Numbers convey different meanings when used in different contexts (Wiese, 2003). In a cardinal context, a number will tell us how many entities are in a set and convey quantity meaning. In an ordinal context, a number will refer to the relative position (or rank) of one element within a sequence; non-numerical ordered series (e.g. the letters of the alphabet) can also be used to provide meaningful order information. Because quantity and order are linked up with each other in the cognitive number domain (the larger the quantity a number refers to, the later it is located in the conventional number sequence), the question of whether they rely on some common or distinct underlying mechanism(s) is theoretically relevant and was addressed in the present thesis. Experimental studies showed evidence of both similarities (similar distance and SNARC effects, recruitment of parietal and frontal regions, and conjoint impairment or preservation after brain damage) and dissociations (different developmental course, dissociation after cerebral lesion, and specific behavioural markers) between quantity and order neuro-functional processes. The aim of the present thesis was to clarify the relationship between numerical quantity and order processing and to test the hypothesis that they rely on (at least partially) dissociated mechanisms. We tested this hypothesis in a single case study, an electrophysiological study and in two behavioural experiments. In the neuropsychological study, we reported the case of patient CO, who showed Gerstmann syndrome after bilateral parietal damage and became unable to process sequence order relations (e.g. he couldn't recite the number sequence backwards, nor decide whether a number, letter, day or month comes before or after a given target in the corresponding sequence, and he was unable to verify the order of items in a pair). Nonetheless, the patient had largely preserved quantity processing abilities (he could compare numbers and dot patterns to find the smaller or larger, and showed a standard distance effect, he could produce a number smaller or larger than a given target, and match dot patterns with Arabic numerals). Overall, CO's pattern of performance was interpreted as reflecting the involvement of different mechanisms when processing quantity or sequence order relations. Our electrophysiological study corroborated this finding since different spatio-temporal patterns of the distance effect were observed when subjects had to process numbers in a quantity comparison task or in an order judgment task. Quantity processing elicited an early distance effect over the P2p component on left parietal sites, whereas the distance effect was slightly delayed and bilaterally distributed in the numerical order judgment task; and this latter task additionally recruited prefrontal regions on a later (P3-counterpart) component. Finally, our behavioural study further emphasized the involvement of different mechanisms underlying the processing of quantity and numerical order and provided some evidence about the nature of these specific mechanisms. In the number comparison (quantity) task, the standard distance effect was proposed to reflect the involvement of a magnitude comparison mechanism; whereas the reverse distance effect observed in the numerical order verification task was taken as evidence for the recruitment of a serial search (recitation) process. Besides, the pair-order effect was also found to specifically affect order but not quantity judgments. Taken together, the data collected in the present thesis lend further support to the hypothesis that quantity and numerical order rely on distinct processing mechanisms that can be damaged selectively after cerebral lesions, that recruit similar brain areas but with a different spatio-temporal course and that show specific behavioural markers.

Numbers convey different meanings when used in different contexts (Wiese, 2003). In a cardinal context, a number will tell us how many entities are in a set and convey quantity meaning. In an ordinal context, a number will refer to the relative position (or rank) of one element within a sequence; non-numerical ordered series (e.g. the letters of the alphabet) can also be used to provide meaningful order information. Because quantity and order are linked up with each other in the cognitive number domain (the larger the quantity a number refers to, the later it is located in the conventional number sequence), the question of whether they rely on some common or distinct underlying mechanism(s) is theoretically relevant and was addressed in the present thesis. Experimental studies showed evidence of both similarities (similar distance and SNARC effects, recruitment of parietal and frontal regions, and conjoint impairment or preservation after brain damage) and dissociations (different developmental course, dissociation after cerebral lesion, and specific behavioural markers) between quantity and order neuro-functional processes. The aim of the present thesis was to clarify the relationship between numerical quantity and order processing and to test the hypothesis that they rely on (at least partially) dissociated mechanisms. We tested this hypothesis in a single case study, an electrophysiological study and in two behavioural experiments. In the neuropsychological study, we reported the case of patient CO, who showed Gerstmann syndrome after bilateral parietal damage and became unable to process sequence order relations (e.g. he couldn't recite the number sequence backwards, nor decide whether a number, letter, day or month comes before or after a given target in the corresponding sequence, and he was unable to verify the order of items in a pair). Nonetheless, the patient had largely preserved quantity processing abilities (he could compare numbers and dot patterns to find the smaller or larger, and showed a standard distance effect, he could produce a number smaller or larger than a given target, and match dot patterns with Arabic numerals). Overall, CO's pattern of performance was interpreted as reflecting the involvement of different mechanisms when processing quantity or sequence order relations. Our electrophysiological study corroborated this finding since different spatio-temporal patterns of the distance effect were observed when subjects had to process numbers in a quantity comparison task or in an order judgment task. Quantity processing elicited an early distance effect over the P2p component on left parietal sites, whereas the distance effect was slightly delayed and bilaterally distributed in the numerical order judgment task; and this latter task additionally recruited prefrontal regions on a later (P3-counterpart) component. Finally, our behavioural study further emphasized the involvement of different mechanisms underlying the processing of quantity and numerical order and provided some evidence about the nature of these specific mechanisms. In the number comparison (quantity) task, the standard distance effect was proposed to reflect the involvement of a magnitude comparison mechanism; whereas the reverse distance effect observed in the numerical order verification task was taken as evidence for the recruitment of a serial search (recitation) process. Besides, the pair-order effect was also found to specifically affect order but not quantity judgments. Taken together, the data collected in the present thesis lend further support to the hypothesis that quantity and numerical order rely on distinct processing mechanisms that can be damaged selectively after cerebral lesions, that recruit similar brain areas but with a different spatio-temporal course and that show specific behavioural markers.

Orientador: Flávia Heloísa dos Santos / Banca: César Alexis Galera / Banca: Vitor Geraldi Haase / Resumo: Há evidências de que o treino musical é subsidiado pela capacidade da memória operacional e envolve conceitos matemáticos. Entretanto não se tem muito conhecimento sobre a relação entre as habilidades matemáticas e a memória operacional durante o desenvolvimento das habilidades musicais em crianças com discalculia do desenvolvimento. Foram realizados dois estudos: Estudo pré-treino: O objetivo foi investigar o perfil de prejuízos das crianças com discalculia do desenvolvimento típico (DT) na capacidade intelectual, desempenho escolar, funções executivas, memória operacional, cognição numérica e comportamento emocional. Foram avaliadas 58 crianças de 8 anos etários: grupo DD (N=28) e DT (n=30). Por meio de testes cognitivos e escalas de comportamento. Os resultados demonstraram que o grupo DD apresentou piores resultados no subteste aritmética do TDE e seu total, assim, como em 11 subtestes e no total da Zareki-R em comparação com o grupo DT, em contraste com os dados normativos as crianças com DD apresentaram resultados abaixo do esperado para faixa etária nos subtestes Contagem Oral em Ordem inversa, Estimativa Contextual, Cálculo Mental e no Total da Zareki-R em comparação com o grupo DT, em contraste com os dados normativos as crainças com DD apresentaram resultados abaixo do esperado para faixa etária nos subtestes Contagem Oral em Ordem inversa, Estimativa Contextual Mental e no Total da Zareki-R. Crianças com DD apresentaram déficits específicos no processamento numérico e cálculo, resultado compatível com diagnóstico. Além disso, as crianças com DD possuíam reconhecimento sobre sua dificuldade, sendo assim, os sinais de incompetência podem levar a uma visão exacerbada de suas dificuldades, portanto, é necessário um diagnóstico especializado para um tratamento adequado. Estudo pós-treino: ... (Resumo completo, clicar acesso eletrônico abaixo) / Abstract: There are evidences that musical training is subsidized by the capacity of working memory and involves mathematical concepts. However do not have much knowledge about the relationship between working memory and mathematical skills during the development of musical abilities in children with developmental dyscalculia. Two studies were conducted. Pre-training study: The objective was to asses the damage profile of children with developmental dyscalculia (DD) compared to children with typical development (DT) in intellectual ability, school performance, executive function, working memory, numerical cognition and emotional behavior. The study included 58 children, age of 8 years, divided into two groups, DD group (N=28) and DT (N=30). Children were evaluated by cognitive taks and behavior scales. The results showed that the DD group the worst results in arithmetic subtest and TDE total, as well as 11 subtests and total Zareki-R, compared with DT, in constrast to normative data, the results were lower than expected for the age, in subtests Oral Count in reverse Order, Contextual Estimation, Mental Calculation and Total Zareki-R DD children showed specific deficits in number processing and calculation, compatible with dvscalculia. In addition, children with DD have recognition of their difficulty, so the signs of inadequacy can lead to a heightened view of the difficulties, thus requires a specialized diagnosis for adequate treatment. Post-training study: The aim of this study was to investigate the effects of two techniques of musical training on the ability of intelligence, shool performance, executive functions, working memory, numerical cognition and emotional behavior, through pre and post musical training in children DD and DT. The study included 53 children, divided into four groups according to the order of the musical training received: Typical... (Complete abstract click electronic access below) / Mestre

Orientador: Flávia Heloisa Dos Santos / Banca: João dos Santos Carmo / Banca: Hugo Ferrari Cardoso / Resumo: A Discalculia do Desenvolvimento (DD) é um transtorno de aprendizagem de caráter persistente e intrínseco, relacionado a déficits na cognição numérica, que não são causados por deficiência intelectual, ou estudo inadequado. Muitas crianças com DD adquirem uma atitude negativa frente à matemática, denominada Ansiedade à Matemática (AM); caracterizada por respostas cognitivas, fisiológicas e comportamentais de esquiva à disciplina. O surgimento da AM parece incluir variáveis genéticas, culturais, sociais e cognitivas. A maioria das pesquisas indica que a AM afeta negativamente a performance matemática. Contudo, os resultados das pesquisas são contraditórios e inconclusivos, possivelmente por variáveis como idade, sexo e método diagnóstico. Entre as intervenções para apoio às crianças com DD, há evidências de que Treinos Cognitivos Computadorizados (TCC) podem, além de melhorar habilidades matemáticas, diminuir os sintomas de AM. O presente estudo teve como objetivo analisar os efeitos de um TCC de habilidades matemáticas sobre a AM em crianças com DD, além de caracterizar a AM quanto a idade, e o sexo; considerando a comparação entre duas escalas avaliativas : Entrevista de Ansiedade a Matemática (MAI) e Escala de Ansiedade a Matemática (EAM). Participaram do estudo 72 estudantes de idade entre 8 e 10 anos, diagnosticados com DD, de ambos os sexos, do interior do estado de São Paulo, provenientes de escolas da área urbana. As crianças foram divididas em três grupos: grupo Contr... (Resumo completo, clicar acesso eletrônico abaixo) / Abstract: Developmental dyscalculia (DD) is a persistent and intrinsic learning disorder, related to deficits in numerical cognition, which are not caused by intellectual disability, or inadequate study. Many children with DD acquire a negative attitude towards mathematics, called Mathematics Anxiety (MA); characterized by cognitive, physiological and behavioral responses to the discipline. The emergence of MA seems to include genetic, cultural, social and cognitive variables. Many researchs indicates that MA affects mathematical performance negatively. However, these studies are contradictory and inconclusive, possibly by variables such as age, sex and diagnostic method. Among the interventions to support children with DD, there is evidence that Computerized Cognitive Training (CCT) can, in addition to improving mathematical skills, decrease MA symptoms. This study aimed to analyze the effects of a CCT mathematical skills on MA in children with DD, in addition to characterizing MA in terms of age and sex; considering the comparison between two evaluative scales: Mathematics Anxiety Interview (MAI) and Mathematics Anxiety Scale (MAS). A total of 72 students aged between eight and ten years, diagnosed with DD, of both genders, from the third and fourth year of elementary in urban schools, living in the countrysde of São Paulo State were participated. The children were divided into three groups: Control Group (CG), Adaptive Group (GA) and Non-Adaptive Group (NAG). All children made two n... (Complete abstract click electronic access below) / Mestre

Clinical variants of dementia are limiting their diagnosis and can leads to underdiagnosing or substitution of two different diseases with the same symptomatology. The aim of this study is a better understanding of a factors involved in the clinical variability of rare dementias. Progressive supranuclear palsy and Gerstmann-Sträussler-Scheinker syndrome caused by mismatch mutation P102L in Prion protein are used as model diseases. In this thesis, we firstly demonstrate the influence of the distribution of neuropathology and its spread on the clinical phenotype of the disease. Although a single neurodegenerative disease increases the risk of neurodegenerative comorbidity, this other neuropathology does not affect the phenotypic presentation of the primary disease. Monogenetically inherited proteinopathies can have a different clinical subtype, which is not only conditioned by causal protein polymorphisms, but can be influenced by the wild type allele of causal protein. A more accurate understanding of the symptomatic variability in dementias will allow a better focus of a drug studies and, in the future a treatment, but it will also lead to a better understanding of the pathogenesis of neurodegenerative diseases. Keywords: dementia, Progressive supranuclear palsy, Gerstmann-Sträussler-Scheinker...