Síndrome de crouzon: fatores envolvidos no desenvolvimento neuropsicologico e na qualidade de vida

A síndrome de Crouzon é caracterizada por deformidade craniana, alterações faciais e exoftalmia. O retardo no desenvolvimento neuropsicomotor é observado em alguns casos. Este estudo tem como objetivo analisar a influência do momento da cirurgia, da classe sócio-econômica associada ao nível educacional dos pais e da ocorrência de malformações do sistema nervoso central no desenvolvimento cognitivo destes pacientes correlacionando estes achados à qualidade de vida deles e de suas famílias. Foram estudados 11 pacientes com diagnóstico de síndrome de Crouzon com idade entre um ano e quatro meses e treze anos. A avaliação multidisciplinar dos pacientes incluiu, avaliação social, avaliação cognitiva, estudo do encéfalo por ressonância magnética e avaliação da qualidade de vida. O quociente de inteligência variou de 46 a 102 (m=84,2) e foi correlacionado de forma inversa com o Fator 4 do Questionário de Recursos e Estresse Simplificado (incapacidade da criança); não se correlacionou com as alterações encefálicas, com a condição sócio-econômica dos pais e nem com o momento do tratamento neurocirúrgico.

Contribuições da educação permanente para avaliação docente da prática profissional do estudante de medicina

Pós-graduação em Educação - FFC

Evaluation of indexed language used in collective catalogues of university libraries: a socio-cognitive study using verbal protocol

The use of indexing language in university libraries collective catalogs and the socio-cognitive context of indexing and users were evaluated. The methodology consisted of a diagnostic study elaboration of the functioning and treatment procedures of the indexing information from nine libraries of the UNESP Network, representing the Civil Engineering, Pedagogy and Dentistry areas from a data collection using the Verbal Protocol introspective technique in the Individual and Group forms. The study conducted a reflection upon the statements issued by the seventy-two participating individuals whose the results revealed unsatisfactory results about the use of the Subject Headings List of the BIBLIODATA Network, indexing language utilizing by the UNESP Libraries Network, Brazil, in the representation and in the information retrieval process in the ATHENA catalog, about the sequent aspects of the language: lack of specialized vocabulary as well as updated; lack of remissives and of specific headings, and others. We have concluded that the adequate use of indexing languages of specialized scientific areas becomes by means of evaluation as to updating, specificity and compatibility in order to meet the needs of indexing and information retrieval.

Subtle Cognitive Deficits in Adults With a Previous History of Sydenham's Chorea During Childhood

Objective. To evaluate the neuropsychological profile and health-related quality of life (HRQOL) of adults who had rheumatic fever (RF) during childhood with and without Sydenham's chorea (SC).Methods. Three groups of patients were assessed: adults who had RF with SC during childhood (SC group), adults who had RF without SC during childhood (RF group), and controls (CT group). A range of neuropsychological tests looked at several cognitive domains. HRQOL was measured through a Brazilian version of the Short Form 36 (SF-36) health survey.Results. Twenty patients were included in the SC group, 23 patients in the RF group, and 19 patients in the CT group. The 3 groups were homogeneous regarding sex (P = 0.078), age (P = 0.799), schooling (P = 0.600), socioeconomic status (P = 0.138), intelligence quotient (P = 0.329), and scores for anxiety (P = 0.156) and depression (P = 0.076). The SC group demonstrated inferior performance in tests that assessed attention (Digit Span Forward [ P = 0.005], Corsi Block Forward [ P = 0.014]), speeded information processing (Trail Making A [ P = 0.009], Symbol Search [ P = 0.042]), and executive functions and working memory (Corsi Block Backward [ P = 0.028]), and higher scores for attention deficit scale (P = 0.030) when compared with the RF and CT groups. They also showed a tendency toward lower scores in the physical aspects, vitality, emotional aspects, and mental health domains of the SF-36. The RF group had a lower score for the general health domain than the CT group (P = 0.030).Conclusion. Patients who had SC during childhood can exhibit inferior performance in tasks that evaluate attention, speeded information processing, executive functions, and working memory in adult life. Therefore, there is indirect evidence of the persistence of dysfunction in cerebral circuits involved with the basal ganglia. They also presented a worse self-evaluation in HRQOL that was not related to cognitive impairments.

Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Apert syndrome: Factors involved in the cognitive development

Apert syndrome is characterized by craniosynostosis, symmetric syndactyly and other systemic malformations, with mental retardation usually present. The objective of this study was to correlate brain malformations and timing for surgery with neuropsychological evaluation. We also tried to determine other relevant aspects involved in cognitive development of these patients such as social classification of families and parents' education. Eighteen patients with Apert syndrome were studied, whose ages were between 14 and 322 months. Brain abnormalities were observed in 55.6% of them. The intelligence quotient or developmental quotient values observed were between 45 and 108. Mental development was related to the quality of family environment and parents' education. Mental development was not correlated to brain malformation or age at time of operation. In conclusion, quality of family environment was the most significant factor directly involved in mental development of patients with Apert syndrome.

Learning strategies evaluation in students with attention deficit hyperactivity disorder

This study aim to verify the use of learning strategies in students of the elementary level presenting interdisciplinary diagnosis of attention dei cit hyperactivity disorder (ADHD). Nine students, male gender, attending 3rd to 9th grade level of the elementary level, average age 10 years and 7 months, presenting interdisciplinary diagnosis of attention dei cit hyperactivity disorder (ADHD). h e students were submitted to the application of the Evaluation of Learning Strategies from elementary level – EAVAP-EF – scale, which aimed to evaluate the strategies reported and used by students in situation of study and learning, as follows: cognitive strategies, metacognitive strategies and absence of dysfunctional metacognitive strategies. h e general result at EAVAP-EF scale, showed that students with ADHD reached the percentile 25%, considered as low performance in the use of the learning strategies. For the variable absence of dysfunctional metacognitive strategies, the students presented percentile 30%, percentile 25% for cognitive strategies and 55% for metacognitive strategies. h e results showed that ADHD students do not use ef ectively the learning cognitive and metacognitive strategies and present the use of dysfunctional metacognitive strategies. h ese alterations match with the framework of ADHD because the entry of information, either visual or auditory, showed alterations, derived from inattention, which af ected the learning in classroom situation.

Auditory evoked potential: a proposal for further evaluation in children with learning disabilities

The information presented in this paper demonstrates the author's experience in previews cross-sectional studies conducted in Brazil, in comparison with the current literature. Over the last 10 years, auditory evoked potential (AEP) has been used in children with learning disabilities. This method is critical to analyze the quality of the processing in time and indicates the specific neural demands and circuits of the sensorial and cognitive process in this clinical population. Some studies with children with dyslexia and learning disabilities were shown here to illustrate the use of AEP in this population.