Introdução: As tarefas de decisão lexical possibilitam estimar o funcionamento cognitivo ou QI pré-mórbido e através desta estimativa, podese estabelecer se houve estabilidade ou declínio em relação ao funcionamento pregresso do sujeito. Considerando o rápido envelhecimento da população idosa e as doenças neurodegenerativas associadas, fazer o diagnóstico precoce destas se torna cada vez mais imperioso. Embora a avaliação do QI pré-mórbido seja fundamental para o mapeamento do funcionamento cognitivo global prévio, não há estudos na população brasileira que utilizaram tarefas de decisão lexical para se obter esta medida. Objetivo: Avaliar o desempenho de idosos saudáveis, Comprometimento Cognitivo Leve (CCL) e Doença de Alzheimer (DA) em um teste de decisão lexical (TDL). Métodos: Cento e vinte e cinco indivíduos, sendo 38 idosos saudáveis, 61 CCL e 26 DA, com diagnóstico previamente estabelecido por equipe médica, recrutados no Setor de Neurologia do Hospital das Clínicas (GNCC) e no Centro de Referência em distúrbios cognitivos do Hospital das Clínicas (CEREDIC). O desempenho dos pacientes no TDL foi comparado ao de outras provas já validadas para população brasileira. Resultados: as médias dos grupos no TDL não diferiram significativamente (p=0,091). A escolaridade e o TDL, em conjunto, explicaram 66,3% da variabilidade do QI estimado do grupo-controle. Foi encontrada uma correlação moderada entre o subteste Vocabulário da Escala Wechsler para Adultos e do TDL (ambos medidas de inteligência cristalizada), com r=0,56 e p < 0,001. Estudos futuros com diferentes amostras da população brasileira poderiam corroborar ou expandir os resultados obtidos. Conclusão: Os resultados preliminares do TDL mostraram que a decisão lexical pode ser uma medida de QI prémórbido nos indivíduos de uma amostra da população brasileira / Introduction: Lexical decision tasks make it possible to estimate previous cognitive functioning or pre morbid IQ. Through this estimate we can establish whether there was a cognitive decline or maintenance in comparison to the previous functioning. If we take into account the rapid aging of the elderly population and the associated neurodegenerative diseases, early diagnosis of these these desease is crucial. Although the assessment of pre morbid IQ is fundamental for mapping the previous global cognitive functioning, there are no studies involving Brazilian population that used lexical decision task to obtain this measurement. Objective: To evaluate the performance of healthy elderly, Mild Cognitive Impairment (MCI) and Alzheimer\'s disease (AD) participants using a lexical decision test (LDT). Methods: One hundred and twenty-five individuals, 38 healthy elderly subjects, 61 MCI and 26 AD, with diagnosis previously established by medical staff, were recruited from the Cognitive and Behaviour Neurology Group (GNCC) of the Hospital das Clinicas and from the Reference Center for Cognitive Disorders of the Hospital das Clinicas (CEREDIC. The performance of patients in the LDT was compared to that of other tests already validated for the Brazilian population. Results: Group means in the LDT did not differ significantly (p = 0.091). Education and LDT together accounted for 66.3% of the variability in the control group estimated IQ. It was found a moderate correlation between vocabulary subtest of the Wechsler Scale for Adults and LDT scores (both are measures of crystallized intelligence), with r = 0.56 and p < 0.001. Future studies with different samples of the Brazilian population might support or expand the obtained results. Conclusion: Preliminary results of the LDT showed that lexical decision may be a measure of pre morbid IQ in individuals from a sample from the Brazilian population

INTRODUÇÃO: O maior desafio no diagnóstico de perdas cognitivas na nossa população é sua heterogeneidade educacional, com um espectro que vai do analfabetismo, analfabetismo funcional até os escolarizados com diferentes graus de alfabetização mesmo com o mesmo grau de escolarização. OBJETIVOS: Comparar os resultados obtidos no S-TOFHLA entre indivíduos controles, pacientes com comprometimento cognitivo leve (CCL) e pacientes com doença de Alzheimer (DA) e correlacioná-los aos anos de escolarização formal, aos testes de Avaliação Neuropsicológica e aos escores alcançados no teste Matrizes Progressivas Coloridas de Raven e nos subtestes Vocabulário e Cubos do WAIS-III como medida de nível intelectual estimado. MÉTODOS: A amostra foi composta por 148 sujeitos, sendo 61 controles saudáveis, 42 pacientes com CCL e 45 com DA. Todos os participantes foram submetidos a avaliação neuropsicológica, S-TOFHLA e avaliação funcional. RESULTADOS: Na avaliação observou-se que nos testes: Cubos, Raven e QI Estimado foram encontradas diferenças estatísticas entre os grupos controle e CCL; controle e DA, mas não entre os grupos CCL e DA. No S-TOFHLA, observou-se diferença estatisticamente significante no item de Compreensão e Leitura e no escore total nos três grupos (controle, CCL e DA). No item Numérico, a diferença estatística ocorreu somente entre os grupos controle e DA. O S-TOFHLA correlacionou-se fortemente o QI estimado. CONCLUSÕES: O S-TOFHLA sofre influência da progressão da doença apresentando diferença entre os grupos estudados. As alterações em inteligência fluida ocorrem desde início da doença. O subteste Vocabulário permaneceu com resultados semelhantes durante os graus de comprometimento cognitivo, mostrando que memória semântica e inteligência cristalizada são preservadas. / INTRODUCTION: The greatest challenge in the diagnosis of cognitive loss in our population is its educational heterogeneity, with a spectrum ranging from illiteracy, functional illiteracy and various degrees of literacy even with the same level of schooling. OBJECTIVES: To compare the results obtained in the S-TOFHLA among control subjects, patients with mild cognitive impairment (MCI) and patients with Alzheimer\'s disease (AD) and correlate those scores with years of formal schooling, Neuropsychological Assessment, and the scores achieved on Raven\'s Colored Progressive Matrices and Vocabulary and Block Design subtests of the WAIS-III as a measure of estimated intellectual level. METHODS: The sample consisted of 148 subjects, of which 61 were healthy controls, 42 had MCI and 45 had AD. All participants underwent neuropsychological assessment, S-TOFHLA and functional evaluation. RESULTS: In the neuropsychological evaluation it was observed that in the tests Block Design, Raven and IQ Estimate statistical differences were found between MCI and control groups, control and AD, but not between the MCI and AD groups. In the S-TOFHLA, there was a statistically significant difference in reading comprehension and in the total score in all three groups (control, MCI and AD). In the Numeric item, the only statistical difference occurred between control and AD. The S-TOFHLA strongly correlated with the estimated IQ. CONCLUSIONS: The S-TOFHLA is influenced by disease progression showing significant difference between groups. The changes in fluid intelligence occur since the onset of disease. The Vocabulary subtest remained with similar results in different degrees of cognitive impairment, showing that semantic memory and crystallized intelligence are preserved.

Introdução: a desorientação topográfica é um sintoma comum em pacientes com doença de Alzheimer (DA), no entanto não tem tão estudada quanto outros sintomas da DA. Objetivos: Verificar a ocorrência da desorientação topográfica em pacientes com DA e identificar quais transtornos neuropsicológicos estão relacionados com a desorientação. Métodos: Foram avaliados vinte e sete pacientes com DA provável (12 do sexo feminino, 14 com DA com demência de leve intensidade) e 30 indivíduos controles sem demência (21 do sexo feminino). Os pacientes foram submetidos a um questionário específico sobre orientação topográfica e foram submetidos aos seguintes testes: Mini Exame do Estado Mental, extensão de dígitos, fluência verbal, teste de cancelamento, teste dos blocos de Corsi, localização de pontos no espaço, julgamento de orientação de linhas, cópia de figuras em três dimensões e figuras sem sentido e testes de rotação mental. Foram realizados testes de reconhecimento de marcos topográficos, orientação pessoal, descrição de rotas, localização de cidade no mapa do Brasil. Resultados: Pacientes e controles não apresentaram diferença quanto à escolaridade e gênero. Houve diferença entre pacientes e controles no questionário sobre orientação topográfica e em todos os testes utilizados, exceto nas tarefas de funções vísuo-espaciais. O grupo de pacientes com demência de leve intensidade demonstrou diferença no questionário sobre orientação topográfica, descrição de rotas, reconhecimento de marcos topográficos, orientação espacial julgamento de orientação de linhas e localização de cidades no mapa. Apenas o reconhecimento de marcos topográficos, foi capaz de diferenciar entre pacientes com demência de leve ou de moderada intensidade. Conclusões: O questionário sobre orientação topográfica demonstrou que até mesmo os pacientes com DA com demência de leve intensidade tiveram desorientação topográfica. Os testes de reconhecimento de marcos topográficos, xi orientação pessoal egocêntrica e alocêntrica, estão alterados precocemente e podem contribuir para a desorientação topográfica, A memória operacional espacial e as funções visuo-espaciais não estão comprometidas inicialmente e provavelmente não contribuíram para a desorientação topográfica em pacientes com demência de leve intensidade. No entanto, pacientes com demência de intensidade moderada demonstraram comprometimento nas tarefas de memória operacional especial e testes de funções vísuoespaciais, o que provavelmente contribuiu para a desorientação topográfica nestes pacientes / Background: Topographical disorientation is a common symptom in patients with Alzheimer´s disease (AD) that has not been as extensively studied as other common manifestations of this disease. Objective: To verify the occurrence of topographical disorientation in patients with Alzheimer\'s disease (AD) and to identify which neuropsychological dysfunctions are causally related to the presence of this manifestation. Methods: Twenty seven patients meeting criteria for probable AD (12 female, 14 with mild dementia) and 30 subjects (21 female) without dementia were analyzed. The subjects and the caregivers were interviewed with a questionnaire on topographical orientation. The following tests were given: Mini mental state examination (MMSE), digit span, verbal fluency, cancellation task, Corsi\'s block tapping test, point localization, line orientation judgment, three dimension and nonsense figure copy and mental rotation tests. Landmarks recognition, personal orientation, recalling routes were tested in a descriptive task and geographic knowledge was evaluated with a Brazilian map. Results: Patients and control subjects were not different regarding schooling years and gender. There were differences between patients and controls in the questionnaire on topographical orientation and in all tests except in visual spatial tasks. Patients with mild dementia had difference in the questionnaire, recalling routes, landmark recognition, personal orientation, geographical knowledge and line orientation judgment. Only the landmark recognition test was found to differentiate between patients with mild or moderate dementia. Conclusions: The topographic orientation questionnaire showed that even in mild dementia, AD patients have topographical disorientation. The landmark recognition, self-centered and world-centered orientation were precociously affected and may contribute for topographical disorientation. Spatial working memory and spatial visual functions are not compromised initially and xiii probably did not contribute for topographical disorientation in the patients with mild dementia. However, patients with moderate dementia showed impairment in spatial working memory and spatial visual tests, which may have contributed to the topographic disorientation of these patients

A nomeação constitui uma das tarefas mais importantes no processamento da linguagem. A nomeação de diferentes categorias semânticas e gramaticais diverge em suas propriedades lexicais e possui substratos neuroanatômicos distintos. Esta pesquisa tem como objetivos caracterizar as alterações de nomeação de sujeitos com afasia, comparando-os aos sujeitos sem alteração de linguagem e estabelecer relações da tipologia de resposta na tarefa de nomeação de verbos e de substantivos em afásicos. A amostra foi composta por indivíduos divididos em dois grupos: GC e GP. O GC foi constituído de 95 sujeitos sem alteração de linguagem, com média de idade de 56,7 anos (15,4) e escolaridade de 9,3 anos (4,4). O GP foi constituído por 33 afásicos, subdividido em não fluentes (GNF) e fluentes (GF). No GNF foram avaliados 17 sujeitos com média de idade de 65,5 anos (14) e escolaridade de 8,2 anos (6,4). No GF foram avaliados 16 sujeitos com média de idade de 60,5 anos (16,7) e escolaridade de 11,5 anos (7,6). O teste utilizado foi a bateria inglesa An Object and Action Naming Battery (Bateria de Nomeação de Objetos e Verbos - BNOV). Houve diferenças estatisticamente significativas entre os grupos de afásicos no número de acertos para substantivos e verbos (p<0,001). O número de acertos para a nomeação de substantivos é maior do que para verbos no GNF enquanto o número de acertos para a nomeação de verbos é maior no GF. Os sujeitos do GNF apresentaram como tipo de resposta desviante mais frequente a \"complementação por gesto\", seguido dos tipos xiii \"hiperônimo\" e \"componente\" na nomeação de substantivos e \"erros de associação\", \"co-hipônimo\", e \"substantivo relacionado\", na nomeação de verbos. Os sujeitos do GF apresentaram como tipo de resposta desviante mais frequente o \"neologismo\", seguido de \"parafasia fonêmica\" e \"circunlóquios\" para a nomeação de substantivos e \"neologismo\", \"erros fonológicos\" e \"circunlóquios\" para verbos. / Naming is one of the most important functions in language processing. The naming of different semantic and grammatical categories diverge in their lexical properties and possess distinct neuroanatomical substrates. This study aims at characterizing the naming alterations and types of errors of aphasic individuals in a verbs and nouns naming. The sample was composed of subjects divided into two groups: GC and GP. The GC consisted of 95 subjects with no language impairment, with the mean age of 56.7 years (15.4) and schooling of 9.3 years (4.4). The GP comprised 33 aphasic patients, subdivided into non fluent (GNF) and fluent (GF). In the GNF, 17 subjects with the mean age of 65.5 years (14) and schooling of 8.2 years (6.4) were evaluated. In the GF, 16 subjects with the mean age of 60.5 years (16.7) and 11.5 years of schooling (7.6) were evaluated. The test used was the English battery An Object and Action Naming Battery. There were statistically significant differences between the aphasic groups as to the number of correct answers for nouns and verbs (p <0.001). The number of correct answers in the naming task for nouns was higher than for verbs in the GNF, while the number of correct answers in the naming of verbs was higher in the GF. The most frequent type of error presented by the GNF presented \"complementation by gesture\", followed by \"hyperonym\" and \"component\" in the naming of nouns and \"association errors\", \"hyponym\", and \"related noun\" in the naming of verbs. The most frequent type of error presented by the GF was \"neologism\", followed by \"phonemic paraphasia\" and \"circumlocutions\" in the naming of nouns and \"neologism\", \"phonological errors\" and \"circumlocutions\" for verbs.

Introdução: A doença de Wilson (DW) é uma doença genética rara, sistêmica, causada por déficit no metabolismo do cobre, levando a acúmulo em diversos órgãos, incluindo o cérebro, especialmente os núcleos da base. Apesar de distonia e disartria serem as alterações neurológicas mais comuns, alterações cognitivas, incluindo demência, podem surgir em casos não tratados. Objetivos: Avaliar as funções cognitivas de um grupo de pacientes com DW e correlacionar com os achados de RM e SPECT. Métodos: Todos os pacientes com DW atendidos consecutivamente em uma clínica de distúrbios do movimento, entre outubro de 2006 e agosto de 2007, foram submetidos ao Miniexame do Estado Mental (MEEM), Bateria Cognitiva Breve (BCB), teste de fluência verbal (animais, FAS e verbos), Bateria de Avaliação Frontal (BAF), STROOP, Extensão de Dígitos (ED) ordem direta e ordem inversa, Escala de Demência de Mattis (DRS), Teste de Seleção de Cartas de Wisconsin (WCST), Hooper e Blocos do WAIS. Depois dos testes, todos os pacientes realizaram RM e SPECT. Alterações de RM (hipersinal, hiposinal e atrofia) foram quantificadas em uma escala (0-17). O SPECT foi analisado por meio de SPM. Pacientes com depressão ou com anartria foram excluídos. Os resultados foram comparados com o desempenho de um grupo de voluntários sadios. Resultados: 20 pacientes com DW (11 homens) e 20 controles (9 homens) foram avaliados. A média de idade no grupo de pacientes com DW e controles foi 30,05±7,25 anos e 32,15±5,37 anos, respectivamente. A escolaridade média foi 11,15±3,73 anos nos pacientes com DW e 10,08 ± 2,62 anos nos controles. Pacientes com DW tiveram comprometimento cognitivo nos seguintes testes: MEEM (26,70±2,45 x 28,75±1,29), DRS (132,45 ±10,77 x 140,55±3,72), Fluência verbal: FAS (22,40±12,40 x 38,75±9,11) e Verbos (8,50±6,63 x 15,40±6,22); ED ordem direta (4,95±0,82 x 6,15±1,42), STROOP (4,40±4,87 x 0,50±0,68), BAF (12,95±2,85 x 16,25±1,25) e BCB: M2 (9,20±0,69 x 9,75±0,44), M5 (8,45±1,35 x 9,50 ±0,76). O diagnóstico de demência foi realizado em dois pacientes. Houve correlação entre o comprometimento cognitivo e a escala de RM (r=0,5348). Essa correlação foi mais forte com as alterações de hipersinal e atrofia (r=0,718). A avaliação por SPM mostrou uma hipoperfusão no caudado de forma simétrica, no cerebelo à esquerda e insula à esquerda. Conclusões: Pacientes com DW apresentam um comprometimento cognitivo, especialmente nas funções executivas, com boa correlação entre cognição e RM / Background: Wilsons disease (WD) is a rare, genetic and systemic disease, caused by a deficit in the copper metabolism, leading to its accumulation in different organs, including the brain, especially the basal ganglia. Although dystonia and dysarthria are the most common neurological signals, cognitive changes, including dementia, may emerge in untreated cases. Objective: To assess cognitive functioning of a group of WD patients and correlate with MRI and SPECT findings. Methods: All WD patients consecutively attended in a Movement Disorders Clinic between October 2006 and August 2007 were submitted to the Mini-Mental State Examination (MMSE), Brief Cognitive Screening Battery (BCSB), verbal fluency tests (animals, FAS and verbs), the Frontal Assessment Battery (FAB), STROOP, Digit Span forward and backward (DS), Dementia Ranting Scale (DRS), Wisconsin Card Sorting Test (WCST), Hooper and WAIS block design. After the tests, a MRI and SPECT were done in all patients. MRI abnormalities (high intensity signal, low intensity signal and atrophy) were ratted in a scale (0-17). SPECT was analyzed with SPM. Patients with depression or with anarthria were excluded. Results were compared with the performance of a group of healthy controls. Results: 20 WD patients (11 men) and 20 controls (9 men) were evaluated. Mean age in the WD and control groups were 30.05±7.25 years and 32.15±5.37, respectively. Mean educational level was 11.15±3.73 years among WD cases and 10.08 ± 2.62 years among controls. The WD patients had a cognitive impairment in the: MMSE (26.70±2.45 x 28.75±1.29), DRS (132.45 ±10.77 x 140.55±3.72), verbal fluency: FAS (22.40±12.40 x 38.75±9.11), Verbs (8.50±6.63 x 15.40±6.22); DS forward (4.95±0.82 x 6.15±1.42), STROOP (4.40±4.87 x 0.50±0.68), FAB (12.95±2.85 x 16.25±1.25) and BCSB: M2 (9.20±0.69 x 9.75±0.44), M5 (8.45±1.35 x 9.50 ±0.76). Dementia diagnostic was made in tow patients. There was a correlation between cognitive impairment and MRI scale (r=0.5348), this correlation was better with high intensity signal plus atrophy (r=0.718). The SPM showed a symmetrical caudate, left cerebellar and left insular hypoperfusion. Conclusion: WD patients presented cognitive impairment, especially in executive functions, with good correlation between cognition and MRI

Abstract Frontotemporal lobar degeneration (FTLD) is the second commonest cause of dementia after Alzheimer’s disease (AD) in patients &lt;65 years. Its most frequent clinical subtype is behavioral variant frontotemporal dementia (bvFTD) characterized by behavioral change and executive deficits. FTLD also encompasses two variants of primary progressive aphasia (PPA) characterized by language deficits. The majority of familial FTLD cases are linked to the C9ORF72 expansion mutation. As both cognitive and behavioral changes are core diagnostic features of FTLD, neuropsychological assessment is vital. However, neuropsychological literature is inconclusive regarding the most functional measures for detecting FTLD. Current knowledge on the cognitive profile of patients with the C9ORF72 expansion is scarce. The aims of this thesis were threefold: (1) to identify the cognitive measures that optimally serve the differential diagnosis of FTLD, (2) to characterize the neuropsychological profile of C9ORF72 expansion; and (3) to examine the utility of the Modified Frontal Behavioral Inventory (FBI-mod) in differentiating FTLD, AD, and mild cognitive impairment (MCI). The participants comprised FTLD, AD, and MCI patients diagnosed in the University Hospitals of Oulu and Kuopio. The patients underwent a detailed neuropsychological assessment including the CERAD neuropsychological battery (CERAD-NB) and the FBI-mod. While bvFTD was characterized by verbal fluency, working memory, and verbal comprehension deficits relative to AD, AD was associated with greater episodic memory impairments. The poorer delayed recall in AD was further evident on the memory tests of the CERAD-NB; however, its overall utility in the differentiation between FTLD and AD was limited. The C9ORF72 expansion carriers showed more severe executive deficits than non-carriers. The C9ORF72 expansion may further be associated with slowly progressing FTLD. On the FBI-mod, bvFTD was linked to amplified behavioral symptoms relative to AD, MCI, and PPA. Findings highlight the importance of incorporating a broad cognitive battery in the neuropsychological evaluation of FTLD. Though the clinical phenotype of C9ORF72 expansion appears broad, executive impairment likely is a core feature of bvFTD patients with the expansion. The use of the FBI-mod is recommended as a structured measure for behavioral symptoms of bvFTD. / Tiivistelmä Otsa-ohimolohkorappeumat on Alzheimerin taudin (AT) jälkeen yleisin työikäisten dementiaa aiheuttava sairausryhmä. Sen yleisin alamuoto on otsalohkodementia, jonka ensioireita ovat käyttäytymisen muutokset ja toiminnanohjauksen ongelmat. Sairausryhmään kuuluu myös kaksi kielellisin oirein ilmenevää alatyyppiä. C9ORF72-toistojaksomutaation on todettu selittävän suurimman osan perinnöllisistä tapauksista. Kognitiivisten ja käyttäytymiseen liittyvien muutosten arvioiminen on keskeinen osa taudin diagnostiikkaa. Tutkimustiedon perusteella on epäselvää, mitkä neuropsykologiset menetelmät soveltuvat parhaiten otsa-ohimolohkorappeumien tunnistamiseen. Tieto C9ORF72-mutaation kantajien kognitiivisesta profiilista on niukkaa. Tutkimuksen tavoitteena oli löytää neuropsykologisia menetelmiä, joista on hyötyä otsa-ohimolohkorappeumien erotusdiagnostiikassa ja selvittää C9ORF72-mutaation kantajien neuropsykologisia erityispiirteitä. Lisäksi haluttiin tutkia käytösoireita kartoittavan FBI-mod -läheiskyselyn hyödyllisyyttä otsa-ohimolohkorappeumien, AT:n ja lievän kognitiivisen heikentymän (MCI) erottamisessa. Aineisto koostui Oulun ja Kuopion yliopistosairaaloissa diagnosoiduista otsa-ohimolohkorappeuma-, AT- ja MCI-potilaista, joille oli tehty CERAD-tehtäväsarja, laaja neuropsykologinen tutkimus sekä FBI-mod. Otsalohkodementiaa sairastavat suoriutuivat AT-potilaita heikommin sanasujuvuutta, työmuistia ja kielellistä käsityskykyä arvioivissa tehtävissä, kun taas tapahtumamuisti oli heikompi AT:a sairastavilla. Myös CERAD-tehtäväsarjassa AT-potilaat suoriutuivat heikommin viivästetyn mieleenpalautuksen tehtävissä, mutta kokonaisuutena tehtäväsarjan kyky erotella otsa-ohimolohkorappeumaa ja AT:a sairastavat oli rajallinen. C9ORF72-mutaation kantajilla toiminnanohjauksen ongelmat olivat vaikeampia kuin ei-kantajilla. Lisäksi havaittiin, että C9ORF72-mutaatioon liittyvä sairaus voi edetä hyvin hitaasti. FBI-mod erotteli hyvin otsalohkodementiaa sairastavat AT- ja MCI-potilaista sekä otsa-ohimolohkorappeumien kielellistä muotoa sairastavista. Tulokset korostavat laajan neuropsykologisen tutkimuksen merkitystä otsa-ohimolohkorappeumien diagnostiikassa. Vaikka C9ORF72-mutaation kliininen kuva on vaihteleva, ovat toiminnanohjauksen ongelmat keskeinen osa taudinkuvaa. FBI-mod -kyselyn käyttö on suositeltavaa otsalohkodementiaan liittyvien käytösoireiden strukturoidussa arvioinnissa.

The aim of this study was to establish preliminary normative indications for Rey-Osterrieth Complex Figure test administered in English on a non-clinical population of Xhosa, unskilled workers (N = 33). The population has a history of relatively poor quality education and has received no tertiary education. The sample was stratified into two age grouping (18 – 29 and 30 – 40 years). Both sex and level of education were equally distributed within these age groupings. Within-sample statistical comparisons indicated no significant sex effect was present for any of the trials (Copy, IR or DR). Similarly, no significant age effect was present in the Copy trial. Significant age effects were observed for both recall trials (IR and DR). The local sample’s mean performance was descriptively and statistically compared to demographically equivalent nonlocal normative studies. Excluding an illiterate Columbian sample’s performance, the local sample performed significantly lower than all other available normative populations. Lastly, the local sample performed significantly lower than scores derived from a meta-analysis of normative indications in respect of educationally advantaged westernized groups for all three trials (p = .000 in all instances). These results assert the indispensable need for, and use of, culturally appropriate normative datasets in contemporary South African psychological assessments. Equally, these findings serve to highlight the significant influence of quality of education on cognitive test performance, being a critical socio-cultural variable that needs to be taken into consideration for norming purposes.

Concussion is a common form of brain injury, especially amongst sports players of all age groups. ImPACT is a valid and reliable measure of a variety of cognitive functions commonly affected by such injuries, which allows for objective return-to-play decision making (Iverson, Lovell, & Collins, 2003). However, studies show that the transfer of such tests from one ethnic group to another without appropriate standardization is highly problematic (Ardila, 1995). Thus, the relative absence of South African normative data for the ImPACT 3.0 programme is an issue for concern. Consequently, this study aimed to establish norms for semi-rural Xhosa-speaking schoolboys with an advantaged education (ages 14, 16 and 18) for the ImPACT 3.0 programme as administered in English. Administrative and linguistic difficulties that were experienced by individuals during completion of the battery were also identified. Finally, the study included a comparison of the percentile scores of this sample to the USA norms for boys of a similar age group. Subtests scores were generated for 70 schoolboys and the data were then subjected to statistical analysis. A significant difference between English proficiency of the Grade 8 and Grade 12 boys was found. This indicates the importance of including an English proficiency test with the ImPACT battery when assessing such populations. No other significant differences were found between these age group samples. Although this requires further investigation, the comparison of the USA and SA percentiles suggests the use of local norms for this population. South African boys in this study consistently scored lower than the USA sample. Finally, it is recommended that symptom selfreports should be verbally investigated with each boy after testing, given indications of comprehension problems. In closing, limitations and future possible studies are discussed.

The study investigated the long-term neuropsychological effects of repetitive mild traumatic brain injury (MTBI) due to participation in a contact sport amongst South African final year male high school athletes (N=189). The sample was divided by sports affiliation (Contact n = 115; Non-Contact n = 74) and concussion history (2+ Concussion n = 43; 0 Concussion n = 108). Comparative subgroups were statistically equivalent for age, education and estimated IQ (P > 0.05), with the Contact sport groups having markedly higher incidences of concussion than controls (p < 0.000). Measures included the ImPACT Verbal and Visual Memory, Visuomotor Speed and Reaction Time Composites, Digit Symbol Substitution and Digit Symbol Incidental Recall (immediate and delayed), the ImPACT Symptom Scale and a Post-concussion Symptom (PCS) questionnaire. Independent t-tests on cognitive measures at pre-and post-season revealed a predominant trend of Contact and 2+ Concussion groups performing worse, although only ImPACT Reaction Time at pre-season reached significance (p = 0.014). PCS comparisons revealed an overwhelming tendency of enhanced symptoms for Contact and 2+ Concussion groups with total scores being significantly different in most instances at pre-and post-season. Fatigue and aggression were the symptoms most pervasively high for the Contact and 2+ Concussion groups. Dependent t-test analyses at pre- versus post-season, revealed significant practice effects for the Contact group, not in evidence for controls on ImPACT Visual Motor Speed and Digit Symbol Incidental Recall-Delayed. Overall the results imply the possible presence of lingering neurocognitive and symptomatic concussion sequelae amongst South African final year high school participants of a contact sport. The indications gain potency when understood against the background of (i) Brain Reserve Capacity threshold theory, and (ii) the known risk of Type II error in group MTBI research, that might result in under-emphasis of subtle effects and miscalculation of cost-benefit risks. Clinical implications, and the need for prospective case-based research to ratify the results of this predominantly cross-sectional study, are discussed.

Introdução: A doença de Wilson (DW) é uma doença genética rara, sistêmica, causada por déficit no metabolismo do cobre, levando a acúmulo em diversos órgãos, incluindo o cérebro, especialmente os núcleos da base. Apesar de distonia e disartria serem as alterações neurológicas mais comuns, alterações cognitivas, incluindo demência, podem surgir em casos não tratados. Objetivos: Avaliar as funções cognitivas de um grupo de pacientes com DW e correlacionar com os achados de RM e SPECT. Métodos: Todos os pacientes com DW atendidos consecutivamente em uma clínica de distúrbios do movimento, entre outubro de 2006 e agosto de 2007, foram submetidos ao Miniexame do Estado Mental (MEEM), Bateria Cognitiva Breve (BCB), teste de fluência verbal (animais, FAS e verbos), Bateria de Avaliação Frontal (BAF), STROOP, Extensão de Dígitos (ED) ordem direta e ordem inversa, Escala de Demência de Mattis (DRS), Teste de Seleção de Cartas de Wisconsin (WCST), Hooper e Blocos do WAIS. Depois dos testes, todos os pacientes realizaram RM e SPECT. Alterações de RM (hipersinal, hiposinal e atrofia) foram quantificadas em uma escala (0-17). O SPECT foi analisado por meio de SPM. Pacientes com depressão ou com anartria foram excluídos. Os resultados foram comparados com o desempenho de um grupo de voluntários sadios. Resultados: 20 pacientes com DW (11 homens) e 20 controles (9 homens) foram avaliados. A média de idade no grupo de pacientes com DW e controles foi 30,05±7,25 anos e 32,15±5,37 anos, respectivamente. A escolaridade média foi 11,15±3,73 anos nos pacientes com DW e 10,08 ± 2,62 anos nos controles. Pacientes com DW tiveram comprometimento cognitivo nos seguintes testes: MEEM (26,70±2,45 x 28,75±1,29), DRS (132,45 ±10,77 x 140,55±3,72), Fluência verbal: FAS (22,40±12,40 x 38,75±9,11) e Verbos (8,50±6,63 x 15,40±6,22); ED ordem direta (4,95±0,82 x 6,15±1,42), STROOP (4,40±4,87 x 0,50±0,68), BAF (12,95±2,85 x 16,25±1,25) e BCB: M2 (9,20±0,69 x 9,75±0,44), M5 (8,45±1,35 x 9,50 ±0,76). O diagnóstico de demência foi realizado em dois pacientes. Houve correlação entre o comprometimento cognitivo e a escala de RM (r=0,5348). Essa correlação foi mais forte com as alterações de hipersinal e atrofia (r=0,718). A avaliação por SPM mostrou uma hipoperfusão no caudado de forma simétrica, no cerebelo à esquerda e insula à esquerda. Conclusões: Pacientes com DW apresentam um comprometimento cognitivo, especialmente nas funções executivas, com boa correlação entre cognição e RM / Background: Wilsons disease (WD) is a rare, genetic and systemic disease, caused by a deficit in the copper metabolism, leading to its accumulation in different organs, including the brain, especially the basal ganglia. Although dystonia and dysarthria are the most common neurological signals, cognitive changes, including dementia, may emerge in untreated cases. Objective: To assess cognitive functioning of a group of WD patients and correlate with MRI and SPECT findings. Methods: All WD patients consecutively attended in a Movement Disorders Clinic between October 2006 and August 2007 were submitted to the Mini-Mental State Examination (MMSE), Brief Cognitive Screening Battery (BCSB), verbal fluency tests (animals, FAS and verbs), the Frontal Assessment Battery (FAB), STROOP, Digit Span forward and backward (DS), Dementia Ranting Scale (DRS), Wisconsin Card Sorting Test (WCST), Hooper and WAIS block design. After the tests, a MRI and SPECT were done in all patients. MRI abnormalities (high intensity signal, low intensity signal and atrophy) were ratted in a scale (0-17). SPECT was analyzed with SPM. Patients with depression or with anarthria were excluded. Results were compared with the performance of a group of healthy controls. Results: 20 WD patients (11 men) and 20 controls (9 men) were evaluated. Mean age in the WD and control groups were 30.05±7.25 years and 32.15±5.37, respectively. Mean educational level was 11.15±3.73 years among WD cases and 10.08 ± 2.62 years among controls. The WD patients had a cognitive impairment in the: MMSE (26.70±2.45 x 28.75±1.29), DRS (132.45 ±10.77 x 140.55±3.72), verbal fluency: FAS (22.40±12.40 x 38.75±9.11), Verbs (8.50±6.63 x 15.40±6.22); DS forward (4.95±0.82 x 6.15±1.42), STROOP (4.40±4.87 x 0.50±0.68), FAB (12.95±2.85 x 16.25±1.25) and BCSB: M2 (9.20±0.69 x 9.75±0.44), M5 (8.45±1.35 x 9.50 ±0.76). Dementia diagnostic was made in tow patients. There was a correlation between cognitive impairment and MRI scale (r=0.5348), this correlation was better with high intensity signal plus atrophy (r=0.718). The SPM showed a symmetrical caudate, left cerebellar and left insular hypoperfusion. Conclusion: WD patients presented cognitive impairment, especially in executive functions, with good correlation between cognition and MRI