The benign spectrum of hypothalamic hamartomas: infrequent epilepsy and normal cognition in patients presenting with central precocious puberty.

PURPOSE: Hypothalamic hamartoma (HH) is the main structural cause of central precocious puberty (CPP). HH is frequently associated with cognitive impairment and epileptic encephalopathies. Disease severity in case series from neurology services may be biased towards more neurologically impaired patients. AIM: To perform a prospective cognitive evaluation in patients with HH presenting with CPP in an endocrinology outpatient clinic setting. METHODS: We evaluated fifteen consecutive patients with CPP due to HH presenting to an endocrinology outpatient clinic. CPP was diagnosed at a median age of 0.7 yr (0.4-7 yr). Mean age at neurologic evaluation was 13.9 yrs. Eight patients (53.3%) were male. Epileptic seizures occurred in 5/15 (33%) patients. Two patients presented a single unprovoked seizure (SUS). Three patients were diagnosed with epilepsy. Cognitive evaluation, using age-appropriate Wechsler Intelligence Scale, was performed in 11 patients. RESULTS: All patients without epilepsy, including two patients with a history of a SUS, had normal neurologic and cognitive evaluation. Epilepsy and SUS were only seen in patients with sessile HH. Three patients with epilepsy presented cognitive or behavioral findings. Reduced intelligence quotients (IQ), in the borderline range, were noted in both patients with epilepsy who underwent full cognitive evaluation. We found no significant correlation between HH diameter or shape and mean full-scale IQ. CONCLUSIONS: Patients who presented with isolated CPP without epilepsy displayed normal cognition when evaluated after a mean period of 13 years. Occurrence of epilepsy, seen in a minority of patients, but not of a single seizure, was associated with mild cognitive deficit and behavioral disturbances in this case series.

Language impairment in Huntington's disease.

Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (p<0.0001), oral comprehension (p<0.0001), repetition (p<0.0001), oral agility (p<0.0001), reading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

Language impairment in Huntington's disease / Alterações de linguagem na doença de Huntington

Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (p<0.0001), oral comprehension (p<0.0001), repetition (p<0.0001), oral agility (p<0.0001), reading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

Alterações de linguagem são descritas na doença de Huntington (DH), mas sua natureza exata e a correlação com outras funções cognitivas ainda estão em investigação. Os objetivos deste estudo foram caracterizar o prejuízo de linguagem na DH e correlacioná-lo aos aspectos motores e cognitivos da doença. Foram estudados 23 pacientes com DH e 23 controles, equiparados quanto à idade e escolaridade. Usamos os testes de Boston para Diagnóstico da Afasia, de Nomeação de Boston, Token, Modalidades de Símbolos e Dígitos, Stroop, Organização Visual de Hooper (TOVH), fluência de animais, fonêmica e verbos. Pacientes com DH apresentaram pior desempenho na fluência verbal (p<0,0001), compreensão oral (p<0,0001), repetição (p<0,0001), agilidade oral (p<0,0001), compreensão de leitura (p=0,034) e narrativa escrita (p<0,0001). Houve correlação moderada entre os índices Componente de Expressão e Competência de Linguagem e o TOVH (r=0,519, p=0,011 e r=0,450, p=0,031, respectivamente). Alterações de linguagem na DH parecem refletir prejuízos nas regiões frontostriatais e frontotemporais.

Psychological assessment in patients with phobic postural vertigo.

UNLABELLED: Phobic postural vertigo (PPV) is a frequent diagnosis which can be challenging to treat. OBJECTIVE: To investigate the presence of psychiatric disturbances in patients with PPV; to assess the psychological status of patients using adaptive diagnosis; to verify possible correlations between severity of psychiatric disturbance and adaptive efficacy. METHOD: A total of nineteen subjects were assessed and two instruments applied: the Primary Care Evaluation of Mental Disorders Questionnaire (PRIME-MD) and the Adaptive Operationalized Diagnostic Scale (AODS), and results from both tests were compared. RESULTS: Fourteen patients presented with mood disorder and thirteen with anxiety. All patients presented compromised adaptive efficacy. Correlation was found between overall outcome on the PRIME and the AODS (tau= -0.42, p=0.027), Separate analysis revealed correlation between results of the AODS and anxiety disorders (tau= -0.45, p=0.018) but not with mood disorders (tau= -0.36, p=0.054). CONCLUSION: Adaptive compromise was observed in individuals with PPV which was shown to be associated to psychiatric disorders.

Psychological assessment in patients with phobic postural vertigo / Avaliação psicológica em pacientes com vertigem postural fóbica

Phobic postural vertigo (PPV) is a frequent diagnosis which can be challenging to treat. OBJECTIVE: To investigate the presence of psychiatric disturbances in patients with PPV; to assess the psychological status of patients using adaptive diagnosis; to verify possible correlations between severity of psychiatric disturbance and adaptive efficacy. METHOD: A total of nineteen subjects were assessed and two instruments applied: the Primary Care Evaluation of Mental Disorders Questionnaire (PRIME-MD) and the Adaptive Operationalized Diagnostic Scale (AODS), and results from both tests were compared. RESULTS: Fourteen patients presented with mood disorder and thirteen with anxiety. All patients presented compromised adaptive efficacy. Correlation was found between overall outcome on the PRIME and the AODS (tau= -0.42, p=0.027), Separate analysis revealed correlation between results of the AODS and anxiety disorders (tau= -0.45, p=0.018) but not with mood disorders (tau= -0.36, p=0.054). CONCLUSION: Adaptive compromise was observed in individuals with PPV which was shown to be associated to psychiatric disorders.

A vertigem postural fóbica (VPF) é um diagnóstico freqüente e de tratamento difícil. OBJETIVO: Investigar a presença de distúrbios psiquiátricos em pacientes com VPF; avaliar as condições psicológicas dos pacientes através do diagnóstico adaptativo. MÉTODO: Foram avaliados 19 sujeitos e aplicados dois instrumentos de avaliação: Questionário Primary Care Evaluation of Mental Disorders (PRIME-MD) e Escala Diagnóstica Adaptativa Operacionalizada (EDAO) e comparados os resultados de ambos os testes. RESULTADOS: Quatorze pacientes apresentaram transtorno de humor e treze de ansiedade. Todos os pacientes foram avaliados com adaptação ineficaz. Houve correlação entre o resultado geral do PRIME e da EDAO (tau= -0,42, p=0,027). Ao se analisar de maneira separada, foi observada correlação entre os resultados da EDAO e os transtornos de ansiedade (tau= -0,45, p=0,018), não havendo correlação com os transtornos de humor (tau= -0,36, p=0,054). CONCLUSÃO: Há prejuízo na qualidade adaptativa das pessoas que sofrem de VPF e este prejuízo está associado a transtornos psiquiátricos.

Desempenho de uma amostra da população adulta saudável com alta escolaridade em testes atencionais / Performance of healthy adult population and highly educated the attentional functions

Introdução: Este artigo constitui uma investigação das funções atencionais em uma amostra da população adulta saudável com alta escolaridade. Objetivo: Avaliar o desempenho de uma amostra da população adulta saudável com alta escolaridade em testes neuropsicológicos atencionais. Método: Foi realizada avaliação neuropsicológica de 20 mulheres provenientes da comunidade saudável de São Paulo, de 30 a 44 anos, com escolaridade acima de 17 anos, sem história prévia de transtorno psicótico ou esquizofrenia, quadros neurodegenerativos, institucionalização e sem tratamento neurológico para alterações cognitivas. Utilizou-se os testes TMT e Stroop para avaliar as funções atencionais. Resultados: A análise estatística dos resultados revelou que pessoas com menores idades e maior grau de escolaridade obtiveram melhor desempenho em tarefas atencionais. Conclusão: Os resultados sugerem a influência do grau de escolaridade e da idade como uma possível explicação para os resultados obtidos nos testes.(AU)

Introduction: This article investigates the attentional functions in a sample of healthy adult population and highly educated. Objective: To evaluate the performance of a healthy adult population sample and highly educated on the attentional functions. Method: A neuropsychological valuation was done among 20 ladies from a healthy community in São Paulo, in the age range from 30 for 44 years with education above 17 years, without any previous history of psychotic perturbation or schizophrenia, neurodegenerating pictures, institutionalized and without neurologic treatment for cognitive alterations. The tests TMT and Stroop were used to valuate attentional functions. Results: The statistical analysis of the results revealed that people with lower ages and higher educational level performed better on tests attentional. Conclusions: The results suggest the influence of the educational and for age, as a possible explanation for the differences observed in regard to the test performance.(AU)

O paradigma stroop em uma amostra de idosos brasileiros / The stroop paradigm in a brazilian elderly sample

Objetivos: Verificar o efeito Stroop, a influência da escolaridade, do QI e da idade em uma amostra de sujeitos idosos da população brasileira. Métodos: Foram incluídos 61 idosos saudáveis com idades entre 60 e 85 anos, do estado de São Paulo e com escolaridade entre 2 e 18 anos. A amostra foi estratificada por faixa etária e também por faixa de escolaridade (2 à 8 anos e de 9 à 18 anos). Resultados: A análise revelou um desempenho geral inferior ao da referência normativa da amostra canadense. O desempenho mostrou-se melhor quando estratificou-se por escolaridade, apesar de manter padrão inferior. Os desvios-padrão mostraram-se mais amplos na amostra estudada e um menor efeito de interferência foi encontrado. Há correlações significativas entre as variáveis escolaridade e QI com o desempenho, porém sem significância entre idade e desempenho.Conclusão: Os resultados sugerem a influência da escolaridade/QI no desempenho, mas não da idade.Há variabilidade de desempenho necessitando-se mais estudos com amostras mais amplas da população brasileira.(AU)

Objectives: To verify the Stroop effect, the influence of education, IQ and age in a sample of elderly subjects of the population. Methods: We included 61 healthy elderly aged 60 to 85 years, from São Paulo State and education between 2 and 18 years. The sample was stratified for age group and also for years of education ( 2 to 8 years and 9 to 18 years).Results: The analysis revealed an overall performance inferior compared to the normative reference sample Canadian. The performance was better when stratified for education, although mantaining an inferior pattern. The standart desviations are more amples to the studied sample and a smaller interference effect was found. There are significatives correlations between the variables education and IQ with the performance, however without significance between age and performance. Conclusion: The results suggest the influence of education/IQ on the performance, but not to the age. There is variability of performance demanding more researches with larger samples of the Brazilian population.(AU)

Cognitive deficits associated with optic aphasia: Neuropsychological contribution to a differential diagnosis.

Optic aphasia is characterized by a deficit in naming objects presented visually, as a result of left occipito-temporal lesion. It differs from other neuropsychological disorders due to the nature of the deficits and impairment of cognitive function. A 52 year-old patient, admitted after an episode of sub-acute infarction in the territory of the left posterior cerebral artery involving the temporo-occipital region, was submitted to neuropsychological evaluation as part of a diagnostic investigation and presented specific characteristics of this disorder, as well as impairment to episodic memory. The relevance of the present case is justified not only due to the rarity of the disorder, but also because it highlights the importance of differential diagnosis in the treatment of patients.

Afasia óptica consiste num déficit de nomear objetos apresentados visualmente como resultado de lesão occipito-temporal esquerda. Difere de outras desordens neuropsicológicas devido à natureza do déficit e comprometimento de funções cognitivas. Um paciente de 52 anos, internado após episódio de infarto subagudo em território de artéria cerebral posterior esquerda com acometimento da região têmporo-occipital, foi submetido à avaliação neuropsicológica como parte de investigação diagnóstica e apresentou características específicas desta desordem, bem como comprometimento de memória episódica. A relevância deste estudo é justificada não somente pela raridade com que a afasia óptica se apresenta, mas porque evidencia a importância do diagnóstico diferencial no tratamento dispensado aos pacientes.

Torre de Londres e Torre de Hanói: contribuições distintas para avaliação do funcionamento executivo / Tower of London and Tower of Hanoi: distinct contributions to the assessment of executive functioning

OBJETIVO: Realizar um estudo teórico sobre comparações de aspectos cognitivos e metodológicos envolvidos no desempenho das Torres de Hanói e de Londres em indivíduos normais. MÉTODO: Revisão narrativa da literatura. RESULTADOS: O desempenho nesses instrumentos não compartilha a variância esperada em virtude das similaridades aparentes em sua estrutura e demanda de solução. Verificaram-se correlações significativas, porém moderadas, entre o desempenho das duas torres, refletindo em média 75 por cento da variância não compartilhada. CONCLUSÕES: Ambas as torres recrutam diferentes processos executivos para solução da tarefa e não podem ser utilizadas como instrumentos permutáveis.

OBJECTIVE: To produce theoretical study about comparisons of cognitive and methodological aspects involved in the performance of Towers of Hanoi and London in normal individuals. METHOD: Narrative review of the literature. RESULTS: The performances in these instruments do not share the expected variance due to the apparent similarities in your structure and solution demand. Correlations significant, however moderate, among the two towers performance, were found reflecting the average of 75 percent of the nonshared variance. CONCLUSIONS: Both towers recruit different executive processes for task solution and cannot be used as exchangeable instruments.

Aspectos cognitivos relacionados à noção de intervalos de tempo / Cognitive aspects related to time interval notion

OBJETIVO: Procuramos analisar os aspectos cognitivos e metodológicos envolvidos na avaliação da noção de intervalos de tempo, verificando os aspectos cognitivos envolvidos durante a sua execução, e apontar a relevância, aplicabilidade e sensibilidade dessa avaliação no exame neuropsicológico. MÉTODOS: Realizou-se revisão bibliográfica abrangendo artigos e pesquisas das literaturas nacional e internacional no período de 1990 a 2005. RESULTADOS: Encontraram-se 13 diferentes estudos. São controversas as discussões acerca de teorias sobre a noção de tempo. Os estudos podem ser agrupados de acordo com duas perspectivas diferentes: a teoria da contagem atencional, de grande utilização por pesquisadores americanos, e a teoria do relógio interno, muito abordada por estudiosos franceses que se diferem tanto em relação à abordagem teórica quanto à metodológica. CONCLUSÕES: Ambos os grupos de pesquisa, americanos e franceses, retratam a relevância da aplicação dos testes de estimativa, reprodução e produção de tempo no estudo das funções executivas.

OBJECTIVE: We tried to analyze the cognitive and methodological aspects involved in the evaluation of time intervals notion, verifying the cognitive aspects involved during your execution, and to point the relevance, applicability and sensibility of this evaluation in the neuropsychologic exam. METHODS: Bibliographical revision was accomplished including articles and researches of the national and international literature in the period of 1990 to 2005. RESULTS: Were found 13 different researches. The discussions concerning theories about the time notion are controversial. These studies can be grouped into two different perspectives: the attentional counter theory, used by American researchers, and the internal clock theory, very boarded by French studious, that are differed in relation to the theoretical and to the methodological approaches. CONCLUSIONS: Both research groups, Americans and French, show the relevance of the application of the time-interval estimation tests, reproduction and production in the study of the executive functions.