Methodological approach to spatial analysis of agricultural pest dispersal in olive landscapes.

The effectiveness of a Geographical Information Systems cost-distance tool for detecting landscape permeability in relation to the movement of pests in olive landscapes was established. The simplification of agricultural systems is linked to an increased incidence of pests on crops. Therefore, it is important to understand the impact of different land uses surrounding olive groves on pests. In this work, we analysed the effect of the structure of the olive landscape on the movement of two main olive pests-the olive fruit fly, Bactrocera oleae (Rossi) (Diptera: Tephritidae) and the olive moth, Prays oleae (Bernard) (Lepidopetera: Praydidae). We applied linear mixed effects models to analyse the relationship between pest abundance and cost-distance, using different hypotheses to evaluate those land uses that are favourable or unfavourable for the movement of these pests. The results show that this methodology is effective in detecting possible unfavourable land uses with a barrier effect, such as woodland and artificial land uses, and favourable land uses with a corridor effect such as olive groves. Whether other land uses, such as scrubland or riverbanks, act as a barrier or corridor depends on the pest and its life cycle stage. The effect that different land uses have in maintaining low levels of pest populations and ensuring the long-term sustainability of these agricultural systems are discussed. The implications of landscape permeability for the physical structure of the landscape and the dispersal of organisms, and the potential of that landscape to impact the continuous flow of natural processes are also addressed.

Bi-allelic mutations in EGR2 cause autosomal recessive demyelinating neuropathy by disrupting the EGR2-NAB complex.

BACKGROUND AND PURPOSE: Mutations in the early growth response 2 gene (EGR2) cause demyelinating, but also axonal, neuropathies differing in severity and age of onset. Except for one family, all reported cases have autosomal dominant inheritance and mutations are localized within the three zinc finger (ZNF) DNA-binding domain. The aim of this study was to provide a clinical and molecular analysis of a novel recessive mutation in EGR2. METHODS: Clinical and electrophysiological assessments of three affected patients, from a consanguineous family, were performed. Genetic analyses of EGR2 were carried out by Sanger sequencing. Functional effects of clinical recessive mutations were assessed using a mammalian two-hybrid assay. RESULTS: A novel missense mutation (c.791C>T; p.P264L) in the homozygous state was detected outside the ZNF domains of the EGR2 gene. Three affected siblings presented with distal demyelinating polyneuropathy with severe sensory loss, progressive thoracolumbar scoliosis and trigeminal neuralgia. Respiratory compromise and cranial nerve dysfunction were also found. Our data indicate that the p.P264L mutation prevents interaction of EGR2 transcription factor with NAB corepressors, suggesting that a disruption of the NAB-EGR2 protein interactions can result in dramatic neuropathy. CONCLUSION: Mutations in, or next to, the R1 domain of EGR2 should be considered with extreme caution for genetic counseling, since these could cause a severe neuropathy with an autosomal recessive manner of transmission.

PLA2G6 mutations associated with a continuous clinical spectrum from neuroaxonal dystrophy to hereditary spastic paraplegia.

PLA2G6-associated neurodegeneration (PLAN) and hereditary spastic paraplegia (HSP) are 2 groups of heterogeneous neurodegenerative diseases. In this study, we report PLA2G6 gene mutations in 3 families from Turkey, Morocco, and Romania. Two affected Turkish siblings presenting HSP adds the disease to PLAN phenotypes. They were homozygous for the PLA2G6 missense c.2239C>T, p.Arg747Trp variant and the ages of onset were 9 and 21. Parkinsonism, dystonia or cognitive decline were not the clinical elements in these patients contrary to the cases that has been previously reported with the same variant, however, iron accumulation was evident in their cranial magnetic resonance imaging. The Moroccan patient was homozygous for a novel missense c.1786C>T, p.Leu596Phe variant and the Romanian patient had 2 novel mutations; c.1898C>T, p.Ala633Val and c.1765_1768del, p.Ser589ThrfsTer76. Both of these patients conformed better to childhood onset PLAN with the age of onset at 4 and 7 years, respectively. Interestingly, all identified mutations were affecting the highly conserved patatin-like phospholipase domain of the PLA2G6 protein.

European consensus for starting and stopping enzyme replacement therapy in adult patients with Pompe disease: a 10-year experience.

BACKGROUND AND PURPOSE: Pompe disease is a rare inheritable muscle disorder for which enzyme replacement therapy (ERT) has been available since 2006. Uniform criteria for starting and stopping ERT in adult patients were developed and reported here. METHODS: Three consensus meetings were organized through the European Pompe Consortium, a network of experts from 11 European countries in the field of Pompe disease. A systematic review of the literature was undertaken to determine the effectiveness of ERT in adult patients on a range of clinical outcome measures and quality of life. A narrative synthesis is presented. RESULTS: Consensus was reached on how the diagnosis of Pompe disease should be confirmed, when treatment should be started, reasons for stopping treatment and the use of ERT during pregnancy. This was based on expert opinion and supported by the literature. One clinical trial and 43 observational studies, covering a total of 586 individual adult patients, provided evidence of a beneficial effect of ERT at group level. At individual patient level, the response to treatment varied, but factors associated with a patient's response to ERT were not described in many studies. Eleven observational studies focused on more severely affected patients, suggesting that ERT can also be beneficial in these patients. There are no studies on the effects of ERT in pre-symptomatic patients. CONCLUSIONS: This is the first European consensus recommendation for starting and stopping ERT in adult patients with Pompe disease, based on the extensive experience of experts from different countries.

Microsporidians xenomas of anglerfish from NE Atlantic waters.

The presence of emergent visible parasites at commercial valuable fish species is increasingly causing problems at fisheries and seafood industries. Microsporidians have been previously reported to appear forming apparent xenomas complexes in anglerfish species, but no effort has been carried out to simultaneously integrate epidemiological data, phenotypic, genotypic and fine structural characterizations in the same parasite sample. In this work, specimens of Lophius budegassa and Lophius piscatorius from NE Atlantic waters were sampled and examined to provide information about specific site of infection and demographic data of two groups of different sizes of xenomas present at both fish species. Histological descriptions and scanning and transmission electron microscopy were carried out on fresh spores of Lophius budegassa for ultrastructural studies. In both types of xenomas, it was observed simultaneously the microsporidian genus Spraguea in the form of two different types of spores. Molecular analyses of both xenomas from the two fish species, based on the small subunit ribosomal DNA gene, were also performed to genetically support the morphological diagnostic provided.

Ischaemic stroke in children with cardiopathy: An epidemiological study. / Ictus isquémico en niños con cardiopatía: estudio epidemiológico.

INTRODUCTION: Ischaemic stroke is rare during childhood. Congenital and acquired heart diseases are one of the most important risk factors for arterial ischaemic stroke (AIS) in children. PATIENTS AND METHODS: We conducted a retrospective study of all children with AIS and heart disease diagnosed between 2000 and 2014. RESULTS: We included 74 children with heart disease who were eligible for inclusion. 60% were boys with a mean stroke age of 11 months. 20% of the patients died during the study period. 90% of the patients had a congenital heart disease, while cyanotic heart disease was identified in 60%. Hypoplastic left heart syndrome was the most frequent heart disease. In 70% of patients AIS was directly associated with heart surgery, catheterisation or ventricular assist devices. Most patients with AIS were in the hospital. Seizures and motor deficit were the most frequent symptoms. Most patient diagnoses were confirmed by brain CT. The AIS consisted of multiple infarcts in 33% of the cases, affected both hemispheres in 27%, and involved the anterior and posterior cerebral circulation in 10%. CONCLUSIONS: Arterial ischaemic strokes were mainly associated with complex congenital heart diseases, and heart procedures and surgery (catheterisation). AIS presented when patients were in-hospital and most of the patients were diagnosed in the first 24hours.

Prognostic value of brachioradialis muscle oxygen saturation index and vascular occlusion test in septic shock patients.

OBJECTIVES: To compare rSO2 (muscle oxygen saturation index) static and dynamic variables obtained by NIRS (Near Infrared Spectroscopy) in brachioradialis muscle of septic shock patients and its prognostic implications. DESIGN: Prospective and observational study. SETTING: Intensive care unit. SUBJECTS: Septic shock patients and healthy volunteers. INTERVENTIONS: The probe of a NIRS device (INVOS 5100) was placed on the brachioradialis muscle during a vascular occlusion test (VOT). VARIABLES: Baseline, minimum and maximum rSO2 values, deoxygenation rate (DeOx), reoxygenation slope (ReOx) and delta value. RESULTS: Septic shock patients (n=35) had lower baseline rSO2 (63.8±12.2 vs. 69.3±3.3%, p<0.05), slower DeOx (-0.54±0.31 vs. -0.91±0.35%/s, p=0.001), slower ReOx (2.67±2.17 vs. 9.46±3.5%/s, p<0.001) and lower delta (3.25±5.71 vs. 15.1±3.9%, p<0.001) when compared to healthy subjects (n=20). Among septic shock patients, non-survivors showed lower baseline rSO2 (57.0±9.6 vs. 69.8±11.3%, p=0.001), lower minimum rSO2 (36.0±12.8 vs. 51.3±14.8%, p<0.01) and lower maximum rSO2 values (60.6±10.6 vs. 73.3±11.2%, p<0.01). Baseline rSO2 was a good mortality predictor (AUC 0.79; 95%CI: 0.63-0.94, p<0.01). Dynamic parameters obtained with VOT did not improve the results. CONCLUSION: Septic shock patients present an important alteration of microcirculation that can be evaluated by NIRS with prognostic implications. Monitoring microvascular reactivity in the brachioradialis muscle using VOT with our device does not seem to improve the prognostic value of baseline rSO2.

Macroparasites of allis shad (Alosa alosa) and twaite shad (Alosa fallax) of the Western Iberian Peninsula Rivers: ecological, phylogenetic and zoonotic insights.

Samples of anadromous Alosa alosa (Clupeidae) (n = 163) and Alosa fallax (Clupeidae) (n = 223), caught in Western Iberian Peninsula Rivers from 2008 to 2013, were examined for buccal, branchial and internal macroparasites, which were identified using morphological and molecular methods. Alosa alosa were infected with Anisakis simplex s.s., Anisakis pegreffii, Hysterothylacium aduncum, Rhadinorhynchus pristis, Mazocraes alosae, Hemiurus appendiculatus, Ceratothoa italica and an unidentified ergasilid copepod. Ceratothoa italica represents a new host record for A. alosa. Alosa fallax were infected with A. simplex s.s., A. pegreffii, H. aduncum, H. appendiculatus, Clavellisa emarginata and an unidentified cymothoid isopod. This is the first report of C. italica, C. emarginata and M. alosae in the Iberian Peninsula. The phylogenetic positions of M. alosae, H. appendiculatus and C. emarginata were assessed using 18S and 28S ribosomal RNA (rRNA); our contributions provide a better understanding of the phylogenetic relationships within their groups. Qualitative and quantitative differences in the parasite faunas of these two shad species are consistent with different feeding strategies. The results provide information about host migration behaviour and transmission pathways through diet during the marine trophic phase of the shad's life cycle and their roles as paratenic or final hosts and transporters of parasites between seawater and freshwater environments. The zoonotic parasites A. simplex s.s. and A. pegreffii pose a risk for consumers or riverine mammals (e.g. European otter). The use of parasites as biological tags for shad stocks in Western Iberian Rivers could be a useful approach in multidisciplinary studies concerning fish stock delimitation and characterization.

Anisakis infection in allis shad, Alosa alosa (Linnaeus, 1758), and twaite shad, Alosa fallax (Lacépède, 1803), from Western Iberian Peninsula Rivers: zoonotic and ecological implications.

Spawning individuals of allis shad, Alosa alosa (Linnaeus, 1758), and twaite shad, Alosa fallax (Lacépède, 1803), were sampled from three rivers on the Atlantic coast of the Iberian Peninsula (Ulla, Minho, Mondego) during 2008 to 2013 to assess the presence of the zoonotic marine parasite Anisakis spp. larvae. The results revealed that both shad species were infected by third-larval stage Anisakis simplex s.s. and Anisakis pegreffii. The latter is reported in mixed infections in both shad species of Western Iberian Peninsula for the first time. In A. alosa, the prevalence of Anisakis infection can reach 100%, while in A. fallax, prevalence was up to 83%. Infected individuals of the former species also often contain much higher number of parasites in theirs internal organs and flesh: from 1 to 1138 Anisakis spp. larvae as compared to 1 to 121 larvae, respectively. In general, numbers of A. pegreffii were higher than those of A. simplex s.s. Our results suggest that in the marine environment of the Western Iberian Peninsula, both anadromous shad species act as paratenic hosts for A. simplex s.s. and A. pegreffii, thus widening the distribution of the infective nematode larvae from the marine to the freshwater ecosystem. This finding is of great epidemiological relevance for wildlife managers and consumers, considering the zoonotic and gastroallergic threats posed of these parasites.

Regional oxygen saturation index (rSO2) in brachioradialis and deltoid muscle. Correlation and prognosis in patients with respiratory sepsis.

OBJECTIVE: To compare oxygen saturation index (rSO2) obtained simultaneously in two different brachial muscles. DESIGN: Prospective and observational study. SETTING: Intensive care unit. PATIENTS: Critically ill patients with community-acquired pneumonia. INTERVENTIONS: Two probes of NIRS device (INVOS 5100) were simultaneously placed on the brachioradialis (BR) and deltoid (D) muscles. VARIABLES: rSO2 measurements were recorded at baseline (ICU admission) and at 24h. Demographic and clinical variables were registered. Pearson's correlation coefficient was used to assess the association between continuous variables. The consistency of the correlation was assessed using the intraclass correlation coefficient (ICC) and Bland-Altman plot. The predictive value of the rSO2 for mortality was calculated by ROC curve. RESULTS: Nineteen patients were included with an ICU mortality of 21.1%. The rSO2 values at baseline and at 24h were significantly higher in D than in BR muscle. Values obtained simultaneously in both limbs showed a strong correlation and adequate consistency: BR (r=0.95; p<0.001; ICC=0.94; 95% CI: 0.90-0.96; p<0.001), D (r=0.88; p=0.01; ICC=0.88; 95% CI: 0.80-0.90; p>0.001) but a wide limit of agreement. Non-survivors had rSO2 values significantly lower than survivors at all times of the study. No patient with rSO2 >60% in BR died, and only 17.6% died with an rSO2 value >60% in D. Both muscles showed consistent discriminatory power for mortality. CONCLUSION: Both BR and D muscles were appropriate for measuring rSO2.

Beyond biomass production: Enhancing biodiversity while capturing carbon in short rotation coppice poplar plantations.

Biodiversity is essential for the functioning of ecosystems and the provision of services. In recent years, the role of plantations in mitigating climate change through carbon sequestration has been highlighted. In the Mediterranean area, high-density poplar plantations in short-rotation with resprouting management (SRC) have been established for biomass purposes on mostly irrigated agricultural land, coexisting with rainfed and irrigated agricultural crops. This study aims to assess the contribution of these plantations to this type of agroforest ecosystem in terms of biodiversity. For this purpose, both flora and fauna diversity were evaluated both within and outside of the plantation. Additionally, the accumulated carbon in the biomass, as well as in the accompanying vegetation within the plantation, was assessed. Different indices were used to evaluate both the intrinsic diversity of the forest plantation and the degree of substitution and complementarity between the different communities of the landscape. Our findings reveal distinct biodiversity patterns in the land-use scenarios sampled. Specifically, we observed significantly higher flora-species richness in SRC plantations than in the adjacent agricultural land, whereas fauna richness showed a similar but slightly higher level in the forested area. A moderate level of complementarity between land uses was found for insects and mammals (around 45 %), contrasting with high complementarity for birds (87 %) and flora (90 %). This suggests substantial turnover and replacement among these ecological environments. Our results indicate that a second rotation (4 year) plantation could accumulate a total of 61.6 Mg C ha-1, and even though adventitious flora represents <2 % of the total carbon accumulated, its importance in providing ecosystem services is considerable. Hence, these findings evidence the fact that SRC poplar plantations can enhance biodiversity in Mediterranean agroforest ecosystems and actively contribute to various provisioning ecosystem services, including carbon sequestration, reflecting a multi-objective approach that extends beyond biomass production.

Clinical practice guidelines for the diagnosis and management of Charcot-Marie-Tooth disease.

INTRODUCTION: Charcot-Marie-Tooth disease (CMT) is classified according to neurophysiological and histological findings, the inheritance pattern, and the underlying genetic defect. The objective of these guidelines is to offer recommendations for the diagnosis, prognosis, follow-up, and treatment of this disease in Spain. MATERIAL AND METHODS: These consensus guidelines were developed through collaboration by a multidisciplinary panel encompassing a broad group of experts on the subject, including neurologists, paediatric neurologists, geneticists, physiatrists, and orthopaedic surgeons. RECOMMENDATIONS: The diagnosis of CMT is clinical, with patients usually presenting a common or classical phenotype. Clinical assessment should be followed by an appropriate neurophysiological study; specific recommendations are established for the parameters that should be included. Genetic diagnosis should be approached sequentially; once PMP22 duplication has been ruled out, if appropriate, a next-generation sequencing study should be considered, taking into account the limitations of the available techniques. To date, no pharmacological disease-modifying treatment is available, but symptomatic management, guided by a multidiciplinary team, is important, as is proper rehabilitation and orthopaedic management. The latter should be initiated early to identify and improve the patient's functional deficits, and should include individualised exercise guidelines, orthotic adaptation, and assessment of conservative surgeries such as tendon transfer. The follow-up of patients with CMT is exclusively clinical, and ancillary testing is not necessary in routine clinical practice.

SPG7 mutational screening in spastic paraplegia patients supports a dominant effect for some mutations and a pathogenic role for p.A510V.

Mutations in the SPG7 gene were initially reported in patients with autosomal recessive hereditary spastic paraplegia (HSP). Recent works suggested a dominant effect for some SPG7 mutations. To characterize the SPG7 mutational spectrum in a large cohort of Spanish HSP patients, we sequenced the whole SPG7 gene in a total of 285 Spastic Paraplegia patients. Large gene rearrangements were also ascertained in some patients. We found a total of 14 SPG7 mutations (12 new) in 14 patients; 2 were large deletions. All the mutation carriers had an adult onset age but only five (35%) had a complicated phenotype. We identified a single mutation in 13 patients. Familial analysis suggested a dominant inheritance for one (p.Leu78*) of these mutations. Carriers of the rare p.A510V variant were significantly more frequent in patients vs healthy controls (3% vs 1%), suggesting a pathogenic role for this SPG7 variant. We reported a high frequency of patients with only one SPG7 mutation, and a putative pathogenic role for the p.A510V variant.

Nyctiphanes couchii as intermediate host for Rhadinorhynchus sp. (Acanthocephala, Echinorhynchidae) from NW Iberian Peninsula waters.

In the mesozooplanktonic community of the coastal upwelling system of the Ría de Vigo (NW Spain), the euphausiid Nyctiphanes couchii has been identified for the first time in temperate waters of the NE Atlantic as the intermediate host for cystacanths of Rhadinorhynchus sp. Parasites were identified using morphological characters described in 20 cystacanths. The hooks of the proboscis were arranged in 14 rows of 26 hooks each, while the hooks of the basal circle were only slightly erected and were longer than remaining spines. A maximum-likelihood estimation (ML) tree inferred from the 18S rRNA data set of Palaeacantocephala revealed that our specimens belong to a highly supported clade with Rhadinorhynchus sp., Pararhadinorhynchus sp. and Transvena annulospinosa. Nonetheless, our morphological and phylogenetic analyses suggested that the status of Rhadinorhynchus pristis should be re-examined. The prevalences of parasites were 0.0019% and 0.0001% for frontal and coastal summer communities, and 0.0068% and 0.0008% for coastal and oceanic autumn communities, respectively. The presence of these cystacanths in different mesozooplankton communities throughout the study suggests that the recruitment of parasites may be affected by the oceanography.

Significance of tuber size for complications of tuberous sclerosis complex.

INTRODUCTION: Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. OBJECTIVE: The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. MATERIAL AND METHODS: We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. RESULTS: Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to-average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. CONCLUSIONS: Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous.

Molecular characterization of Maccallumtrema xiphiados (Trematoda: Azygiida) and Molicola sp. (Cestoda: Trypanorhyncha) infecting commercial frozen slices of Atlantic swordfish.

Muscle gross lesions, associated to parasites, were routinely found during self-inspection in a Spanish fish plant processing Atlantic swordfish. To determine the taxonomic status of these parasites, molecular analysis was performed based on 18S, ITS1, 5.8S, ITS2 and 28S rDNA sequences, obtaining a consensus sequences of 4581 bp for the cestode and 4200 bp for the trematode. Taxonomic affiliation was determined by phylogenetic analysis of combined SSU + LSU rDNA regions using maximum likelihood models. Molecular characterization allows us to identify the trematode Maccallumtrema xiphiados and the cestode Molicola sp. infecting the musculature of the Atlantic swordfish. Both parasites are responsible of significant economic loss to fish industry due to commercial rejection of parasitized products.

Self-perception of primary health-care staff about physical activity on prescription: A qualitative semi-structured interview.

OBJECTIVE: This study aimed to analyze the self-perception at primary health-care (PHC) nurses and general practitioners (GPs) toward PAP implementation in PHC centers. MATERIAL AND METHODS: Two semi-structured group interviews were performed separately, with five GPs and nurses working in the PHC system in the region of Madrid (Spain). An expert psychologist guided each semi-structured session. The interviews were transcribed verbatim and consensually analyzed using a content analysis. RESULTS: Half of the PHC staff considered themselves physically active and were convinced that physically active staff behavior could facilitate PAP with patients. Both GPs and nurses showed a lack of knowledge of exercise prescription but were interested in PAP and motivational training courses, as well as leadership or to collaborate under a multidisciplinary or interdisciplinary PAP approach. Some of the most relevant self-perceived PAP barriers were a confident method to measure sedentary and physical activity levels. Besides lack of staff awareness, time of consultation, and improving local community relationships and PAP policies strategies. CONCLUSIONS: There are some common self-perceptions, barriers, and facilitators among PHC nurses and GPs for PAP implementation. Following a socio-ecologic approach, this organizational data provides further insight to design a future cost-effective policy strategy to improve patient health and health-care system sustainability.

Nyctiphanes couchii as intermediate host for the acanthocephalan Bolbosoma balaenae in temperate waters of the NE Atlantic.

Cystacanths of the acanthocephalan Bolbosoma balaenae (Gmelin, 1790) were found encapsulated in the cephalothorax of the euphausiid Nyctiphanes couchii (Bell, 1853) from temperate waters in the NE Atlantic Ocean. Euphausiids were caught in locations outside the Ría de Vigo in Galicia, NW Spain, and prevalence of infection was up to 0.1%. The parasite was identified by morphological characters. Cystacanths were 8.09 ± 2.25 mm total length (mean ± SD) and had proboscises that consisted of 22 to 24 longitudinal rows of hooks, each of which had 8 or 9 hooks per row including 2 or 3 rootless ones in the proboscis base and 1 field of small hooks in the prebulbar part. Phylogenetic analyses of 18S rDNA and cytocrome c oxidase subunit I revealed a close relationship with other taxa of the family Polymorphidae (Meyer, 1931). The results extend northwards ot the known distribution of B. balaenae. Taxonomic affiliation of parasites and trophic ecology in the sampling area suggest that N. couchii is the intermediate host for B. balenae, and we suggest that the whales Balaenoptera physalus (Linnaeus, 1758) and B. acutorostrata (Lacepède, 1804) are its definitive hosts. This life cycle is probably completed with or without paratenic hosts.

Schizencephaly: a study of 16 patients.

OBJECTIVE: To present 16 patients with schizencephaly and neurological involvement, and analyse their characteristics and neuroimages. MATERIAL AND METHODS: The study included 16 patients, 8 males and 8 females, all of whom were diagnosed with schizencephaly at less than 3 years of age; 2 patients were diagnosed prenatally. Schizencephaly was identified by computerized tomography (CT) in 1 patient and by MR or three-dimensional MR (3DMR) with a 1.5tesla apparatus in the others. Most patients were referred for evaluation because of psychomotor delay, motor disabilities and/or seizures. RESULTS: Five patients had bilateral schizencephaly with open lips (2 of them had suffered intrauterine cytomegalovirus infections); 2 showed unilateral schizencephaly with separated lips, 8 presented unilateral schizencephaly with fused lips, and 1 had schizencephaly with open lips on one side and fused lips on the other. Prenatal cytomegalovirus infection was diagnosed in 2 patients. A cerebral malformation that affected the midline was diagnosed by routine ultrasound studies in 2 patients. Eight patients (50%) presented with seizures that were focal, except for one patient who showed secondary generalisation. The latter was the only patient whose disease was refractory to complete seizure control with antiepileptic medication. All patients had some degree of motor deficit, which was either unilateral (hemiparesis) or bilateral (tetraparesis). CONCLUSION: 3D MR imaging was very important in diagnosing of schizencephaly in our patients because it showed the polymicrogyria that covered the area of the cleft and permitted us to rule out porencephaly. Neuronal migration disorders such as heterotopias and, more frequently, cortical dysplasias, were observed in several patients. Half of the patients had epilepsy which was controlled with antiepileptic medication, except in 1 patient.

[Congenital cytomegalovirus infection and cortical/subcortical malformations]. / Citomegalia congénita y malformaciones corticales y subcorticales.

INTRODUCTION: Intrauterine infection due to cytomegalovirus is the most common of the intrauterine viral/parasitic infections that affect the central nervous system and cause permanent lesions in the cortex as well as the subcortical white matter. Studies using brain magnetic resonance imaging (MRI) are limited. MATERIAL AND METHODS: Six patients (4 females and 2 males) were studied in the first months of life in order to make a diagnosis of congenital cytomegalovirus, and identify the cortical and subcortical lesions using the necessary MRI sequences. RESULTS: The six patients showed malformations of cortical development (MDC) (schizencephaly, polymicrogyria or lissencephaly-pachygyria) from the neonatal period, and diffuse changes of the white matter, which remained with few changes during the first two years. They then began reducing in size in the form of high signal areas in T2, restricted to certain areas, and were evident for a few years more with little change. CONCLUSION: Intrauterine infection due to cytomegalovirus causes changes in the cortical grey matter, which consists of MDC, and in the subcortical white matter. The latter show a changing aspect as they appear as diffuse and wide areas of high signal intensity, which is usually due to delay in myelinisation, but could also be caused directly by the cytomegalovirus. These changes in the white matter are subjected to morphological changes throughout the first years of life, leading to brain atrophy. The neurological sequelae of these lesions left by these alterations are severe and chronic.