Abstract Objective: To investigate how language deteriorates over the Alzheimer’s Disease course. Methods: A cross-sectional, observational study was carried out. 35 patients diagnosed with dementia due to AD using the NINCDS-ARDRA criteria and undergoing treatment for AD with a therapeutic dose of acetylcholinesterase inhibitors were assessed by the Boston Diagnostic Aphasia Examination (BDAE). The sample comprised 15 patients with mild AD (MMSE > 23, CDR = 0 or 0.5‒1.0) and 20 patients with moderate AD (MMSE = 13‒23, CDR = 2). The results for the 2 groups on all language tasks were compared. Results: A statistically significant difference was found between the mild and moderate AD groups for total score on the BDAE (95% CI 47.10‒114.08, t = 5.0, DF = 21, p = 0.000*), as well as on several tasks involving oral and writing comprehension, language oral expression and writing. Conclusion: The study results showed major changes in the moderate stage. Also, the decline in language performance correlated with the worsening of dementia syndrome, independently of sociodemographic variables. Objective Alzheimers Alzheimer s course Methods crosssectional, crosssectional cross sectional, sectional cross-sectional out 3 NINCDSARDRA NINCDS ARDRA BDAE. . (BDAE) 1 MMSE 23 0.5‒1.0 0510 5 1323 13 13‒23 2. 2) compared Results 95% 95 (95 471011408 47 10 114 08 47.10‒114.08 50 5.0 21 0.000*, 0000 0.000* , 000 0.000*) comprehension Conclusion stage Also syndrome variables (BDAE 0.5‒1. 051 132 13‒2 9 (9 47101140 4 11 47.10‒114.0 5. 0.000 00 0.5‒1 05 13‒ ( 4710114 47.10‒114. 0.00 0.5‒ 471011 47.10‒114 0.0 0.5 47101 47.10‒11 0. 4710 47.10‒1 471 47.10‒ 47.10 47.1 47.

RESUMO Introdução: Durante o estágio moderado da demência na doença de Alzheimer (DA), o distúrbio de linguagem se intensifica, afetando a comunicação. A identificação minuciosa das habilidades linguísticas mais comprometidas nessa fase da doença pode auxiliar no uso de estratégias facilitadoras para a comunicação. Este estudo visou identificar as alterações de linguagem em pacientes com DA na fase moderada. Métodos: 20 pacientes com o diagnóstico de DA provável com base nos critérios do NINCDS-ARDRA, MMSE entre 13 e 23 pontos, CDR=2 e em tratamento medicamentoso para DA foram submetidos à avaliação da global da linguagem por meio do teste de Boston para diagnóstico de Afasia. Os resultados obtidos foram comparados aos da população normal. Resultados: Os pacientes apresentaram escores normais nas tarefas de compreensão oral e escrita de palavras, repetição, mecânica da escrita, ditado do primeiro nível e soletração para o ditado, mas também apresentaram comprometimento em todos os níveis do processamento linguístico, tanto na compreensão quanto nas emissões oral e escrita. Em geral, como esperado, as tarefas envolvendo o acesso lexical foram mais afetadas, porém os pacientes obtiveram desempenho adequado para a nomeação responsiva em que a pista semântica é o eliciador para a resposta. Além disso, os pacientes tiveram melhor desempenho em tarefas de compreensão escrita do que oral. Conclusão: A gravidade da alteração linguística não foi homogênea com algumas habilidades linguísticas mais alteradas do que outras. As habilidades identificadas como preservadas podem contribuir para o uso de estratégias facilitadoras da comunicação nessa fase da DA.

ABSTRACT Background: During the moderate stage of dementia due to Alzheimer’s disease (AD), language disorder is more evident and it impacts on communication. An overview of language impairment could be helpful to find compensatory communication strategies for these patients. Objective: To identify all language impairments among patients with moderate-stage of AD. Methods: 20 patients diagnosed with probable AD based on the criteria of the NINCDS-ARDRA, with a MMSE score of 13-23 points and CDR=2, who were undergoing treatment for AD with therapeutic doses of acetyl cholinesterase inhibitors, were assessed using the Boston Diagnostic Aphasia Examination (BDAE), a test that provides a broad assessment of language. The results were compared with the performance of a normal population. Results: The patients assessed in this study presented normal scores for oral and written word recognition, repetition, mechanics of writing, primer-level dictation and spelling to dictation but also had impairment at most levels of linguistic processing, in oral and written comprehension and production. In general, as expected, the tasks relying on access to the mental lexicon were most significantly affected. However, they performed well in the naming task, in which semantic cues were presented. Moreover, the patients assessed in this study had better performance in written comprehension tasks than in oral ones. Conclusion: The severity of the language impairments was not homogenous, with some linguistic abilities more impaired than others. The abilities that were found to be preserved can help to guide strategies for aiding in communication at this stage of AD.

RESUMO Introdução: Na doença de Parkinson (DP) a disfunção dos circuitos dos núcleos da base é um fator determinante na fisiopatologia dos sinais clássicos da DP e a disartria hipocinética é uma das manifestações da doença. No que se refere aos distúrbios da fala associados à DP, os modelos recentes de processamento de fala complicam a visão antiga da disartria como um déficit apenas de execução motora. Baseado nos achados de que as disfunções nos gânglios basais podem causar alterações de fala e que os distúrbios não estão apenas relacionados aos déficits de execução motora, mas também de programação motora, o objetivo deste estudo foi investigar a presença de distúrbios sensórios-motores da programação motora além dos de execução motora já descritos na fala de pacientes com DP. Métodos: O estudo é transversal e se baseou numa amostra composta por 60 adultos pareados por sexo, idade e escolaridade: 30 adultos diagnosticados com DP idiopática e 30 adultos sadios (grupo controle). Dados obtidos em um estudo prévio que analisou alterações de fluência em indivíduos com DP foram reanalisados acrescentando-se todos os tipos de manifestações/erros na fala, a fim de verificar falhas de programação e/ou execução motora. Os pacientes também realizaram avaliação da apraxia orofacial. Resultados: Todos os pacientes tinham disartria hipocinética. O grupo com DP obteve pior desempenho em todas as tarefas de fala. Conclusão: As características clínicas das manifestações/erros de fala encontrados em pacientes com DP são sugestivas de déficits de execução e de programação motora.

ABSTRACT Introduction: Dysfunction in the basal ganglia circuits is a determining factor in the physiopathology of the classic signs of Parkinson's disease (PD) and hypokinetic dysarthria is commonly related to PD. Regarding speech disorders associated with PD, the latest four-level framework of speech complicates the traditional view of dysarthria as a motor execution disorder. Based on findings that dysfunctions in basal ganglia can cause speech disorders, and on the premise that the speech deficits seen in PD are not related to an execution motor disorder alone but also to a disorder at the motor programming level, the main objective of this study was to investigate the presence of sensorimotor disorders of programming (besides the execution disorders previously described) in PD patients. Methods: A cross-sectional study was conducted in a sample of 60 adults matched for gender, age and education: 30 adult patients diagnosed with idiopathic PD (PDG) and 30 healthy adults (CG). All types of articulation errors were reanalyzed to investigate the nature of these errors. Interjections, hesitations and repetitions of words or sentences (during discourse) were considered typical disfluencies; blocking, episodes of palilalia (words or syllables) were analyzed as atypical disfluencies. We analysed features including successive self-initiated trial, phoneme distortions, self-correction, repetition of sounds and syllables, prolonged movement transitions, additions or omissions of sounds and syllables, in order to identify programming and/or execution failures. Orofacial agility was also investigated. Results: The PDG had worse performance on all sensorimotor speech tasks. All PD patients had hypokinetic dysarthria. Conclusion: The clinical characteristics found suggest both execution and programming sensorimotor speech disorders in PD patients.

O objetivo deste estudo foi avaliar a frequência de ocorrência e caracterizar a tipologia das disfluências em indivíduos com doença de Parkison (DP), controlando-se as variáveis, idade, sexo, escolaridade, tempo de doença, escore na escala de Hoehn e Yahr e status cognitivo (escore no Exame do Minimental). Realizamos um estudo transversal com amostra composta por 60 adultos pareados por sexo, idade e escolaridade. O grupo I foi formado por 30 adultos com DP idiopática, e o grupo II por 30 adultos sadios. Para a avaliação da fluência da fala foi solicitada a emissão de uma narrativa a partir de uma sequência de figuras e realizada a trascrição de 200 sílabas para verificação das disfluências de fala. Os pacientes com DP apresentaram um número maior de disfluências na fala com um grande número de disfluências atípicas e, adicionalmente, observamos que houve interferência das variáveis status cognitivo, tempo de doença e idade na ocorrência das disfluências.

The objective of this study was to determine the frequency of occurrence and to characterize the typology of dysfluencies in individuals with Parkinson’s disease (PD), including the variables age, gender, schooling, disease duration, score on the Hoehn and Yahr scale and cognitive status (score on Mini-Mental State Examination). A cross-sectional study of a sample comprising 60 adults matched for gender, age and schooling was conducted. Group I comprised 30 adults with idiopathic PD, and Group II comprised 30 healthy adults. For assessment of fluency of speech, subjects were asked to utter a narrative based on a sequence of drawings and a transcription of 200 fluent syllables was performed to identify speech dysfluencies. PD patients exhibited a higher overall number of dysfluencies in speech with a large number of atypical dysfluencies. Additionally, results showed an influence of the variables cognitive status, disease duration and age on occurrence of dysfluencies.

Migrânea e terror noturno (TN) são transtornos de alta prevalência que compartilham muitas similaridades. O objetivo desse estudo foi avaliar a ocorrência do antecedente de TN, comparando adolescentes com migrânea e adolescentes saudáveis. Métodos: Todos os pacientes foram submetidos a um questionário detalhado sobre sua cefaleia e foram instruídos a preencher um diário durante um período de dois meses. A idade dos sujeitos variou entre 10 e 19 anos. O diagnóstico de TN foi definido de acordo com a Classificação Internacional dos Transtornos do Sono. Resultados: Foram avaliados 158 sujeitos. Desses indivíduos, 50 apresentavam migrânea episódica, 57 migrânea crônica e 51 eram controles. Participantes que tinham o antecedente de TN apresentavam significativamente mais crises de migrânea do que aqueles que não tinham. Conclusões: Migrânea esteve fortemente associada ao antecedente de TN na população de adolescentes independentemente de variáveis demográficas e intensidade da dor.

Migraines and sleep terrors (STs) are highly prevalent disorders with striking similarities. The aim of this study was to evaluate the effect of the antecedent of STs by comparing adolescents suffering from migraines with healthy controls in a large consecutive series. METHODS: All patients were subjected to a detailed headache questionnaire and were instructed to keep a headache diary during a two-month period. The age range was 10 to 19 years. The diagnosis of STs was defined according to the International Classification of Sleep Disorders. RESULTS: A total of 158 participants were evaluated. Of these participants, 50 suffered from episodic migraines (EMs), 57 had chronic migraines (CMs) and 51 were control subjects (CG). Participants who had a history of STs had significantly more migraines than participants who did not. CONCLUSIONS: Migraine is strongly associated with a history of STs in the adolescent population independent of demographics and pain intensity.

An inflammatory process has been involved in numerous neurodegenerative disorders such as Parkinson's disease, stroke and Alzheimer's disease (AD). In AD, the inflammatory response is mainly located in the vicinity of amyloid plaques. Cytokines, such as interleukin-8 (IL-8) and interleukin-1α (IL-1α), have been clearly involved in this inflammatory process. Polymorphisms of several interleukin genes have been correlated to the risk of developing AD. The present study investigated the association of AD with polymorphisms IL-8 -251T > A (rs4073) and IL-1α-889C > T (rs1800587) and the interactive effect of both, adjusted by the Apolipoprotein E genotype. 199 blood samples from patients with AD, 146 healthy elderly controls and 95 healthy young controls were obtained. DNA samples were isolated from blood cells, and the PCR-RFLP method was used for genotyping. The genotype distributions of polymorphisms IL-8, IL-1α and APOE were as expected under Hardy-Weinberg equilibrium. The allele frequencies did not differ significantly among the three groups tested. As expected, the APOE4 allele was strongly associated with AD (p < 0.001). No association of AD with either the IL-1α or the IL-8 polymorphism was observed, nor was any interactive effect between both polymorphisms. These results confirm previous studies in other populations, in which polymorphisms IL-8 -251T > A and IL-1α-889C > T were not found to be risk factors for AD.

OBJETIVO: Identificar elementos prognósticos para a letalidade da neurotuberculose na criança, a partir das manifestações clínicas, dados epidemiológicos e laboratoriais. MÉTODO: Registros de pacientes internados durante o período de 1982 a 2005 foram retrospectivamente avaliados. Os elementos prognósticos considerados foram: sexo, idade, história de contato íntimo com indivíduo com tuberculose, vacinação com o bacilo de Calmette-Guérin (BCG), teste tuberculínico (PPD), concomitância de tuberculose miliar, convulsões, resultados da análise do LCR e presença de hidrocefalia. RESULTADOS: 141 pacientes com diagnóstico de neurotuberculose foram incluídos. Dezessete por cento dos pacientes foram a óbito. Os fatores associados ao óbito foram história negativa de contágio, ausência de reatividade ao teste de PPD, coma e tempo de internação prolongado. Análise por regressão logística múltipla foi usada para investigar as relações entre os elementos prognósticos e o desfecho óbito. CONCLUSÃO: Os fatores prognósticos na previsão de óbito nos pacientes com neurotuberculose foram a presença de coma no momento do diagnóstico, a ausência de história de contágio e a ausência de reação ao PPD

OBJECTIVE: To identify prognostic factors predicting a fatal outcome in HIV-negative children with neurotuberculosis based on clinical, epidemiological, and laboratory findings. METHOD: The clinical records of all in-patients diagnosed with neurotuberculosis from 1982 to 2005 were evaluated retrospectively. The following prognostic parameters were examined: gender, age, close contact with a tuberculosis-infected individual, vaccination for bacillus Calmette-Guérin, purified protein derivative (PPD) of tuberculin results, concomitant miliary tuberculosis, seizures, CSF results, and hydrocephalus. RESULTS: One hundred forty-one patients diagnosed with neurotuberculosis were included. Seventeen percent of the cases resulted in death. The factors that were correlated with a negative outcome included lack of contact with a tuberculosis-infected individual, negative PPD reaction, coma, and longer hospitalisation time. A multiple logistic regression analysis was performed to identify which of these factors most often resulted in death. CONCLUSION: Coma at diagnosis, lack of tuberculosis contact, and a non-reactive PPD were the most important predictors of fatality in patients with neurotuberculosis