE diagnosis; iii) admission from January 2001 to December 2016; iv) available clinical data. Results: We enrolled 124 patients. Mean and median age was 4.6 ± 4.2 years and 3.3 [1.2-7.5] years respectively. SE had a “de novo” onset in 66.9%. Focal convulsive-SE was the most common semiology (50.8%) whilst generalised (32.3%) and nonconvulsive-SE (NCSE) (16.9%) were less represented. Some etiologies showed a different age distribution: febrile in youngest age (p = 0.002, phi 0.3) and idiopathic-cryptogenic in older children (p = 0.016, phi 0.2). A statistical significance correlation was detected between semiology and etiology (p < 0.001, Cramer's V 0.4), chemotherapy and NCSE (n = 6/21 vs 3/103, p < 0.001) as well as PRES and NCSE (n = 7/21 vs 5/103, p < 0.001). Only 17.7% had a RSE. No correlation was found in demographic and clinical data, but NCSE, acute and idiopathic-cryptogenic etiologies were more frequently associated to RSE. Encephalitis was the most common diagnosis in acute etiologies whereas unknown epilepsy in idiopathic-cryptogenic group. Conclusion: Most of our findings were previously described however we found a significant role of non-antiepileptic treatments (chemotherapy-dialysis) and comorbidity (PRES) determining acute etiology and NCSE. Acute (mostly encephalitis), idiopathic-cryptogenic (mainly unknown-epilepsy) and NCSE were frequently detected in RSE. In the above mentioned conditions a high level of suspicion was recommended.

The HAP4MARBLE project was funded by the European Commission within the call H2020-MSCA-IF-2014 of the Marie Skłodowska-Curie Actions (Grant Agreement n. 655239). The goal of the project was to develop an innovative conservation treatment for marble artworks exposed outdoors, which suffer from several deterioration processes predicted to be worsened by future climate change.

Background The coronavirus disease 2019 (COVID-19) is currently rare in children and they seem to have a milder disease course and better prognosis than adults. However, SARS-Cov-2 pandemic has indirectly caused problems in pediatric medical assistance. In view of this we wanted to draw a picture of what happened during health emergency and analyze future prospects for restarting. Methods We involved the Italian pediatric scientific societies institutionally collected in the Italian Federation of Associations and Scientific Societies of the Pediatric Area (FIARPED); We sent a questionnaire to all scientific societies about the pediatric care activity during the COVID-19 emergency and future perspectives for the phase of post-containment. Results The analysis of the questionnaires showed significant decrease of:admission, outpatient visits and specialist consultancy activities during the COVID-19 emergency, primarily linked to the fear of infection. Instead it was increased the serious degree of diseases admitted. Most of scientific societies maintained the relationship with chronic patients through some form of telemedicine, reporting a strong positive opinion about this modality. Finally showed the need to give life a new approach for hospitalizations and outpatient visits through a greater use of telemedicine, educational programs on families and a more decisive role of family pediatricians. Conclusions Our study highlighted many aspects that can be improved in pediatric care. We think that It will be necessary a new shared strategy to improve the management and continuity of care for pediatric patients, primarily developing a network of collaboration between families, family pediatrician and hospitals and by enhancing the use of new methods of telecommunications.

The present study tackles the problem of a sustainable and efficient conservation of cave art, by using innovative materials and techniques for the different steps of the restoration process - Biodeactivation, Biocolonization prevention and Consolidation. The Magura cave in northwest Bulgaria is the case study. It contains an impressive display of prehistoric paintings made of guano as far back as 5’500 years ago. In the last forty years the cave suffered progressive microbial colonization. The detrimental effects are biofilm formation, physical penetration into the stone and chemical reaction with the stone/paintings by pigments. Therefore, as a first step, we investigated biodeactivation by non-thermal plasma sterilization. The oxidative atmosphere obtained introducing Ar/O2 (0.2 and 0.1) in the plasma device, was carried out on lab samples inoculated with the targeted for Magura Cave microorganisms. The main advantage of the non-contact treatment with atmospheric pressure plasma (APP) is the lack of any mechanical and chemical modification of the underlying stone/guano layers. As for sterilization of wounds on human skin the plasma treatment on wet surfaces produces mainly hydrogen peroxide and nitrates which lead to a localized reduction of the pH. The obtained biodeactivation is assured without heat (< 40 °C), toxic and environmentally harmful liquid. In a second step, we tested two possible alternatives for consolidation of the cave. A commercial ethyl silicate (ES) product was compared with an innovative phosphate treatment, based on application of a hydro-alcoholic solution of a phosphate salt (diammonium hydrogen phosphate, DAP). The consolidation efficiency and compatibility of the ES and DAP consolidants were investigated on samples representative for the Magura Cave substrate, i.e. stone alone and stone covered with guano to resemble the prehistoric drawings. In addition, a combination of plasma activation of the stone surface and consolidation was tested, to investigate whether the two treatments may have a synergistic effect, thus making the combined treatment more efficient than consolidation alone.

The present study investigates the strengthening ability and the compatibility of two different ammonium phosphate solutions used for consolidation of mortars based on slaked lime, natural hydraulic lime or cement, to resemble historic and modern mortars. After impregnation for 24 h, new calcium phosphate phases were detected in all types of mortar. New calcium phosphates induced significant mechanical consolidation, with only slight alterations in the pore system and in water transport properties. In lime-based mortars (initially white) no color alteration was visible after treatment, while colored mortars experienced some color change when the more concentrated phosphate solution was used.

The present study deals with the deterioration of materials in the dome of the “Woodpecker” dance club designed by the architect Filippo Monti (1928–2015) and built in Milano Marittima (near Ravenna, Italy) in 1968. This place, considered extremely valuable due to its landscape and architectural features, has been in a state of abandonment for around 40 years, but recently the local Municipality has started a process for its restoration. The dome is made of 23 fibreglass precast rib vaults hiding a steel structure, and in early 2000s a large part of its internal surface was painted with a graffito by Blu, an internationally renowned Italian street artist. Nowadays, both the dome and the graffito are safeguarded for their historic-architectural-artistic value by putting restrictions to their demolishment and modification. However, both the dome and the graffito are affected by harsh kinds of degradation, very different from those affecting historical building materials, which endanger their state of preservation. This study investigates whether the methodological approach of the conservative restoration can be applied to this structure, which is an example of contemporary architecture. Because of the lack of project drawings, a visual investigation was carried out to define geometric features and the most important technical details, and to deduce plausible hypotheses about building techniques that were adopted. The degradation affecting the dome and the graffito was then investigated, and samples of fibreglass, steel and paint were collected. Laboratory analyses on these samples, in addition to environmental studies concerning wind, ground water and relative humidity, were conducted to characterize the materials and identify their main deterioration processes. A possible restoration solution in accordance with conservative restoration principles was finally proposed, showing that the application of the same methodological approach used for ancient buildings arises many challenges, due to some key peculiarities of XX century architectural heritage materials.

Surface coatings made of hydroxyapatite (HAP) have been proposed to protect marble artworks from dissolution in rain, originated by the aqueous solubility of calcite. However, HAP coatings formed by wet chemistry exhibit incomplete coverage of marble surface, which results in limited protective efficacy. In this study, electrodeposition was explored as a new route to possibly form continuous coatings over the marble surface, leaving no bare areas. Electrodeposition was performed by placing marble samples in poultices containing the electrolyte (an aqueous solution with calcium and phosphate precursors) and the electrodes. The influence of several parameters was investigated, namely the role of the working electrode (cathode or anode), the distance between the marble sample and the working electrode, the deposition conditions (potentiostatic or galvanostatic), the electrolyte composition and concentration, the applied voltage, and time. The coating morphology and composition were assessed by SEM/EDS and FT-IR. The protective ability of the most promising formulations was then evaluated, in all cases comparing electrodeposition with traditional wet synthesis methods. The results of the study suggest that electrodeposition is able to accelerate and improve formation of HAP coatings over the marble surface, even though the obtained protective efficacy is not complete yet.

In recent years, steel reinforced polymer (SRP) composites have emerged as a new technology for structural strengthening, and several researches have validated the effectiveness of SRP for masonry strengthening. Research has been carried out to study the bond behavior of SRP composites applied to a masonry substrate. However, how the moist and salt on masonry surface will affect bond, which is the weak link in real strengthening applications, is little known yet. This study aims at investigating the bond behavior of SRP composites applied to moist and salt-laden masonry blocks that were subjected to an artificial weathering protocol. Single-lap shear tests were conducted to determine the bond behavior, while ion chromatography provided the salts distribution of weathered specimens to interpret some results of the shear tests.

It is well known that multi-leaf walls, cavity walls as well as rubble masonry are prone to brittle collapse mechanism due to weak transverse connections. Twisted steel bars are widely used to strengthen such kind of masonry buildings, in particular to improve the transverse connection between different structural elements. Due to the shape of the twisted bar, the connector works as a self-threading screw, anchoring to the substrate material without any binder. The effectiveness of such technique mainly relies on the bond between the bar and the substrate, which relies on mechanical interlocking and friction mechanisms. In this paper, an experimental study on the pull-out behavior of twisted steel connectors inserted in units of different materials (bricks and stones) that can be commonly found in existing masonry buildings is presented. Mechanical and microstructural characterization of the substrate materials was conducted to investigate the influence of some key parameters (compressive strength, tensile strength, elastic modulus as well as abrasion resistance) on the pull-off load of the connector. To get a preliminary insight on the structural behavior of such connectors in presence of different loading regimes, the bars were also subjected to two cycles of unloading/reloading. Additionally, a 3D scanner was employed to geometrically characterize the inner surface of the hole left by the bar after its extraction. Results showed that among the different mechanical and microstructural parameters investigated, the substrate compressive strength plays a key role in the pull-out behavior of the connectors.

The Life Cycle Assessment (LCA) represents a suitable methodology to evaluate quantitatively the environmental impact related to a product or a process and it can be used as a guiding tool to make effective environmental sustainability choices. While the LCA-based methods are more and more diffused in the assessment and selection of materials for new constructions, they are still scarcely applied in the field of conservation and repair of historical buildings, although these buildings, especially in Europe, represent a high percentage of the building stock. In the present paper, the LCA method was applied to the field of the restoration, with particular reference to cleaning technologies and materials, in order to investigate if LCA can be applied in this field. The analysis of results pointed out the different environmental impact of the cleaning methods investigated (ascribed to different impact categories), but also the shortcomings and proxies arising from the lack of specific database. The impact of the waste treatment stage was also analysed, in order to highlight the main impact spots related with the end of life of materials and equipment. Results showed that, for some cleaning methods, the impacts related to manufacturing and disposal are very similar, which emphasizes the importance of performing LCA including the end of life scenarios. Finally, an evaluation of externalities was performed, to provide a monetary value of the environmental damage.

Il contributo ricostruisce l’attuale lettura interpretativa e giurisprudenziale delle norme e delle questioni emergenti in materia di scomparsa, assenza e morte presunta.

Il commento analizza i poteri degli amministratori a seguito del verificarsi di una causa di scioglimento

L'autore affronta in modo originale la tematica del diritto di superficie e della proprietà superficiaria seguendo gli sviluppi della dottrina e della giurisprudenza ed apportando innovazioni interpretative in merito alla proprietà temporanea e alla proprietà separate

L'autore esamina tutte le obbligazioni pecuniarie, obbligazioni alternative, obbligazioni facoltative, obbligazioni solidali, obbligazioni indivisibili, obbligazioni divisibili, individuando i percorsi della dottrina e della giurisprudenza ed esprimendo per ogni istituto delle riflessioni originali e innovative.

Il commento analizza le modalità di nomina e revoca degli amministratori nonché i criteri di svolgimento della liquidazione e le conseguenze qualora gli amministratori omettano la convocazione prevista dalla norma

Il commento analizza gli obblighi degli amministratori in conseguenza del verificarsi di una causa di scioglimento

Analisi delle principali problematiche inerenti la pubblicità della nomina dei liquidatori e gli effetti della medesima

Il commento affronta le principali questioni problematiche in tema di cause di scioglimento delle società per azioni emerse tanto nel dibattito dottrinale quanto nelle applicazioni giurisprudenziali

Il contributo fornisce una ricostruzione delle questioni emergenti in materia di separazione legale dei coniugi alla luce della recente casistica giurisprudenziale di legittimità e di merito.

Given the high energy consumption connected to old buildings and their large environmental impact, there is a strong need for effective solutions for the building envelope retrofitting. Among these solutions, external thermal insulation composite systems (ETICS) have found large application in recent decades. In this paper a new kind of large-size thermal insulation composite boards, prefabricated using porcelain stoneware slab finishing, was developed. Different thermal insulating materials and adhesives, with and without glass fibre mesh, were tested by both current methodologies and purposely designed tests, in order to assess their physical–mechanical properties and durability performance, finally selecting the most suitable materials for the composite board. The strong points of this composite board are mainly: (i) its short placing time and improved execution quality, due to prefabrication; (ii) its high aesthetical value; (iii) its high durability, as the finishing layer is mostly insensible to weathering. The results highlight the good performances of the prefabricated composite board developed in this study (generally higher than current ETICS). The testing procedure followed in this study is also meant to give a contribution to the establishment of methodologies for the selection and durability assessment of materials for the building envelope retrofitting.

The use of ammonium phosphate solutions has proven to be very promising for protection and conservation of marble. However, all the studies carried out so far have been performed on uncontaminated marble. Unfortunately, this is rarely the case in the field, because marble artifacts exposed outdoors are often affected by sulfation, i. e. formation of a gypsum crust on the surface. Because gypsum is much more soluble than calcite, the outcome of the ammonium phosphate treatment is expected to be sensibly altered by the presence of gypsum. Therefore, in this study the nature and morphology of the new calcium phosphate phases formed by reacting gypsum with aqueous solutions of diammonium hydrogen phosphate (DAP) were investigated. In particular, the effect of DAP concentration, ethanol addition (aimed at reducing gypsum solubility), and pH were explored. The result is that phase formation can be controlled by suitably tuning the above mentioned parameters. Phases with low solubility (such as tricalcium phosphate and hydroxyapatite) can be obtained by increasing the ethanol concentration, the DAP concentration or the pH. However, their formation is associated with diffused cracking, likely because of excessive growth of the new phases. Among the investigated formulations, treatment with a 0.1 M DAP solution with 30 vol % ethanol at pH=8 seems to be the most suitable one, as it leads to formation of brushite (about 30 times less soluble than gypsum), without cracking, so that a reduction in gypsum solubility in rain is expected.

Salt weathering is among the most severe phenomena affecting porous limestone, often leading to the loss of precious material from historical sculptures and building facades. Together with exerting a weathering action, salts can also hamper the success of consolidation treatments, by interfering with consolidants’ penetration and/or curing reactions. On site, application of consolidants on non-contaminated stone is rarely feasible, especially in the case of porous limestones; for this reason, testing application of consolidants on salt laden stone is of particular relevance to guarantee their successful application in the field. In this paper, two hydroxyapatite (HAP)-based treatments have been applied to Lecce Stone, a very porous organogenic limestone, highly susceptible to salt weathering. The two treatments differ for DAP concentration, application procedure and presence/absence of ethanol in the formulation. Prior to treating, specimens were subjected to salt crystallization cycles in a sodium sulphate solution, to cause salt contamination and induce weathering. They were then desalinated, purposely leaving a percentage of salts in the stone (SO4 2- after desalination ~0.15-0.3 wt%). Phase formation and distribution as a result of different contamination levels were investigated, and the efficacy of the treatments in comparison to ethyl silicate was determined, as it is currently the most used consolidant for this lithotype. Finally, the durability of the most promising treatment to further salt crystallization was evaluated, again in comparison with ethyl silicate. Promising results were obtained, as the presence of sodium sulphate did not prevent the HAP- based consolidants from uniformly distributing in the stone, sealing cracks and forming insoluble phosphate phases. Interestingly, the application on non-desalinated stone revealed that the nature and the amount of the phases that form as a result of the treatment are dependent on the amount of salts in the stone. Finally, most promising HAP-based formulation was found to enhance the stone’s durability towards further salt crystallization, also compared to ethyl silicate.

Self-cleaning finishing treatments based on the use of TiO 2 nanoparticles have been recently proposed for the protection of materials in cultural heritage, with the aim of increasing the durability of these materials in polluted environments. The results on stone seem encouraging, but the experimental tests on different porous materials, such as bricks and renders used in historical buildings, are still extremely limited. In the present paper, the application of water dispersion of titania nanoparticles to the repair renders of the former Del Corso hotel (XVIII cent., Bologna, Italy) was evaluated. The building and its environment were first analysed for assessing the appropriateness of such finishing, then samples of painted renders identical to those provided for in the intervention work were manufactured and treated with the dispersion, both by brushing on the dry paint and by brushing on the wet paint (the latter procedure in order to enhance the particles retention by exploiting the mechanism of fresco paintings). The outcome of the different treatments was investigated mainly to evaluate its compatibility with the substrate and its durability, which could be of interest also for the application of photocatalytic finishing to renders in new constructions.

Old reinforced concrete (RC) structures often exhibit unsatisfactory performances in terms of both residual serviceability and seismic behavior. The durability requirements were not considered at all at the age of design and construction, exposing such structures to severe decay in aggressive urban and/or industrial environment; in the meantime, the use of plain steel bars (with poor bond strength) was common at the age of construction and this jeopardizes the RC performance under cyclic load reversals like those induced by earthquakes. In this paper, the deep impregnation of cement-based mortars and concrete with a solution of ethyl silicate and with electrochemical treatments by means of a solution of sodium carbonate was investigated, with a twofold aim: (1) increasing the bond strength of the embedded plain steel bars in existing structures without any need for demolition and (2) increasing the durability and safety of the reinforced concrete elements. Both the treatments are already exploited in other fields: ethyl silicate solutions are widely used for stone consolidation (and only recently proposed for concrete surface protection), while migration of sodium carbonate under DC voltage is used for RC realkalization. In the present study these treatments are expected to penetrate inside mortar and concrete and to modify their microstructure, hence improving their properties. The effects of the treatments are investigated by comparing the characteristics of treated and untreated mortar and concrete samples reinforced with plain bars, in terms of penetration inside the samples, pull-out strength, water absorption, pore size distribution, carbonation resistance and corrosion resistance.

Rising damp is one of the main problems affecting historical masonry structures all over the world, as it jeopardises materials integrity, inhabitants’ health and performances of the building envelope. Despite the great effort spent over last century to understand water capillary rise phenomenon, the problem of damp removal is still substantially open, as not only the technologies adopted in the field frequently fail, but their working principles in real masonries have not been fully elucidated yet. This paper aims at providing, through a review of international literature, a coherent picture of the technologies so far proposed for the removal of rising damp and the results obtained, both in laboratory and on-site. Based on the results, the open challenges for research in this field are tentatively pointed out, according to the author’s opinion.

Loss of function mutations in PAK3 contribute to non-syndromic X-linked intellectual disability (NS-XLID) by affecting dendritic spine density and morphology. Linkage analysis in a three-generation family with affected males showing intellectual disability, agenesis of corpus callosum, cerebellar hypoplasia, microcephaly and ichthyosis, revealed a candidate disease locus in Xq21.33q24 encompassing over 280 genes. Subsequent to sequencing all coding exons of the X chromosome, we identified a single novel variant within the linkage region, affecting a conserved codon of PAK3. Biochemical studies showed that, similar to previous NS-XLID-associated lesions, the predicted amino acid substitution (Lys389Asn) abolished the kinase activity of PAK3. In addition, the introduced residue conferred a dominant negative function to the protein that drives the syndromic phenotype. Using a combination of in vitro and in vivo studies in zebrafish embryos we show that PAK3N389 escapes its physiologic degradation and is able to perturb MAPK signaling via an uncontrolled kinase-independent function, which in turn leads to alterations of cerebral and craniofacial structures in vivo. Our data expand the spectrum of phenotypes associated with PAK3 mutations, characterize a novel mechanism resulting in a dual molecular effect of the same mutation with a complex PAK3 functional deregulation, and provide evidence for a direct functional impact of aberrant PAK3 function on MAPK signaling.

BACKGROUND: Esophago-gastrointestinal symptoms are frequently reported by patients with eating disorders. Scanty data exist on the relationship between psychopathological traits and digestive complaints. AIMS: To prospectively analyze (i) prevalence of digestive symptoms; (ii) psychopathological traits; (iii) relationship between symptom scores and psychopathological profiles. METHODS: Psychopathological and digestive symptom questionnaires were completed at baseline, at discharge, at 1 and 6 months' follow-up in 48 consecutive patients (85.4% female, median age, 15 years) hospitalized for eating disorders. RESULTS: The most frequently reported symptoms were postprandial fullness (96%) and abdominal distention (90%). Pooled esophageal (4; IQR 0-14) and gastrointestinal (34; IQR 19-53) symptoms significantly decreased at 6 months' follow-up (1; IQR 0-3 and 10; IQR 4-34; p

This paper reports on the diagnostic investigations performed on the bronze panel (a so-called “paliotto”) with high-relief figures inside the Church of San Moisè in Venice (Italy), carried out so as to identify the materials and the causes of corrosion. In particular, both the bulk metal alloy and surface layers (consisting of corrosion products and organic compounds, related with previous restoration actions or original protective treatments) were investigated through a range of microscopic/spectroscopic techniques: the alloy composition and microstructure were determined by VP-SEM/EDS, whilst the corrosion products were analysed by Raman micro-spectroscopy and X-Ray Diffractometry. The organic compounds in surface layers were analysed by Pyrolysis Gas Chromatography Mass Spectrometry (Py-GC-MS). The adjacent mortars were analysed as well, in terms of mineralogical composition and soluble salts content, in order to highlight their role in the degradation processes which took place in the contact areas between the wall and the bronze panel. The results showed that the alloy is a quaternary bronze (Cu-Sn-Zn-Pb): the high-relief figures were cast separately then mechanically joined to the body of the panel. Traces of the clay core were detected in the back of the panel. The bronze surface was artificially patinated by liver of sulfur (K2S). The organic protective layer consisted of stearin and paraffinic wax. However, siccative oil was detected in the layer at the interface with the metal, likely corresponding to the original protective treatment. Also pine resin was detected among the organic compounds. The corrosion products mainly consisted of basic copper chlorides (paratacamite, atacamite) and mixed Cu and Na carbonates (chalconatronite), deriving from the interaction with the surrounding mortar.

Consolidation of sugaring marble (i.e., marble affected by granular disaggregation) still lacks fully effective solutions. Consequently, the use of an innovative phosphate-based treatment, aimed at bonding calcite grains by formation of hydroxyapatite at grain boundaries, has recently been proposed. In this paper, firstly a novel method for producing artificially decayed marble samples, by contact with a heating plate, is proposed. Then, some results are presented about the effectiveness and the compatibility of two different formulations of the phosphate treatment, differing in terms of concentration of the phosphate precursor (3.0 M or 0.1 M aqueous solutions of diammonium hydrogen phosphate, DAP), possible ethanol addition to the DAP solution and number of DAP solution applications (1 or 2). The results of the study point out that the new weathering method allows to obtain specimens with a gradient in microstructural and mechanical properties with thickness, just like naturally weathered samples. Both phosphate treatments were able to significantly improve marble cohesion, without causing significant changes in thermal behaviour and aesthetic appearance after treatment. The addition of small quantities of ethanol to the DAP solution seems to be a very promising method for favouring HAP formation and improving the treatment performance.

This article describes a case of pyruvate dehydrogenase deficiency in a 3-year-old boy who presented generalized hypotonia, severe psychomotor development delay, and generalized and partial seizures and was refractory to antiepileptic drugs. After the diagnosis, the patient was put on a ketogenic diet. Six months later, seizure frequency was reduced and psychomotor development had improved. At the same time he presented some side effects, such as 2 episodes of significant increases in cholesterol and triglycerides associated with viral respiratory infections. The latter decreased with a supplementation of ω-3 fatty acids and an increase in caloric intake.

Pantothenate kinase-associated neurodegeneration (PKAN) is a rare disorder associated with brain iron accumulation. The brain MRI abnormality consists of T2 hypointensity in the globus pallidus with a small hyperintensity in its medial part, called the "eye-of-the-tiger" sign. We report on 2 patients affected by PKAN, in whom MRI examination did not demonstrate the "eye-of-the-tiger" sign in the early stages; the typical abnormalities were detected only in the following examinations. Case 1 is a 4-year-old boy first studied at age 2 years for psychomotor delay. The brain MRI was normal. In the following 2 years, the motor impairment progressed. The second brain MRI at age 4 years demonstrated the "eye-of-the-tiger" sign. Molecular analysis of the PANK2 gene revealed a missense mutation F228S in exon 2 in homozygosis. Case 2 is a 6-year-old boy first studied at age 2 years because of psychomotor delay. His brain MRI did not demonstrate abnormalities in the globus pallidus. In the following years spastic-dystonic tetraparesis became evident. A brain MRI at age 4 years demonstrated the "eye-of-the-tiger" sign. Molecular analysis of the PANK2 gene revealed a missense mutation in exon 5 (N501I). Our 2 cases demonstrate that the observation of a normal globus pallidus in the early stage of the disease does not exclude the diagnosis of classic PKAN.

Following a previous preliminary report on a group of children suffering from partial epilepsies, we present the final considerations on the same group in order to evaluate the long-term efficacy, tolerability and safety of oxcarbazepine (OXC). We enrolled 36 patients (mean age 8.5), between January 2003 and December 2004, with new diagnosis of partial epilepsy: 25 patients were affected by idiopathic partial epilepsy, eight by symptomatic epilepsy and three by cryptogenic epilepsy. Each patient was scheduled to attend the center four times after the initial examination: 3 months (T1), 12 months (T2), 24 (T3) months and 36 (T4) months after the beginning of OXC-monotherapy (T0). At the end of our study, 20 patients were seizure free (SF): nine stopped OXC because of SF for at least 2 years, 11 were still on therapy. One patient showed a reduction of seizure frequency >or=50%, three were non responders (but still on therapy), nine stopped OXC due to a non-responder condition during follow-up before T4 and one because of adverse effects. At the end of the study no EEG focal abnormalities became generalized because of treatment. Normalization of EEG was observed in ten patients. Our preliminary findings have been confirmed. OXC can be considered an effective and well tolerated first line drug for long-term monotherapy in children with epilepsy, both for idiopathic and symptomatic/cryptogenic forms.

Background and purpose: To review our experience of the efficacy and tolerability of felbamate in children younger than 4 years. Methods: We used a retrospective chart review to identify 53 children with seizures who were younger than 4 years. Efficacy was evaluated based on the occurrence of responsiveness, defined as seizure frequency reduction of more than 50% for a minimum period of 4 months. Tolerability was based on parent-reported side effects. Results: Twenty-two (41%) patients resulted to be responders and 31 (59%) did not. By univariate analysis, those achieving seizure remission were probably much older, to have a shorter history of epilepsy and a lower frequency of seizures before felbamate therapy. The number of antiepileptic drugs (AEDs) used before felbamate therapy was the only significant predictor of the duration of response to felbamate, with a longer responsiveness to the drug seen in those who were placed under fewer than three AEDs before felbamate compared with those who had taken more than three (median, 16 months vs. 7 months; P

Research letter to the editor

Background To evaluate evidence and prognosis of refractory cases of absence seizures. Methods Subjects with refractory absence seizures were identified retrospectively in 17 Italian epilepsy pediatrics Centers. We analyzed age at onset, family history, presence of myoclonic components, seizure frequency, treatment with antiepileptic drugs (AEDs), interictal electroencephalography (EEG) and neuropsychological assessment. Two subgroups were identified: one with patients with current absence seizures and another with patients that had become seizure free with or without AED treatment. The chi-square test was applied. Results A total of 92 subjects with drug-resistant absence seizures were analyzed. 45 subjects still show absence seizures (49%) and the other 47 became seizure free (51%) after a period of drug-resistance. The statistical analysis between these two groups showed no correlation between age of onset, family history and abnormalities at interictal EEG. Statistically significant differences were observed with regard to the number of AEDs used and intellectual disability. Conclusion Typical absence epilepsy classifiable as Childhood Absence Epilepsy could not be considered so “benign”, as suggested in literature. A longer duration of disease and a higher frequency of seizure seem to be correlated with a higher presence of cognitive impairment. No significant risk factor was observed to allow the faster and better recognition of patients with worse prognosis.

Juvenile myoclonic epilepsy is a common type of epilepsy with onset occurring during adolescence. This review provides a collection of evidence relating to the treatment of this type of epilepsy. Historically, the large majority of patients become seizure-free when treated with valproate. Over recent years, there has been a marked improvement in the pharmacological armamentarium by the physicians. Currently, administration of new antiepileptic drugs, such as levetiracetam, lamotrigine and topiramate, seems to have beneficial effects in the patients with poor response to valproate.

Risk factor analysis of posterior reversible encephalopathy syndrome after allogeneic hematopoietic SCT in children

PURPOSE: The aim of the present study was to assess serum lipid levels before and after treatment with oxcarbazepine in children with epilepsy. METHODS: We measured total cholesterol (TC), triglycerides (TGs) and high-density lipoprotein cholesterol (HDL-C) in 28 patients whereas only TC levels in 11 patients, during baseline period and at 3 months after the beginning of therapy with oxcarbazepine. RESULTS: During baseline period, median values were: 4.38 (IQR=4.12-5.03) mmol/L for TC levels, 1.72(IQR=1.42-2.01) mmol/L for HDL-C levels and 1.54 (IQR=1.29-1.96) mmol/L for TGs levels. At three months, median values were: 4.38 (4.10-4.95) mmol/L for TC levels (p

Objective: To give a contribution about the existence of non familial benign infantile convulsions during the first two years of life. Patients and Methods: We evaluated 58 patients (29 females and 29 males) with seizure onset ranging between 4 and 24 months of life, regular psychomotor development, unremarkable neuro-imaging studies and interictal electroencephalogram. We analyzed gender, age at onset, duration, manifestations and frequency of seizures, family history of febrile or infantile convulsions. Results: 17 patients had a family history of benign epilepsy, while 41 had not. Among the latter, 12 showed a family history of febrile seizures and the remaining 29 had an uncertain or fully negative familiarity with it. No clinical differences have been observed between the familial and the non familial groups. Nobody experienced seizures after the age of two and the psychomotor development remained normal in all patients, during follow-up. Conclusions: This study confirms the existence of non familial benign infantile convulsions and the benign outcome of this type of seizures either familial or non-familial. In addition to this, we also discuss the possibility to avoid anti-epileptic treatment.

Emilio Franzoni,1 Stefano Gualandi,1 Vincenzo Caretti,2 Adriano Schimmenti,3 Elena Di Pietro,1 Gaetano Pellegrini,1 Giuseppe Craparo,3 Arianna Franchi,1 Alberto Verrotti,4 Alessandro Pellicciari11Child Neuropsychiatry Unit, University of Bologna, Italy; 2Department of Psychology, University of Palermo, Italy; 3Faculty of Human and Social Sciences, Kore University of Enna, Enna, Italy; 4Department of Pediatrics, University of Chieti, ItalyBackground: The connections between eating disorders (EDs) and alexithymia have not been fully clarified. This study aims to define alexithymia's connections with shame, trauma, dissociation, and body image disorders.Methods: We administered the Dissociative Experience Scale-II, Trauma Symptom Inventory, Experience of Shame Scale, Toronto Alexithymia Scale-20, and Body Uneasiness Test questionnaires to 143 ED subjects. Extensive statistical analyses were performed.Results: The subjects showed higher scores on alexithymia, shame, dissociation, and traumatic feelings scales than the nonclinical population. These aspects are linked with each other in a statistically significant way. Partial correlations highlighted that feelings of shame are correlated to body dissatisfaction, irrespective of trauma or depressed mood. Multiple regression analysis demonstrates that shame (anorexic patients) and perceived traumatic conditions (bulimic and ED not otherwise specified) are associated with adverse image disorders.Conclusion: Shame seems to hold a central role in the perception of an adverse self-image. Alexithymia may be interpreted as being a consequence of previous unelaborated traumatic experiences and feelings of shame, and it could therefore be conceptualized as a maladaptive–reactive construct.Keywords: eating disorders, trauma, alexithymia, shame, body image

Mowat-Wilson syndrome (MWS) is a genetic disease caused by heterozygous mutations or deletions of the ZEB2 gene and is characterized by distinctive facial features, epilepsy, moderate to severe intellectual disability, corpus callosum abnormalities and other congenital malformations. Epilepsy is considered a main manifestation of the syndrome, with a prevalence of about 70-75%. In order to delineate the electroclinical phenotype of epilepsy in MWS, we investigated epilepsy onset and evolution, including seizure types, EEG features, and response to anti-epileptic therapies in 22 patients with genetically confirmed MWS. Onset of seizures occurred at a median age of 14.5 months (range: 1-108 months). The main seizure types were focal and atypical absence seizures. In all patients the first seizure was a focal seizure, often precipitated by fever. The semiology was variable, including hypomotor, versive, or focal clonic manifestations; frequency ranged from daily to sporadic. Focal seizures were more frequent during drowsiness and sleep. In 13 patients, atypical absence seizures appeared later in the course of the disease, usually after the age of 4 years. Epilepsy was usually quite difficult to treat: seizure freedom was achieved in nine out of the 20 treated patients. At epilepsy onset, the EEGs were normal or showed only mild slowing of background activity. During follow-up, irregular, diffuse frontally dominant and occasionally asymmetric spike and waves discharges were seen in most patients. Sleep markedly activated these abnormalities, resulting in continuous or near-to-continuous spike and wave activity during slow wave sleep. Slowing of background activity and poverty of physiological sleep features were seen in most patients. Our data suggest that a distinct electroclinical phenotype, characterized by focal and atypical absence seizures, often preceded by febrile seizures, and age-dependent EEG changes, can be recognized in most patients with MWS.

OBJECTIVE: To describe the electroclinical features and the long-term outcomes of epilepsy in a large cohort of males and females with Down syndrome who developed epilepsy in childhood. STUDY DESIGN: Subjects with Down syndrome and cryptogenic epilepsy with onset in childhood were identified retrospectively from the databases of 16 Italian epilepsy centers over a 40-year period. For each subject, age at onset of seizures, seizure semiology and frequency, electroencephalography characteristics, treatment with antiepileptic drugs, and long-term clinical and electroencephalography outcomes were analyzed. RESULTS: A total of 104 subjects (64 males [61.5%], 40 females [38.5%]) were identified. Seizure onset occurred within 1 year of birth in 54 subjects (51.9%), between 1 and 12 years in 42 subjects (40.4%), and after 12 years in 8 subjects (7.7%). Males had a younger age of seizure onset than females. Of the 104 subjects, 51 (49.0%) had infantile spasms (IS), 35 (33.7%) had partial seizures (PS), and 18 (17.3%) had generalized seizures (GS). Febrile seizures were recorded in 5 (4.8%) subjects. Intractable seizures were observed in 23 (22.1%) subjects, including 5 (9.8%) with IS, 8 (44.4%) with PS, and 10 (31.3%) with GS. CONCLUSION: Cryptogenic epilepsy in Down syndrome may develop during the first year of life in the form of IS or, successively, as PS or GS. Electroclinical features of IS resemble those of idiopathic West syndrome, with a favorable response to treatment with adrenocorticotropic hormone seen. Patients experiencing PS and GS may be resistant to therapy with antiepileptic drugs

The purpose of the study is to explore the causative role of TUBB2B gene mutations in patients with different malformations of cortical development. We collected and evaluated clinical and MRI data of a cohort of 128 consecutive patients (61 females and 67 males) in whom brain MRI had detected a spectrum of malformations of cortical development including polymicrogyria or pachygyria, who were mutation-negative to other possible causative genes. Mutation analysis of the TUBB2B gene was performed. We identified three new TUBB2B mutations in three unrelated patients (3 out of 128; 2.3%) with a diffuse and rather symmetrical cortical abnormality, including diffuse polymicrogyria in two and bilateral regional pachygyria in one. One patient harbored a p.Asp417Asn amino-acid substitution in the C-terminal domain of the protein; one patient a p.Asn256Ser amino-acid substitution in the intermediate domain and one patient a p.Leu117Pro amino-acid substitution in the N-terminal domain. The localization of each mutation within the secondary structure of the β2-tubulin polypeptide suggests that these mutations might alter the proper functions of microtubules. The phenotypic spectrum associated with TUBB2B mutations is wider than previously reported and includes diffuse, symmetric malformations of cortical development.

La parola neurologia origina dal greco neuron (nervo) e loghia (studio del). Lo studio della neurologia inizia sin dai tempi preistorici, ma solo nel XVII secolo, la branca assume un connotato accademico e da scienza basata sulla pura osservazione sviluppa una via sistematica d’approccio allo studio del sistema nervoso ed ai possibili interventi sulle patologie neurologiche. L’equivalente del greco psychés (anima) e iatreon (medico), dal quale il XIX secolo avrebbe tratto il neologismo psichiatria, lo si poteva leggere a Tebe intorno al XIII secolo a.C., sul frontone della biblioteca del faraone Ozymandias [traslitterazione in greco di uno dei nomi usati dal faraone Ramses II il grande (chiamato anche “User-maat-re Setep-en-re“) terzo faraone della XIX dinastia (1279-1213 a.C.)]: questo è uno dei più antichi esempi conosciuti dell’uso di queste due parole riavvicinate.

Corticosteroid treatment is the standard of care in Duchenne muscular dystrophy (DMD), but the optimal age to initiate treatment and dosage pattern remain a matter of discussion. METHODS: We performed a long-term study of alternate-day corticosteroids in five 2- to 4-year-old DMD patients. The primary outcome measure was prolongation of the ability to walk. RESULTS: One patient lost ambulation at age 10. Four patients, aged 16 to 18 were fully ambulant, and 3 of them could still climb stairs. Respiratory function was moderately reduced in 2. Left ventricular ejection fraction was > 45%. Short stature and delayed puberty were the most relevant side effects. Although the negative impact of corticosteroid treatment on growth rate remained their major concern, parents and patients stated that they preferred corticosteroid therapy. CONCLUSIONS: Long-term corticosteroid treatment is effective in prolonging function but not in recovering lost function, and its early use seems appropriate

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