Aproximación clínica al conocimiento de las funciones ejecutivas de un grupo de antiguos consumidores de sustancias psicoactivas

L'estudi intenta descriure la simptomatologia disejecutiva així com les característiques socials, toxicològiques i mèdiques d'un grup de pacients que estan rebent tractament farmacològic per a la seva addicció en una unitat de conductes addictives de València i que, romanent abstinents, presenten deteriorament cognitiu segons la impressió clínica del facultatiu. Els instruments d'avaluació utilitzats són la història clínica, el mini-examen cognoscitiu de Lobo, el qüestionari disejecutiu DEX-Sp, l'escala de comportament del sistema frontal (FrSBe-Sp), el test de la figura complexa de rei, el test de Stroop , diferents subtest d’intel•ligència per a adults del WAIS III i l'escala EEAG.

Reversible acquired epileptic frontal syndrome and CSWS suppression in a child with congenital hemiparesis treated by hemispherotomy.

A boy with a right congenital hemiparesis due to a left pre-natal middle cerebral artery infarct developed focal epilepsy at 33 months and then an insidious and subsequently more rapid, massive cognitive and behavioural regression with a frontal syndrome between the ages of 4 and 5 years with continuous spike-waves during sleep (CSWS) on the EEG. Both the epilepsy and the CSWS were immediately suppressed by hemispherotomy at the age of 5 years and 4 months. A behavioural-cognitive follow-up prior to hemispherotomy, an per-operative EEG and corticography and serial post-operative neuropsychological assessments were performed until the age of 11 years. The spread of the epileptic activity to the "healthy" frontal region was the cause of the reversible frontal syndrome. A later gradual long-term but incomplete cognitive recovery, with moderate mental disability was documented. This outcome is probably explained by another facet of the epilepsy, namely the structural effects of prolonged epileptic discharges in rapidly developing cerebral networks which are, at the same time undergoing the reorganization imposed by a unilateral early hemispheric lesion. Group studies on the outcome of children before and after hemispherectomy using only single IQ measures, pre- and post-operatively, may miss particular epileptic cognitive dysfunctions as they are likely to be different from case to case. Such detailed and rarely available complementary clinical and EEG data obtained in a single case at different time periods in relation to the epilepsy, including per-operative electrophysiological findings, may help to understand the different cognitive deficits and recovery profiles and the limits of full cognitive recovery.

Further characterization of the executive memory impairment following frontal lobe lesions.

In this article we describe a 41-year-old man who, following an operation to repair a ruptured anterior communicating artery aneurysm, manifested the "hallmark" features of a dysexecutive memory impairment. Of particular note was the patient's apparently normal level of recognition memory but impaired recall on tasks matched for difficulty in control subjects. However, further testing revealed that the patient's recognition memory was not normal under all circumstances. Implications of these data for the interpretation and further investigation of the dysexecutive deficit are discussed.

Reversible acquired epileptic frontal syndrome and CSWS suppression in a child with congenital hemiparesis treated by hemispherotomy

Rapport de synthèse : Cette thèse a étudié en détail le cas d'un enfant souffrant d'une hémiplégie congénitale sur un infarctus prénatal étendu qui a développé une forme particulière d'épilepsie, le syndrome des pointes ondes continues du sommeil (POCS), associé à une régression mentale massive. Les caractéristiques de cette détérioration pointaient vers un dysfonctionnement de type frontal. Une chirurgie de l'épilepsie (hémisphérotomie) a, non seulement, permis la guérison de l'épilepsie mais une récupération rapide sur le plan comportemental et cognitif, suivie d'une reprise plus lente du développement, avec finalement à l'âge de 11 ans un niveau de déficience intellectuelle modérée. L'intérêt de cette étude réside dans le fait que l'enfant a pu être suivi prospectivement entre l'âge de 4.5 ans et 11 ans par des enregistrements électro-encéphalographiques (EEG) ainsi que des tests neuropsychologiques et des questionnaires de comportements sériés, permettant de comparer les périodes pré-, péri- et postopératoires, ce qui est rarement réalisable. Un enregistrement EEG de surface a même pu être effectué durant l'opération sur l'hémisphère non lésé, permettant de documenter l'arrêt des décharges épileptiformes généralisées dès la fin de l'intervention. L'hypothèse que nous avons- souhaité démontrer est que la régression comportementale et cognitive présentée par l'enfant après une période de développement précoce presque normale (retard de langage) était de nature épileptique : nous l'expliquons par la propagation de l'activité électrique anormale à partir de la lésion de l'hémisphère gauche vers les régions préservées, en particulier frontales bilatérales. L'hémisphérotomie a permis une récupération rapide en déconnectant l'hémisphère gauche lésé et épileptogène de l'hémisphère sain, qui a ainsi pu reprendre les fonctions cognitives les plus importantes. Les progrès plus lents par la suite et l'absence de rattrapage au delà d'un niveau de déficience mentale modérée sont plus difficiles à expliquer: on postule ici un effet de l'épilepsie sur le développement de réseaux neuronaux de l'hémisphère initialement non lésé, réseaux qui sont à la fois à un stade précoce de leur maturation et en cours de réorganisation suite à la lésion prénatale. La littérature sur les déficits cognitifs avant et après hemisphérotomie s'est surtout préoccupée du langage et de sa récupération possible. À notre connaissance, notre étude est la première à documenter la réversibilité d'une détérioration mentale avec les caractéristiques d'un syndrome frontal après hémisphérotomie. La chirurgie de l'épilepsie a offert ici une occasion unique de documenter le rôle de l'activité épileptique dans la régression cognitive puisqu'en interrompant brusquement la propagation de l'activité électrique anormale, on a pu comparer la dynamique du développement avant et après l'intervention. La mise en relation des multiples examens cliniques et EEG pratiqués chez un seul enfant sur plusieurs années a permis d'obtenir des informations importantes dans la compréhension des troubles cognitifs et du comportement associés aux épilepsies focales réfractaires. ABSTRACT : A boy with a right congenital hemiparesis due to a left pre-natal middle cerebral artery infarct developed focal epilepsy at 33 months and then an insidious and subsequently more rapid, massive cognitive and behavioural regression with a frontal syndrome between the ages of 4 and 5 years with continuous spike-waves during sleep (CSWS) on the EEG. Both the epilepsy and the CSWS were immediately suppressed by hemispherotomy at the age of 5 years and 4months. A behavioural-cognitive follow-up prior to hemispheratomy, an per-operative EEG and corticography and serial post-operative neuropsychological assessments were performed until the age of 11 years. The spread of the epileptic activity to the "healthy" frontal region was the cause of the reversible frontal syndrome. A later gradual long-term but incomplete cognitive recovery, with moderate mental disability was documented. T9ris outcome is probably explained by another facet of the epilepsy, namely the structural effects of prolonged epileptic dischazges in rapidly developing cerebral networks which are, at the same time undergoing the reorganization imposed by a unilateral early hemispheric lesion. Group studies on the outcome of children before and after hemispherectomy using only single IQ measures, pre- and postoperatively, may miss particular epileptic cognitive dysfunctions as they are likely to be different from case to case. Such detailed and rarely available complementary clinical and EEG data obtained in a single case at different time periods in relation to the epilepsy, including peroperative electrophysiological findings, may help to understand the different cognitive deficits and recovery profiles and the limits of full cognitive recovery.

Paranasal sinuses in children: size evaluation of maxillary, sphenoid, and frontal sinuses by magnetic resonance imaging and proposal of volume index percentile curves.

Our objective was to establish the age-related 3D size of maxillary, sphenoid, and frontal sinuses. A total of 179 magnetic resonance imaging (MRI) of children under 17 years (76 females, 103 males) were included and sinuses were measured in the three axes. Maxillary sinuses measured at birth (mean+/-standard deviation) 7.3+/-2.7 mm length (or antero-posterior)/4.0+/-0.9 mm height (or cranio-caudal)/2.7+/-0.8 mm width (or transverse). At 16 years old, maxillary sinus measured 38.8+/-3.5 mm/36.3+/-6.2 mm/27.5+/-4.2 mm. Sphenoid sinus pneumatization starts in the third year of life after conversion from red to fatty marrow with mean values of 5.8+/-1.4 mm/8.0+/-2.3 mm/5.8+/-1.0 mm. Pneumatization progresses gradually to reach at 16 years 23.0+/-4.5 mm/22.6+/-5.8 mm/12.8+/-3.1 mm. Frontal sinuses present a wide variation in size and most of the time are not valuable with routine head MRI techniques. They are not aerated before the age of 6 years. Frontal sinuses dimensions at 16 years were 12.8+/-5.0 mm/21.9+/-8.4 mm/24.5+/-13.3 mm. A sinus volume index (SVI) of maxillary and sphenoid sinus was computed using a simplified ellipsoid volume formula, and a table with SVI according to age with percentile variations is proposed for easy clinical application. Percentile curves of maxillary and sphenoid sinuses are presented to provide a basis for objective determination of sinus size and volume during development. These data are applicable to other techniques such as conventional X-ray and CT scan.

Neuronal and nonneuronal quantitative BACE immunocytochemical expression in the entorhinohippocampal and frontal regions in Alzheimer's disease.

In this study, we quantitatively investigated the expression of beta-site amyloid precursor protein cleaving enzyme (BACE) in the entorhinohippocampal and frontal cortex of Alzheimer's disease (AD) and old control subjects. The semiquantitative estimation indicated that the intensity of BACE overall immunoreactivity did not differ significantly between AD and controls, but that a significantly stronger staining was observed in the hippocampal regions CA3-4 compared to other regions in both AD patients and controls. The quantitative estimation confirmed that the number of BACE-positive neuronal profiles was not significantly decreased in AD. However, some degeneration of BACE-positive profiles was attested by the colocalization of neurons expressing BACE and exhibiting neurofibrillary tangles (NFT), as well as by a decrease in the surface area of BACE-positive profiles. In addition, BACE immunocytochemical expression was observed in and around senile plaques (SP), as well as in reactive astrocytes. BACE-immunoreactive astrocytes were localized in the vicinity or close to the plaques and their number was significantly increased in AD entorhinal cortex. The higher amount of beta-amyloid SP and NFT in AD was not correlated with an increase in BACE immunoreactivity. Taken together, these data accent that AD progression does not require an increased neuronal BACE protein level, but suggest an active role of BACE in immunoreactive astrocytes. Moreover, the strong expression in controls and regions less vulnerable to AD puts forward the probable existence of alternate BACE functions.

Thorax, pelvis and hip pattern in the frontal plane during walking in unilateral transtibial amputees: biomechanical analysis.

BACKGROUND Lower limb amputees exhibit postural control deficits during standing which can affect their walking ability. OBJECTIVES The primary purpose of the present study was to analyze the thorax, pelvis, and hip kinematics and the hip internal moment in the frontal plane during gait in subjects with Unilateral Transtibial Amputation (UTA). METHOD The participants included 25 people with UTA and 25 non-amputees as control subjects. Gait analysis was performed using the Vicon(r) Motion System. We analyzed the motion of the thorax, pelvis, and hip (kinematics) as well as the hip internal moment in the frontal plane. RESULTS The second peak of the hip abductor moment was significantly lower on the prosthetic side than on the sound side (p=.01) and the control side (right: p=.01; left: p=.01). During middle stance, the opposite side of the pelvis was higher on the prosthetic side compared to the control side (right: p=.01: left: p=.01). CONCLUSIONS The joint internal moment at the hip in the frontal plane was lower on the prosthetic side than on the sound side or the control side. Thorax and pelvis kinematics were altered during the stance phase on the prosthetic side, presumably because there are mechanisms which affect postural control during walking.

Perifollicular erythema as a trichoscopy sign of progression in frontal fibrosing alopecia.

INTRODUCTION Frontal fibrosing alopecia (FFA) in an entity characterized by the recession of the frontotemporal hairline (FTHL) with alopecic scarring change. In recent years there are numerous articles discussing the usefulness of dermoscopy for the clinical diagnosis of different types of scarring alopecia. MATERIALS AND METHODS We value 79 patients diagnosed with FFA, evaluating some trichoscopical findings described as typical for FFA: Absence of follicular opening, follicular hyperkeratosis, follicular plugs and erythema. RESULTS In a population of 79 women, 100% showed no follicular opening, 72.1% follicular hyperkeratosis, 66.3% perifollicular erythema and 44.8% follicular plugs. Thus, 100% of patients had at least one of the dermoscopic elements described as suggestive of FFA, 53% two of them, 45% three and 27%, all those elements. Perifollicular erythema was present in 95% of cases in which the disease was active. CONCLUSIONS We consider that the presence of perifollicular erythema will be a direct marker of FFA activity.

Disseny i fabricació del frontal d'un monoplaça de competició en fibra de carboni

El projecte estudia la industrialització d’una peça de material compòsit per a un vehicle de competició tenint en compte consideracions de resistència estructural i, en especial, el posterior procés de fabricació. S’ha escollit el frontal d’un monoplaça de competició per estudiar-ne el procés d’industrialització utilitzant una sèrie de programes per fer les simulacions pertinents a partir d’un model informàtic 3D del frontal i de l’aleró que va fixat a la part inferior del frontal

Differential damage in the frontal cortex with aging, sporadic and familial Alzheimer's disease.

In order to understand relationships between executive and structural deficits in the frontal cortex of patients within normal aging or Alzheimer's disease, we studied frontal pathological changes in young and old controls compared to cases with sporadic (AD) or familial Alzheimer's disease (FAD). We performed a semi-automatic computer assisted analysis of the distribution of beta-amyloid (Abeta) deposits revealed by Abeta immunostaining as well as of neurofibrillary tangles (NFT) revealed by Gallyas silver staining in Brodman areas 10 (frontal polar), 12 (ventro-infero-median) and 24 (anterior cingular), using tissue samples from 5 FAD, 6 sporadic AD and 10 control brains. We also performed densitometric measurements of glial fibrillary acidic protein, principal compound of intermediate filaments of astrocytes, and of phosphorylated neurofilament H and M epitopes in areas 10 and 24. All regions studied seem almost completely spared in normal old controls, with only the oldest ones exhibiting a weak percentage of beta-amyloid deposit and hardly any NFT. On the contrary, all AD and FAD cases were severely damaged as shown by statistically significant increased percentages of beta-amyloid deposit, as well as by a high number of NFT. FAD cases (all from the same family) had statistically more beta-amyloid and GFAP than sporadic AD cases in both areas 10 and 24 and statistically more NFT only in area 24. The correlation between the percentage of beta-amyloid and the number of NFT was significant only for area 24. Altogether, these data suggest that the frontal cortex can be spared by AD type lesions in normal aging, but is severely damaged in sporadic and still more in familial Alzheimer's disease. The frontal regions appear to be differentially vulnerable, with area 12 having the less amyloid burden, area 24 the less NFT and area 10 having both more amyloid and more NFT. This pattern of damage in frontal regions may represent a strong neuroanatomical support for the deterioration of attention and cognitive capacities as well as for the presence of emotional and behavioral troubles in AD patients.

Simultaneous Doppelgänger and limb amputation impression in right frontal opercular stroke.

A case is described of a patient who presented almost simultaneously the impression that his left arm was amputated and the feeling of the presence of his invisible Doppelgänger. While these body scheme disorders have both been described after (right) parietal lesions, a right frontal opercular ischaemic stroke was found in the neurological work up. Diffusion tensor imaging showed that the stroke involved the ventral bundle of the superior longitudinal fasciculus that connects the parietal to the frontal lobe. The unusual clinical presentation of this frontal lesion may have been due to a 'diaschisis'-like phenomenon via the superior longitudinal fasciculus.

O contributo do músico Ildo Lobo na divulgação da cultura Cabo-verdiana

O presente trabalho de fim de curso que tem como tema: “O contributo do músico Ildo Lobo na divulgação da cultura cabo-verdiana”, vem no sentido de não só cumprir as obrigações curriculares, no que tange à obtenção do grau de licenciado, como também realizar um sonho de há muito, uma vez que com o desaparecimento físico da minha mãe, pessoa bastante apreciada no seio da cultura cabo-verdiana, começou a despertar-se em mim uma certa curiosidade, relativamente ao mistério da morte e da sua aceitabilidade ou não no seio da sociedade. A partir de então, aproveitando-me do espírito inovador e criativo que sempre me acompanhou e com o intuito de contribuir e prestar o meu tributo à cultura cabo-verdiana, com a devida vénia, começou a emanar em mim, a intenção de trabalhar um tema desta natureza. Deste modo, o que aqui se faz presente, não só é o cumprimento de mais uma etapa da formação, mas sobretudo a concretização de um sonho. Contudo, a escolha do tema «O contributo do músico Ildo Lobo na divulgação da cultura cabo-verdiana» deve-se a dois factores essenciais: o anseio por um conhecimento mais aprofundado sobre a cultura cabo-verdiana, cuja curiosidade foi despertada pela cativante e interessante cadeira do mesmo nome, que tivemos no decorrer da nossa formação e a autenticidade, a originalidade que a mesma oferece, por exemplo, através das composições interpretadas, da miscigenação, da língua, enfim, dos elementos culturais. Considerando a sociedade cabo-verdiana, nas suas práticas sociais, linguísticas e sobretudo culturais, fruto da miscigenação e da diferença de classe, achamos merecido um especial destaque para autores compositores, intérpretes que vivenciaram desde há muito tempo, acontecimentos culturais nacionais de maior relevância. É neste sentido que aqui se torna foco da nossa análise o músico cabo-verdiano Ildo Lobo. Com este trabalho não se pretende fazer uma reflexão acabada sobre a música, mas uma pesquisa inicial, requisito preciso para obtenção do grau de licenciado, em estudos Cabo-Verdianos e Portugueses, dada a carência de documentos coesos sobre a referida matéria e as dificuldades de informações necessárias, durante a pesquisa. Entretanto, com este tema pretendemos valorizar o músico em si, analisar algumas composições interpretadas por ele, com o intuito de deixar patente a ideologia do músico em estudo, no âmbito da queda dos valores morais dos nossos avós e hoje, a nível mundial, fala-se da crise e da mudança de valores em todas as áreas da actividade humana. 9 Consideramos o tema de extrema importância porque é actual, abrangente, e, no nosso entender, norteador da sociedade, em especial a massa juvenil que tendem a enveredar pelo campo da música. Com essa afirmação pretendemos demonstrar que uma elevada percentagem da população cabo-verdiana vive para a música e que a mesma tem estado a desempenhar um papel educativo, uma vez que visa uma formação integral do indivíduo, dentro da sua época, ajudando-o a ter um espírito crítico, tomando consciência de si, e a integrá-lo no mundo que o rodeia, fazendo com que o mesmo respeite a si próprio e respeite o outro. É nesse sentido que o presente trabalho encontra-se estruturado em quatro capítulos, onde no primeiro capítulo iremos fazer o enquadramento do músico, sem deixar de apresentar a vida e obra do mesmo; No capítulo seguinte, denominado de contextualização e caracterização da música como agente transmissora da cultura, faremos uma abordagem teórica sobre a música como elemento cultural, tentaremos desvendar o significado da música para Ildo Lobo, analisando literariamente algumas composições interpretadas pelo mesmo, demonstrando desse modo o impacto do intérprete na música cabo-verdiana; Prosseguindo, chegaremos ao terceiro capítulo, onde iremos elencar as diversas facetas do músico, priorisando a sua dimensão artística do homem no tempo, manifestando a sua especificidade, vendo-o como intérprete, a solo, destacando a serenata e a morna; Por último patentearemos, no quarto capítulo, o contributo do músico na cultura cabo-verdiana, onde irão ser destacados os seguintes subtítulos: Ildo Lobo, o património da cultura cabo-verdiana, os valores tradicionais retractados nas suas interpretações, relação homem e meio, em Ildo Lobo, o músico e as homenagens. Após isso, serão apontadas algumas considerações finais, bem como as fontes que nortearam este trabalho e anexos.