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This paper describes the psycho-social treatments received by 502 patients with MDS and their relatives, and the costs for care sustained by the families in the previous six month period. Data were collected by the MD-Care Schedule (MD-CS) and the Family Problems Questionnaire (FPQ). Psycho-educational interventions were provided to 72 patients (14.3%), and social/welfare support to 331 patients (65.9%). Social/welfare support was higher in patients with DMD or LGMD, in those showing more severe disability, and in patients who were in contact with centres located in Northern Italy. Psycho-educational interventions were received by 156 (31%) relatives, and social/welfare support by 55 (10.9%) and mainly provided by Family/Patients Associations (83.6%). Relatives with higher educational levels, who spent more daily hours in the assistance of patients with DMD, and in contact with centres in Central Italy more frequently benefited from psycho-educational interventions. In the previous year, costs for care were sustained by 314 (63.9%) relatives. Financial difficulties related to patient's condition, were higher in families of patients who needed more intensive rehabilitation and daily hours of caregiving, and in families who lived further away from the reference's centre. These results showed that psycho-social aspects of MDS care are only partially met in Italy, and that ad hoc supportive interventions for these patients and their families should be potentiated.

Aim. This work reports the analysis of the relation ship between inferior fronto-occipital fasciculus (IFO), neoplastic lesions and surgical resection, in patients operated for gliomas located in the frontal, temporal and insular lobes of the dominant hemisphere. Aim of the study is evaluating the predictive value of inferior fronto-occipital fasciculus DTI-fiber tracking (FT) for determining the extent of resection preoperatively. Methods. We selected 38 cases affected by lesions located in the frontal, temporal and insular lobes of the dominant hemisphere, which were related to the trajectory of the IFO. For each patient preoperative and postoperative MR images and DTI-FT were loaded into the neuronavigation system and merged; volumetric scan analysis was used for establishing tumor location and topography, as well as the volume of the lesion and of the residual tumor. All preoperative fiber tracking dataseis were evaluated and the position of the tract (IFO) compared to the tumor was recorded. Postoperative MR scans were then compared with DTI-FT, in order to evaluate the correspondence between the resection boundaries and the trajectory of the fiber tract. Results. Amongst the cases in which the IFO was inside the lesion, we found only incomplete resections (5 subtotal and 6 partial resections), while considering the cases in which the IFO was located outside the tumor, it was possible to perform a relevant (total/subtotal) resection in 18 of them (78%). Conclusion. FT of the inferior frontal-occipital fasciculus predicts the possibility and the extent of the resection for a frontal, temporal and/or insular lesion of the dominant hemisphere.

We describe how a neuropsychological evaluation in patients with brain tumors should be performed, specifically in the case of low-grade gliomas. Neuropsychological examination is crucial before starting any treatment as well as during the follow-up, since it can improve neurosurgery techniques and reveal potential cognitive effects of chemotherapy and radiotherapy, besides planning rehabilitation. We underline that sensitive and wide-ranging tests are required; specific tasks based on the lesion site should be added. Moreover, some tests can provide additional information about the evolution of the tumor. A careful, thorough examination improves quality of life. © Springer Science+Business Media, LLC. 2012.

The functional role of the uncinate fasciculus is still a matter of debate. We examined 44 patients submitted to awake surgery for removal of a left frontal or temporal glioma. In 18 patients, the removal included the uncinate fasciculus. We compared patients with or without removal on a series of neuropsychological tasks, performed at different time intervals: pre-surgery, in the first week after surgery and 3 months after surgery. Functional magnetic resonance and diffusion tensor imaging, fibre-tracking techniques were performed before surgery. At the last examination, patients with uncinate removal were significantly impaired in naming of famous faces and objects as compared with patients without removal. We further divided patients according to the site of the tumour (either frontal or temporal). At the follow-up, patients with a temporal glioma who underwent uncinate removal had the worst loss of performance in famous face naming. In addition, on the same task, the group with a frontal glioma that underwent resection of the frontal part of the uncinate performed significantly worse than the group with a frontal glioma but without uncinate removal. In conclusion, the resection of the uncinate fasciculus, in its frontal or temporal part, has long-lasting consequences for famous face naming. We suggest that this fibre tract is part of a circuitry involved in the retrieval of word form for proper names. Retrieval of conceptual knowledge was intact. © 2011 The Author.

Low-grade gliomas are slow growing intrinsic lesions that induces a progressive functional reshaping of the brain. Surgical removal of these lesions requires the combined efforts of a multidiscipinary team of neurosurgeon, neuroradiologist, neuropsychologist, neurophysiologist, and neurooncologists that all together contribute in the definition of the location, extension, and extent of functional involvement that a specific lesion has induced in a particular patient. Each tumor has induced particular and specific changes of the functional network, that varies among patients. This requires that each treatment plan should be tailored to the tumor and to the patient. When this is reached, surgery should be accomplished according to functional and anatomical boundaries, and has to aim to the maximal resection with the maximal patient functional preservation. This can be reached at the time of the initial surgery, depending on the functional organization of the brain, or may require additional surgeries, eventually intermingled with adjuvant treatments. The use of so called brain mapping techniques extend surgical indications, improve extent of resection with greater oncological impact, minimization of morbidity and increase in quality of life. To achieve the goal of a satisfactory tumor resection associated with the full preservation of the patients abilities, a series of neuropsychological, neurophysiological, neuroradiological and intraoperative investigations have to be performed. In this chapter, we will describe the rationale, the indications and the modality for performing a safe and rewarding surgical removal of low-grade gliomas by using these techniques, as well as the functional and oncological results.

Low-grade gliomas ([LGGs] WHO Grade II) are slow-growing intrinsic cerebral lesions that diffusely infiltrate the brain parenchyma along white matter tracts and almost invariably show a progression toward malignancy. The treatment of these tumors forces the neurosurgeon to face uncommon difficulties and is still a subject of debate. At the authors' institution, resection is the first option in the treatment of LGGs. It requires the combined efforts of a multidisciplinary team of neurosurgeons, neuroradiologists, neuropsychologists, and neurophysiologists, who together contribute to the definition of the location, extension, and extent of functional involvement that a specific lesion has caused in a particular patient. In fact, each tumor induces specific modifications of the brain functional network, with high interindividual variability. This requires that each treatment plan is tailored to the characteristics of the tumor and of the patient. Consequently, surgery is performed according to functional and anatomical boundaries to achieve the maximal resection with maximal functional preservation. The identification of eloquent cerebral areas, which are involved in motor, language, memory, and visuospatial functions and have to be preserved during surgery, is performed through the intraoperative use of brain mapping techniques. The use of these techniques extends surgical indications and improves the extent of resection, while minimizing the postoperative morbidity and safeguarding the patient's quality of life. In this paper the authors present their paradigm for the surgical treatment of LGGs, focusing on the intraoperative neurophysiological monitoring protocol as well as on the brain mapping technique. They briefly discuss the results that have been obtained at their institution since 2005 as well as the main critical points they have encountered when using this approach.

JavaScript drives the evolution of the web into a powerful application platform. Increasingly, web applications combine services from different providers. The script inclusion mechanism routinely turns barebone web pages into full-fledged services built up from third-party code. Script inclusion poses a challenge of ensuring that the integrated third-party code respects security and privacy. This paper presents a dynamic mechanism for securing script executions by tracking information flow in JavaScript and its APIs. On the formal side, the paper identifies language constructs that constitute a core of JavaScript: dynamic objects, higher-order functions, exceptions, and dynamic code evaluation. It develops a dynamic type system that guarantees information-flow security for this language. Based on this formal model, the paper presents JSFlow, a practical security-enhanced interpreter for fine-grained tracking of information flow in full JavaScript and its APIs. Our experiments with JSFlow deployed as a browser extension provide in-depth understanding of information manipulation by third-party scripts. We find that different sites intended to provide similar services effectuate rather different security policies for the user's sensitive information: some ensure it does not leave the browser, others share it with the originating server, while yet others freely propagate it to third parties.

Colombia is one of the most biodiverse countries in anuran species, however, many of them are in a vulnerable situation. This has the added problem that for most of them the genetic wealth and possible variations between biotopes is unknown. The aim of this study was to characterize the karyotype of Leptodactylus insularum present in a fragment of tropical dry forest of the Caribbean region (Colombia). Was used the technique described for SCHMIDT (1978) and for chromosomal classification parameters used LEVAN et al. (1964). L. insularum presented a modal number of n=11 and 2n=22, with chromosome formula M+4SM+sM+5M. This result varies slightly with respect to the formula of L. insularum present in the Andean region (Colombia) which is 2M+SM+M+ST+M+SM+4M, however, these differences may be associated with biogeographical aspects, so to stay physically separate these populations could have accumulated structural changes in their chromosomes through possible insertion or deletion events. An important aspect is that the specimens that make up this genre generally have a similar chromosome number., Colombia es uno de los países con mayor biodiversidad en especies de anuros, sin embargo, muchos de ellos se encuentran en una situación vulnerable. Esta situación tiene el agravante que para la mayoría de los mismos se desconoce la riqueza genética y las posibles variaciones entre biotopos. El objetivo del presente trabajo fue caracterizar el cariotipo de Leptodactylus insularum presente en un fragmento de BsT la región Caribe (Colombia). Para realizar esto se utilizó técnica de SCHIMID (1978) y para la clasificación cromosomal se utilizó los parámetros de LEVAN et al. (1964). L. insularum presentó un numero modal de n=11 y 2n=22, con formula cromosómica M+4SM+sM+5M. Este resultado varia con respecto a la fórmula del L. insularum presente en la región Andina (Colombia) la cual es 2M+SM+M+ST+M+SM+4M, sin embargo, estas diferencias pueden estar asociadas a aspectos biogeográficos, por lo que al mantenerse separadas físicamente estas poblaciones, podrían haber acumulado mutaciones estructurales en sus cromosomas mediante posibles eventos de inserción o deleción. Un aspecto importante es que los especímenes que componen este género presentan de forma general un similar número cromosómico.

Background: Myosin heavy chain 7 (MYH7)-related myopathies are emerging as an important group of muscle diseases of childhood and adulthood, with variable clinical and histopathological expression depending on the type and location of the mutation. Mutations in the head and neck domains are a well-established cause of hypertrophic cardiomyopathy whereas mutation in the distal regions have been associated with a range of skeletal myopathies with or without cardiac involvement, including Laing distal myopathy and Myosin storage myopathy. Recently the spectrum of clinical phenotypes associated with mutations in MYH7 has increased, blurring this scheme and adding further phenotypes to the list. A broader disease spectrum could lead to misdiagnosis of different congenital myopathies, neurogenic atrophy and other neuromuscular conditions. Results: As a result of a multicenter Italian study we collected clinical, histopathological and imaging data from a population of 21 cases from 15 families, carrying reported or novel mutations in MYH7. Patients displayed a variable phenotype including atypical pictures, as dropped head and bent spine, which cannot be classified in previously described groups. Half of the patients showed congenital or early infantile weakness with predominant distal weakness. Conversely, patients with later onset present prevalent proximal weakness. Seven patients were also affected by cardiomyopathy mostly in the form of non-compacted left ventricle. Muscle biopsy was consistent with minicores myopathy in numerous cases. Muscle MRI was meaningful in delineating a shared pattern of selective involvement of tibialis anterior muscles, with relative sparing of quadriceps. Conclusion: This work adds to the genotype-phenotype correlation of MYH7-relatedmyopathies confirming the complexity of the disorder.

AIM: Surgery for gliomas located inside or in proximity of motor cortex or tracts requires cortical and subcortical mapping to locate motor function; direct electrical stimulation of brain cortex or subcortical pathways allows identification and preservation of motor function. In this study we evaluated the effect which subcortical motor mapping had on postoperative morbidity and extent of resection in a series of patients with gliomas involving motor areas or pathways. METHODS: One hundred and forty-six patients were included in the study. Intraoperative findings of primary motor cortex or subcortical tracts were reported, together with incidence of new postoperative deficits at short (1 week) and long term (1 month) examination. The relationship between intraoperative identification of subcortical motor tracts and extent of resection was reported. RESULTS: The motor strip was found in 133 patients (91%) and subcortical motor tracts in 91 patients (62.3%). New immediate postoperative motor deficits were documented in 59.3% of patients in whom a subcortical motor tract was identified intra-operatively and in 10.9% of those in whom subcortical tracts were not observed; permanent deficits were observed in 6.5% and 3.5%, respectively. A total resection was achieved in 94.4% of patients with high-grade gliomas and in 46.1% of those with low-grade gliomas.

Although the majority of people worldwide are bilingual, the brain representation of language in bilingual persons is still a matter of debate. Since the beginning of the studies conducted on bilinguals, most authors denied that learning a new language requires a new semantic processing or the involvement of new cortical areas. In this paper, we review neurosurgical studies using direct electrocortical or subcortical stimulation techniques for brain mapping in bilingual subjects and compare this data with that obtained from other brain mapping methods. The authors focused on the most controversial issue whether multiple languages are represented in common or distinct cerebral areas. Seven direct brain mapping studies from different teams focused on bilingualism and multilingualism. All these studies showed that even if cerebral representation of language in multilingual patients could be grossly located in the same cortical region, it was possible to individualise distinct language-specific areas by direct cortical stimulation in the dominant frontal and temporo-parietal regions. Task- and language-specific sites were also described, demonstrating an important specialisation of some cortical areas. Using subcortical stimulation, some authors were able to find specific white matter tracts for different languages. Finally, all authors recommend in bilingual patients who need brain mapping for neurosurgical purpose to test all languages in which the subjects are fluent.

The role of the phonological loop in auditory sentence comprehension was examined in a 35-year-old woman with a selective deficit of verbal short-term memory (STM). More specifically, the objective of the experiment was to test whether sentence comprehension is limited by number of propositions, as suggested by Rochon, Waters, and Caplan (2000), or whether it depends on syntactic complexity. In an offline task, severe impairment was present on cleft objects and centre-embedded structures; the deficit on object relatives in right peripheral position was less relevant, and the patient was able to handle sentential coordination easily. In an online task, her processing of centre-embedded structures and object relatives in right peripheral position was significantly slower than that in controls. She was also significantly slower in processing the verb of the object relative in centre-embedded structures. The results obtained do not support the claim that the difficulties encountered by patients with limited working-memory resources are due to the number of propositions in the sentence; they do, however, suggest a direct involvement of the phonological loop in processing syntactically complex sentences.

Surgical treatment for lesions located within or in close proximity of motor areas or pathways requires the identification and preservation of functional cortical and subcortical sites. DTI (diffusion tensor imaging) fiber tracking is a novel technique in MR which allows the identification of white matter tracts and to establish the relationship of the reconstructed tracts with the tumor mass. In this work we described our experience on the use of DTI fiber tracking for motor pathways in series of patients with low or high grade gliomas, who were subsequently submitted to surgery for tumor removal. DTI fiber tracking was incorporated in the presurgical procedures, and used either in the presurgical planning or during surgery, in combination with subcortical mapping. The usefulness of the technique and the actual limitations encountered when it is associated with subcortical motor mapping at the time of surgery are described and discussed.

Functional brain mapping is performed during resection of intracerebral tumors involving motor regions to identify cortical areas and subcortical tracts evoking motor responses when electrically stimulated. In this study we describe the parallel recording of multiple motor evoked responses with electromyographic technique and of cerebral activity with electrocorticographic and electroencephalographic techniques, as a guide for the neurosurgeon relying onto motor brain mapping during resection of intracerebral tumors.