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The severe eutrophication of the Baltic Sea requires mussel (Mytilus spp.) farming to remove nutrients, but farming in a low salinity environment results in smaller mussels that require value enhancement to be economically viable. This study evaluates the biomass valorisation of smaller Baltic mussels, focusing on the extraction of oil, protein and glycogen. It analyses the amino acid profiles, oil and fatty acid contents and glycogen levels of the mussels, as well as their prebiotic properties on beneficial gut bacteria. In addition, the study improves the extraction of bioactive compounds through enzymatic hydrolysis. Results indicate significant seasonal differences, with summer mussels having higher meat and lower ash content, and a rich content of essential fatty acids, particularly omega-3, and amino acids, underscoring the mussels' sustainability as a food source. The enzymatically treated biomass exhibited notable prebiotic activity, proposing health-promoting benefits. The study underscores the valorization of Baltic mussel biomass, highlighting its role in health, nutrition, and environmental sustainability.

Exploiting the underutilised biomass of the Baltic Blue Mussel, Mytilus edulis and Mytilus trossulus hybrid offers a promising avenue for strengthening the economic potential of aquaculture in the Baltic Sea region. This study describes the optimisation of a novel meat extraction technique to address the unique low salinity challenges of the region. The method involves a sequence of procedures, initiated by thawing and desalting via freshwater rinsing to reduce salt content, followed by mechanical disruption. The decantation stages were carefully designed, including the use of citric acid to neutralise off-flavours, thereby improving the sensory profile of the product—a critical determinant of consumer acceptance. Process refinement resulted in a significant increase in meat extraction from an initial 7.62 to 12.06%, with autumn harvests proving superior in both quantity and sensory quality. Further processing steps, including stirring, boiling, centrifugation, and iterative drying, calibrated the moisture content and produced a highly pure, fine, and homogeneous mussel powder. This comprehensive approach highlights the potential for a scalable, efficient, and economically viable extraction method that could make a significant contribution to the regional seafood industry.

Funoran is a promising food polysaccharide with unique gelling properties but understanding its gelation behaviors remains inadequate. This study aimed to investigate the influence of cooling and heating rates on funoran gelation properties and study its gelation mechanism by dynamic rheology and NMR studies. With constant heating rates, the melting point was unaffected by changing cooling rates, whereas higher cooling and heating rates enhance the gel strength and thermal hysteresis. The gelation kinetics mechanism of the funoran solution was elucidated at different quenching temperatures. The rheological measurement results indicated that the funoran solution quenched to lower temperatures resulted in higher G` in the gel state, and faster gelation rate compared with the solution quenched at higher temperatures. 1H NMR results revealed a slow decay in funoran signal intensity at higher temperatures (20 °C) and a fast signal decay at 2 °C. A theoretical model involving second and first-order reactions was proposed to describe the gelation kinetic process of funoran at different quenching temperatures. It was shown longer helix lengths were required for stable intermolecular association and gel formation at higher temperatures than at lower temperatures. These results offer valuable insights for optimizing the funoran gelation properties for various food applications.

Alloy 617 is identified as a potential material for defense applications, particularly in military air platforms. Therefore, this study aims to examine the impact of strain rate on Alloy 617 properties during low-cycle fatigue (LCF) at room temperature (RT). LCF life properties and damage mechanism of alloy 617 are examined across a strain rate range of (5x10-4 ~ 10-2 s-1) in a fully-reversed controlled total strain range of 1.2%. Slow strain rate test (SSRT) is found to be a cost-effective method for assessing the material capability to respond to environmental interference. This study shows that LCF life of Alloy 617 is strongly influenced by the time-dependent mechanism, in terms of SSRT. The relationship between total strain, plastic strain, and time to failure with strain rates are established and expressed using the power law function to describe the fatigue life. Fractured specimens undergo metallography examination using an electron microscope, and fractography is discussed to differentiate the impact of SSRT on the physical damage characteristics under LCF loadings. LCF resistance of Alloy 617 is found to be time-dependent. Based on the results, it is recommended that the factor of safety must be considered in the designing phase to evaluate the fatigue life.

Abstract Background Wound healing is an active, complex, integrated series of cellular, physiological, and biochemical changes initiated by the stimulus of injury in a tissue. The present study was performed to investigate the potential wound healing abilities of Sargassum ilicifolium crude extracts (CE) that were characterized by 1H NMR and FTIR Spectrometric measurements. Materials and methods Seaweed samples were collected from southern coastal sites of Sri Lanka. To determine the cytotoxicity and proliferation of S. ilicifolium CE were used for the MTT and alamarBlue assays respectively. The scratch and exclusion wound models were used to HaCaT and HDF cells to assess the cell proliferation and migration. RAW 264.7 cells (macrophages) were used to evaluate Nitric Oxide (NO) production and phagocytosis activities. Moreover, Fifteen, 8-week-old, female, New Zealand rabbits were selected and divided into five groups: excision skin wounds (10.40 ± 0.60 mm) were induced in groups I, II, and III. Rabbits in groups I and IV were given S. ilicifolium CE (orally, 100 mg/kg day, two weeks), whereas groups II and V were given equal amounts of distilled water. Wound healing properties were measured and wound tissue samples were collated, formalin-fixed, wax-embedded, stained (Hematoxylin and Eosin; Van Gieson) and examined for the healing process. Results Anti-inflammatory and wound healing activities were observed in RAW 264.7, HDF and HaCaT cells treated with S. ilicifolium aqueous extracts when compared to the control groups. S. ilicifolium extracts concentration 8 - 4 μg/μL, (P

Centrifugal pumps are divided into two main components, namely the impeller and the volute. The impeller is a rotating pump component, while the volute is a stationary pump component. The current problem in the industrial world is that the pump capacity that operates is not in accordance with the written capacity so that the pump does not work optimally. In addition, the structural analysis of the pump is minimally carried out so that damage to the pump often occurs. To overcome this, it is necessary to analyze the performance and structure of the centrifugal pump design using computational fluid dynamics and static structural analysis methods on variations in the number of blades & impeller rotation speed. The results of the CFD analysis obtained are validated using existing product specifications and studies that have been carried out. The maximum value of the resulting von-mises stress is 20,238 Mpa, the maximum value of the resulting strain is 0.00010133, while the maximum total deformation value is 0.051984 mm. The three maximum values ​​occur in the variation of the 7 blade impeller with a rotational speed of 1800 rpm.

Abstract Seaweed is a popular edible source and is associated with many foods and pharmaceutical industries around the world. The current research aims to provide information on the chemical composition of 15 seaweed species, consisted of Chlorophyta, Ochrophyta/Phaeophyceae, and Rhodophyta macroalgae, collected from coastal areas of Sri Lanka. Seaweed samples were subjected to the analysis of lipids, proteins, ash and macro, micro, trace and ultra-trace elements. The highest protein content was recorded in the brown algae. Maximum dietary fiber and ash contents were recorded from green algae. The highest predominant fatty acids were observed from green seaweeds (Caulerpa racemosa); however, linoleic acid (C18:2n6) is the dominant fatty acid of all macroalgae. Mineral contents were highest in the red macroalga; however, copper, zinc and magnesium were also comparatively higher in green alga Ulva lactuca. In conclusion, 15 seaweed species belonging to the three different classes of seaweeds are investigated in details to obtain their biochemical, mineral and fatty acid compositions for the synthesis of novel therapeutic agents. In order to explore biorefinery processes for these seaweeds, as well as how they can potentially be cultivated, more extensive studies are required. Studying and determining the nutritional values of seaweeds will be beneficial with the potentials for future industrial uses and research.

Abstract Human envenoming from the bite of the abundant hump-nosed pit viper (Hypnale spp.) (HNPV) is a frequent occurrence with victims experiencing unpleasant and sometimes life-threatening consequences. Further, clinico-pathology, treatment and management measures in HNPV envenomed dogs are under recognized. Prospective investigations were performed to assess the clinico-pathology and management options for HNPV envenomed dogs brought to the University of Peradeniya’s Veterinary Teaching Hospital from January, 2012 to March 2018. We recorded the local and systemic manifestations, hematological and urinary abnormalities of 78 dogs in which HNPV bite had been witnessed by the owner. Mild swelling, extensive swelling, hemorrhagic blistering and hemorrhagic bullae at the site of bite were observed in 59%, 31%, 6% and 4% of the dogs, respectively. Some dogs were subjected to surgical excision of necrotized tissue including limb amputation. We observed the following systemic clinical effects in envenomed dogs: neurotoxicity (13%), acute kidney injury (AKI) (14%) and coagulopathy (16%). All dogs showed leukocytosis with mean white blood cell count of 25.25 × 103/µL. Mild anemia and thrombocytopenia were detected in 29% of the dogs. There was a significant correlation between extent of local tissue injuries with length of hospitalization (LH). The mean time of coagulopathy observed was 21.3 h (IQR: 8–48 h). In coagulopathic dogs, there was a strong correlation between LH and extent of local tissue injury (r s = 0.7751, P

The proper maintenance of the hydraulic axial pump was discussed to maximize its performance and minimize downtime. In this study, reliability assessment by a two-parameter Weibull was applied as a reference for the periodic maintenance of the components in the hydraulic axial pump with a capacity of 2000 litres per second. The two-parameter Weibull consisted of shape parameter β and scale parameter θ. An assessment was conducted to determine the critical parts and determine the failure rate of the formed data patterns. The three critical components of mechanical seal, bearing, shaft were investigated from each of six hydraulic axial pumps. By applying the two-parameter Weibull, the mean time to failure value of mechanical seal, bearing, and the shaft is obtained as 182.91 hours, 381.08 hours, 755.67 hours, respectively. Furthermore, the reliability values of those components were 52%, 52%, and 53%, respectively. By following these results, periodic maintenance could be then conducted regularly.

Seaweed contains a variety of bioactive compounds; the most abundant of them are polysaccharides, which have significant biological and chemical importance. Although algal polysaccharides, especially the sulfated polysaccharides, have great potential in the pharmaceutical, medical and cosmeceutical sectors, the large molecular size often limits their industrial applications. The current study aims to determine the bioactivities of degraded red algal polysaccharides by several in vitro experiments. The molecular weight was determined by size-exclusion chromatography (SEC), and the structure was confirmed by FTIR and NMR. In comparison to the original furcellaran, the furcellaran with lower molecular weight had higher OH scavenging activities. The reduction in molecular weight of the sulfated polysaccharides resulted in a significant decrease in anticoagulant activities. Tyrosinase inhibition improved 2.5 times for hydrolyzed furcellaran. The alamarBlue assay was used to determine the effects of different Mw of furcellaran, κ-carrageenan and ι-carrageenan on the cell viability of RAW264.7, HDF and HaCaT cell lines. It was found that hydrolyzed κ-carrageenan and ι-carrageenan enhanced cell proliferation and improved wound healing, whereas hydrolyzed furcellaran did not affect cell proliferation in any of the cell lines. Nitric oxide (NO) production decreased sequentially as the Mw of the polysaccharides decreased, which indicates that hydrolyzed κ-Carrageenan, ι-carrageenan and furcellaran have the potential to treat inflammatory disease. These findings suggested that the bioactivities of polysaccharides were highly dependent on their Mw, and the hydrolyzed carrageenans could be used in new drug development as well as cosmeceutical applications.

Seaweeds have been regarded as a reservoir of biologically active molecules that are important in the pharmaceutical industry. The aim of the present study was to explore the wound healing properties and to assess the safety of the seaweed Sargassum ilicifolium and Ulva lactuca. Enhanced cell proliferation and cell migration activities were observed in L929 cells treated with S. ilicifolium extract compared to U. lactuca extract treated cells and the control group. In-vivo experiments were conducted using five groups (10 in each) of Albino mice (BALB/c). Mice in group I and group II were treated (Orally, 100 mg/kg BW/day) with aqueous extracts of S. ilicifolium and U. lactuca, respectively for 14 days. Treatment group III received a topical application of the aqueous extract of S. ilicifolium (25% w/w) and ointment base (75% w/w) (2 g/kg BW/day, for 14 days). Group IV (Control) received an equal amount of distilled water, orally and mice in group V kept without wounds. The extract from S. ilicifolium showed stronger wound healing properties than the one from Ulva lactuca. Histopathological findings also revealed that the healing process was significantly enhanced in the mice group treated orally with S. ilicifolium aqueous extract. These findings show that S. ilicifolium species possess promising wound healing properties in-vitro and in-vivo.

Mussel farming is a novel and growing aquaculture field in the Baltic Sea. Nevertheless, there is very little published evidence on the processing of shellfish biomass in the region. The aim of this study is to develop a methodology for the extraction of organic-rich fractions from small-sized blue mussels of the Baltic Sea region that is applicable and economically viable for the feed and food industry. The efficiency of mussel meat separation was evaluated using different processing, drying, and filtration techniques. The laboratory experiments have succeeded in finding a method that is operationally feasible and does not require overly complex and expensive laboratory settings. These trials also showed that the separation of meat from fresh or frozen mussels can be achieved by simple crushing and sedimentation methods and the extraction yielded a significant amount of mussel meat (7.6%) with a high protein content (3.2%, i.e., half of the total protein found in the used mussel-mass). It also appeared that the use of filtration is not practical because the protein loss was extremely high. In addition, filtration makes the process of dry-matter separation more complex, and costs are unlikely to be compensated by the energy saved in drying.

Galactans are important components of many plant cell walls. Besides, they are the major polysaccharides in extracellular matrixes from different seaweeds, and other marine organisms, which have an acidic character due to the presence of sulfate groups in their structures. In particular, most of the red seaweeds biosynthesize sulfated galactans with very special linear backbones, constituted by alternating (1→3)-β-d-galactopyranose units (A-unit) and (1→4)-α-galactopyranose residues (B-unit). In the industrially significant seaweeds as source of hydrocolloids, B-units belong either to the d-series and they produce carrageenans (as in the order Gigartinales), or to the l-series, and they are sources of agarose and/or structurally related polymers (i.e., Gelidiales, Gracilariales). In both cases, the latter units appear as cyclized 3,6-anhydro-α-galactose in certain amounts, which can be increased by alkaline cyclization of α-galactose 6-sulfate units. Besides, it has been clearly shown that some red algae produce different amounts of both galactan structures, known as d/l-hybrids. It is not yet clear if they comprise both diasteromeric types of units in the same molecule, or if they are mixtures of carrageenans and agarans that are very difficult to separate. It has been reported that the biosynthesis of these galactans, showing that the nucleotide transport for d-galactopyranose units is UDP-d-Gal, while for l-galactose, it is GDP-l-Gal, so, there is a different pathway in the biosynthesis of agarans. However, at least in those seaweeds that produce carrageenans as major galactans, but also agarans, both synthetic pathways should coexist. Another interesting characteristic of these galactans is the important variation in the sulfation patterns, which modulate their physical behavior in aqueous solutions. Although the most common carrageenans are of the κ/ι- and λ-types (with A-units sulfated at the 4- and 2-positions, respectively) and usually in agarans, when sulfated, is at the 6-position, many other sulfate arrangements have been reported, greatly influencing the functional properties of the corresponding galactans. Other substituents can modify their structures, as methyl ethers, pyruvic acid ketals, acetates, and single stubs of xylose or other monosaccharides. It has been shown that structural heterogeneity at some extent is essential for the proper functional performance of red algal galactans.

Over the decades, the world has witnessed diverse virus associated pandemics. The significant inhibitory effects of marine sulfated polysaccharides against SARS-CoV-2 shows its therapeutic potential in future biomedical applications and drug development. Algal polysaccharides exhibited significant role in antimicrobial, antitumor, antioxidative, antiviral, anticoagulant, antihepatotoxic and immunomodulating activities. Owing to their health benefits, the sulfated polysaccharides from marine algae are a great deal of interest globally. Algal polysaccharides such as agar, alginate, carrageenans, porphyran, fucoidan, laminaran and ulvans are investigated for their nutraceutical potential at different stages of infection processes, structural diversity, complexity and mechanism of action. In this review, we focus on the recent antiviral studies of the marine algae-based polysaccharides and their potential towards antiviral medicines.

Carrageenases appear in various species of marine bacteria and are widely used for the degradation of carrageenans, the commercially significant sulphated polysaccharides. The carrageenase production ability of six different Cellulophaga species was identified, with ι-carrageenase being the most abundant carrageenolytic enzyme. C. algicola was the most potent strain, followed by C. fucicola and C. geojensis, whereas C. pacifica was the least effective carrageenase producer among the studied strains. The enzyme production was maximized using the one-factor-at-a-time optimization method. The optimal incubation temperature was identified as 25 °C and the incubation time was set as 48 h for all tested species. The optimal medium composition for Cellulophaga strains was determined as 30 g/L sea salt, 1.4 g/L furcellaran, and 3 g/L yeast extract. An ultrafiltered enzyme extracted from C. algicola had the highest activity at around 40 °C. The optimal pH for enzymatic degradation was determined as 7.8, and the enzyme was fairly stable at temperatures up to 40 °C.

The detection of cancer biomarkers in the early stages could prevent cancer-related deaths significantly. Nanomaterials combined with biomolecules are extensively used in drug delivery, imaging, and sensing applications by targeting the overexpressed cancer proteins such as folate receptors (FRs) to control the disease by providing earlier treatments. In this investigation, biocompatible reduced graphene oxide (rGO) nanosheets combined with folic acid (FA)-a vitamin with high bioaffinity to FRs-is utilized to develop an electrochemical sensor for cancer detection. To mimic the cancer cell environment, FR-β protein is used to evaluate the response of the rGO-FA sensor. The formation of the rGO-FA nanocomposite was confirmed through various characterization techniques. A glassy carbon (GC) electrode was then modified with the obtained rGO-FA and analyzed via differential pulse voltammetry (DPV) for its specific detection towards FRs. Using the DPV technique, the rGO-FA-modified electrode exhibited a limit of detection (LOD) of 1.69 pM, determined in a linear concentration range from 6 to 100 pM. This excellent electrochemical performance towards FRs detection could provide a significant contribution towards future cancer diagnosis. Moreover, the rGO-FA sensing platform also showed excellent specificity and reliability when tested against similar interfering biomolecules. This rGO-FA sensor offers a great promise to the future medical industry through its highly sensitive detection towards FRs in a fast, reliable, and economical way.

Alloy 800H is currently under consideration to be used in a very high temperature reactor system. This paper describes the experimental low-cycle fatigue study on Alloy 800H base metal and weldments in the air with fully-reversed strain control at 700 °C. The recommended modern Alloy 800H weldments were fabricated by gas tungsten arc welding. The observed fatigue life is found to be comparable between base metal and weldments at this temperature, indicating the welding technique can compete with base material. The influence of hold time was further investigated at the maximum tensile strain of 0.6% total strain range condition for 60 s, and it is found to reduce the fatigue life. For most of the tests, the base metal revealed an initial cyclic hardening stress response, otherwise, the weldment was entirely softened with cycles. The addition of hold time is also found to induce an unequal cyclic stress response in both materials. Accordingly, for continuous low-cycle fatigue tests, fatigue cracks were initiated and propagated in a classical transgranular fracture mode. However, the hold time affected the mixed-mode fracture mode by means of a transition from transgranular to intergranular fracture.

This paper presents the high-temperature creep-fatigue testing of a Ni-based superalloy of Alloy 617 base metal and weldments at 900 °C. Creep-fatigue tests were conducted with fully reversed axial strain control at a total strain range of 0.6%, 1.2%, and 1.5%, and peak tensile hold time of 60, 180, and 300 s. The effects of different constituents on the combined creep-fatigue endurance such as hold time, strain range, and stress relaxation behavior are discussed. Under all creep-fatigue tests, weldments’ creep-fatigue life was less than base metal. In comparison with the low-cycle fatigue condition, the introduction of hold time decreased the cycle number of both base metal and weldments. Creep-fatigue lifetime in the base metal was continually decreased by increasing the tension hold time, except for weldments under longer hold time (>180 s). In all creep-fatigue tests, intergranular brittle cracks near the crack tip and thick oxide scales at the surface were formed, which were linked to the mixed-mode creep and fatigue cracks. Creep-fatigue interaction in the damage-diagram (D-Diagram) (i.e., linear damage summation) was evaluated from the experimental results. The linear damage summation was found to be suitable for the current limited test conditions, and one can enclose all the data points within the proposed scatter band.

Cyclic stress response and fracture behaviors of Alloy 617 base metal (BM) and Alloy 617 weld joints (WJ) are investigated under strain controlled low cycle fatigue (LCF) loading. Axial fully reversed total-strain controlled tests have been conducted at room temperature with total strain ranges of 0.6, 0.9, 1.2, and 1.5%. At the all testing conditions, weld joint specimens have shown higher peak stresses than the base metal specimens, whereas the plastic strain accumulation of the base metal specimens is comparatively higher than those of the weld joint specimens. The cyclic stress response behavior of both base metal and weld joint specimens revealed initial cyclic hardening during first small number of cycles followed by progressive softening to failure. Higher strain amplitudes decreased the fatigue lives for both base metal and weld joint specimens; subsequently weld joint specimens had lower fatigue resistances relative to base metal specimens. Furthermore, the cracking in weld joint specimens initiated in the weld metal (WM) region. The crack initiation and propagation showed transgranular mode for both base metal and weld joint specimens; especially weld joint specimens showed a wedge type crack initiation about 45 degrees to the loading direction because of the dendritic structure.

The aim of this study is to investigate the fully‐reversed low cycle fatigue properties of Alloy 617 in the air at 950 °C; these tests were conducted at total strain ranges from 0.9% to 1.5% with a constant strain rate of 10−3/s. The result of the fatigue tests showed a decrease in fatigue resistance with an increasing total strain range. The reduction of fatigue resistance was due to the effect of the total strain range and microstructure evolution during high temperature, such as brittle oxides cracking. At all testing conditions, the cyclic softening mechanism was observed as a function of the total strain range in the current high temperature condition. An analysis of low cycle fatigue resistance was performed using the Coffin–Manson relationship and the total strain energy density; it was found that Alloy 617 followed these relationships well. In addition, this study compared well with previous work reported in the literature for a similar testing condition. Post‐fracture analysis on the fracture surfaces of failed specimens revealed a more severe damage cracking at the periphery of specimens due to the increase in the total strain range. The surface connected grain boundary cracks induced by oxidation were obvious at low strain range. Thus, the primary crack propagation occurred in transgranular mode from persistent slip bands.

In this study, we comparatively investigate the low cycle fatigue behavior of Alloy 617 (INCONEL 617) weldments by gas tungsten arc welding process at room temperature and 800 °C in the air to support the qualification in high temperature applications of the Next Generation-IV Nuclear Plant. Axial total-strain controlled tests have been performed with the magnitude of strain ranges with a constant strain ratio (Rε = −1). The results of fatigue tests consistently show lower fatigue life with an increase in total strain range and temperature at all testing conditions. The reduction in fatigue life may result from the higher cyclic plastic strain accumulation and the material ductility at high temperature conditions. A constitutive behavior of high temperature by some cyclic hardening was observed. The occurrence of serrated yielding in the cyclic stress response was also observed, suggesting the influence of dynamic strain aging during high temperature. We evaluated a well-known life prediction model through the Coffin-Manson relationship. The results are well matched with the experimental data. In addition, low cycle fatigue cracking occurred in the weld metal region and initiated transgranularly at the free surface.

Abstract: At the onset of West syndrome a specific impairment of visual function has been clearly demonstrated, while other aspects of sensorial development, and in particular of the auditory function, have been less studied. The aim of this study was to evaluate auditory function and orienting responses at the onset of West syndrome, and to relate the results with EEG patterns, visual function and neurodevelopmental competence. A prospective multicentric study was performed on 25 successively enrolled infants with West syndrome; all the patients underwent a full clinical assessment, including MRI and video-EEG, visual function and auditory orienting responses (AORs) as well as Griffiths'' developmental scales. The whole assessment performed at the onset of spasms (T 0) was repeated after two months (T 1). AORs resulted significantly impaired both at T 0 and T 1. At the onset of spasms a highly significant relationship of auditory attention with visual function and neurodevelopmental competence was shown in both cryptogenic and symptomatic forms, but it was no longer present after two months. Our results may suggest a possible pervasive effect of the epileptic disorder on sensory processing, associated to a deficit of neurodevelopment. Although we failed to show a significant correlation between auditory orienting responses and EEG patterns, some evidence seems to support at least partially an influence of the epileptic disorder per se on the genesis of the sensorial impairment. A longer follow up and a larger cohort will be useful for a better clarification of these findings. [Copyright &y& Elsevier]

Objective: The aim of this study was to evaluate the epileptic and developmental evolution in infants with West syndrome., Methods: A prospective study of 21 infants was performed, with a follow-up at 2 years. Serial assessment included long-term EEG monitoring, visual and auditory evaluation and assessment of neurodevelopment., Results: Neurosensory and developmental impairments at the spasm onset were transitory in seven cases, including four cryptogenic forms. In all other cases, there was a progressive worsening in neurosensory and developmental impairments. The epileptic evolution was generally better: in 11 of the 16 infants without seizures at outcome, spasms had already disappeared by 2 months after disease onset. Statistic analysis of results showed a correlation between neurosensory impairment and development throughout the whole follow-up. In addition, visual function at T1 resulted significant predictor of developmental outcome. Among the epileptic features, disorganization of slow sleep was an unfavorable prognostic factor., Conclusion: Some forms of West syndrome are confirmed to have a benign evolution: among them there are not only cryptogenic cases but also symptomatic ones without significant neurodevelopmental impairment. Abnormalities of sleep organization, expression of the pervasive epileptic disorder, seem to play a role in determining a developmental deterioration. Neurosensory impairment since the onset of the disease could be a relevant cause of the developmental disorder.

Rationale: Electroencephalography (EEG) was performed at term age on 32 infants born prematurely (25-32 weeks). EEG was assessed looking for overall background activity and transients., Methods: A quantitative analysis was performed, selecting 5-min epochs of "tracé alternant" free of artefacts during quiet sleep. EEG findings were compared with cranial ultrasound (US) findings at term age and with neurodevelopmental outcome at 2 years (Student's t-test)., Results: The overall EEG background activity was not always related to the outcome or to the severity of cranial US. Infants with normal US and normal outcome had longer synchrony percentage of bursts, longer maximum duration of bursts and shorter mean of abnormal transients per interbursts than children with major lesions and abnormal outcome. Infants with minor lesions, who all had normal outcome, also had better results than those with major lesions and abnormal outcome, but the range of the EEG findings was more variable., Conclusion: Our results suggest that the EEG performed at term age does not provide additional prognostic information compared to cranial US.

The aim of this study was to evaluate cognitive development at the onset of West syndrome (WS) with regard to electroencephalogram (EEG) patterns and visual function. Twenty-five patients (14 males, 11 females) at the onset of spasms (T0) in WS and 2 months later (T1) underwent a full clinical evaluation, including neuroimaging, cognitive assessment, video-EEG, and visual function. Mean age of the patients at spasm onset was 5.9 months (SD 2.5; range 2 to 13mo). Cognitive development, assessed with Griffiths Mental Development Scales (GMDS), was generally impaired at T0 and was significantly related to visual function (p<0.001) at both T0 and T1. In general, there was a specific major impairment in the eye-hand coordination scale of the GMDS which tended to disappear after 2 months in less severe cases. At the onset of spasms, sleep EEG organization seemed to be better related to cognitive abilities than awake hypsarrhythmia. These results support a close link between visual function and cognitive competence in WS and provide additional information to improve the understanding of possible mechanisms underlying cognitive impairment.

The aim of this study was to assess various aspects of visual function in 6 patients (age range: 9 months to 7 years and 8 months) with methylmalonic aciduria and homocystinuria. All patients had an ophthalmological examination and were tested with a battery of age-appropriate tests assessing various aspects of visual function such as acuity, visual fields and visual attention. None of the patients had significant retinal abnormalities but all 6 had nystagmus which was associated with strabismus in 3 of the 6. They all had some abnormalities on the behavioral tests assessing visual function which appeared to be related to the age of the patients. Visual impairment was more severe in the 3 patients below 3 years of age and milder in the older patients. The presence and the severity of abnormalities, in contrast, did not depend on the age at onset or the age when treatment was started and were only partly related to brain MRI findings. Severe hydrocephalus and basal ganglia involvement were associated with severe visual impairment, but abnormal visual findings were also present in the children with normal MRI and isolated mild periventricular changes. Our results suggest that age, brain lesions and other factors may be responsible for visual abnormalities in methylmalonic aciduria and homocystinuria. Further studies using early and sequential assessment of visual function are needed to establish whether the differences observed between younger and older children may be related to the duration of therapy.

Epilepsy is relatively common in infants with hydrocephalus. Its mechanism is controversial; in fact, studies on etiologically heterogeneous series are not able to clarify the mechanism generating epilepsy or to suggest effective prevention and treatment strategies. Our study is aimed at assessing the onset and evolution of epilepsy, as well as concurrent cognitive development of a homogeneous series of shunted posthemorrhagic hydrocephalus owing to pre- or perinatal intra- or periventricular hemorrhage. Forty patients were enrolled in the study. Twenty-six were patients with grade II-III intraventricular hemorrhage, 16 of whom had associated ischemic lesions. In the remaining 14 patients, a grade IV intra-ventricular hemorrhage was found. Epilepsy was observed in 27 patients. Aside from 10 cases with nonsyndromic forms of epilepsy, it was possible to define at least three different age-dependent epileptic syndromes: symptomatic neonatal location-related epilepsy with transient West's syndrome in infancy in 5 patients; West's syndrome in 8 patients; and continuous spike-waves during sleep in 4 patients. Epilepsy was significantly correlated with ischemic lesions only. Early thalamic injuries frequently evolved toward continuous spike-waves during sleep, indicating that patients with thalamic injury must be monitored to detect continuous spike-waves during sleep early. Cerebellar atrophy, in addition to epilepsy and other brain injuries, accounted for disorders of cognitive development.

Purpose: Several studies have reported behavioral and electrophysiological evidence of visual impairment during the active stage of West syndrome. The underlying mechanisms are, however, poorly understood, and little has been reported about the correlation between visual impairment, EEG patterns, and brain lesions. The aim of the study was to assess visual function at the onset of spasm and 2 months thereafter and relate visual findings to brain lesions and EEG features., Methods: Twenty-five infants with West syndrome were enrolled and studied with (a) a full clinical assessment including a battery of tests specifically designed to assess visual function, (b) a video-polygraphic study, and (c) brain magnetic resonance imaging (MRI). Besides brain neuroimaging and EEG comparison with visual function, an intra-EEG analysis was performed to investigate the possible relation of EEG patterns to fluctuating visual behavior (fixation and following)., Results: Twenty-two children had at least one abnormal result on one or more of the tests assessing visual function at T0. Visual impairment at the spasm onset was related to the sleep disorganization rather than to the hypsarrhythmic pattern in awake EEG. After 2 months, both EEG features become significantly linked to visual function. Visual function improved in several cases after 2 months, in parallel with the seizure regression. No relation was found between EEG patterns and fluctuating visual behavior., Conclusions: The study supplies new evidence of the involvement of visual function in West syndrome. The presence of abnormal visual findings in infants without lesions on brain MRI suggests that visual abnormalities are due not only to brain injury but also to epileptic disorder per se. New insight is also provided into the possible mechanisms underlying clinical and EEG abnormalities., (Copyright 2004 International League Against Epilepsy)

Purpose: We report the case of a male newborn with Ohtahara syndrome and right hemimegalencephaly who presented epileptic negative myoclonus in the first days of life., Methods: Prolonged polygraphic studies were performed, as well as MRI and a full clinical examination., Results: EEG showed a constant and nonreactive pattern of burst suppression. There were several kinds of electro-clinical seizures (generalized myoclonia, short atonias, typical spasm and tonic spasms) at the beginning of the EEG's burst. The periods of EMG silence, lasting less than 300 ms, were associated with stereotyped EEG transients., Conclusions: Epileptic negative myoclonus can be observed also in neonatal age. The short transient impairment of motor function observed in the newborn seems linked to the slow component of spike-wave discharge, but its mechanism is still not clear.

Purpose: To study prospectively the evolution of visual attention in children with West syndrome to evaluate its development before the onset of spasms, its possible deterioration as a consequence of epileptic disorders, and its outcome at the age of 2 years, and the possible relation between the impairment of visual attention and cognitive development., Methods: Infants with symptomatic West syndrome were examined before the onset of spasms and until age 24 +/- 2 months. Visual attention study (through a clinical observation and the fixation-shift test), cognitive assessment, and complete clinical examination including brain magnetic resonance imaging were performed., Results: A maturation defect of fixation shift skills was generally observed in infants with West syndrome. In some cases, the impaired visual-attention abilities paralleled a cognitive deterioration, even months before the onset of spasms. During the acute stage of West syndrome, infants lost the previously acquired visual and cognitive abilities, with a typical fluctuation of arousal. Usually at 2 years, there was a persistent defective visual attention detected with the fixation-shift test., Conclusions: The parallel defect of visual attention and of cognitive competencies is a constant finding in infants with West syndrome; these can precede the clinical onset of epileptic spasms. The severity and persistence of visual inattention might be explained by the age of visual maturation, corresponding to the usual onset of West syndrome.

We report on periodic lateralized epileptiform discharges (PLEDs) on EEG in two infants with neonatal convulsions. In both neonates, the EEG abnormalities were seen soon after the onset of seizures, at a time when cranial ultrasound scans were thought to be normal. Subsequent Magnetic Resonance Imaging scans demonstrated cerebral infarction in both patients. In one case, the localisation of the lesion on MRI was concordant with that of the EEG abnormalities, as usually observed in adults with focal lesions. The other infant showed similar EEG abnormalities, but her MRI showed a localised lesion in the basal ganglia, which has also been reported to be involved in the genesis of these abnormalities.

The aim of this study was to determine whether perceptual-motor competence in school-age children with Down syndrome was generally delayed or varied as a function of type of action. Twenty-two children with Down syndrome (13 males, 9 females), aged between 4.5 and 14 years were assessed on two standardized tests, the Movement Assessment Battery for Children (Movement ABC), a test of motor competence assessing gross and fine motor coordination, and on the Developmental Test of Visual-Motor Integration, a test focusing on shape copying. In order to obtain a profile of each child's performance on the different items contained in the Movement ABC, the test was used in its extended form. This involves testing any child who failed an item appropriate for his/her level, at progressively lower levels until a base-line measure was obtained. All the children obtained scores below the 5th percentile for their age on both tests. However, superimposed on this delay, we found distinct variation as a function of task. Whereas some aspects of gross motor function showed age development with delayed but regular acquisitions, all the aspects of fine motor skills assessed were more severely impaired and showed little development with age. Accuracy and timing of tasks requiring bimanual coordination were most impaired in our sample while balance and ball skills showed more variability. These results suggest that intervention in the motor domain should be varied according to each child's particular profile of performance.