Presence or absence of cognitive complaints in Parkinson's disease: mood disorder or anosognosia?

We intended to evaluate whether non-demented Parkinsons's disease (PD) patients, with or without subjective cognitive complaint, demonstrate differences between them and in comparison to controls concerning cognitive performance and mood. We evaluated 77 subjects between 30 and 70 years, divided as follows: PD without cognitive complaints (n = 31), PD with cognitive complaints (n = 21) and controls (n = 25). We applied the following tests: SCOPA-Cog, Trail Making Test-B, Phonemic Fluency, Clock Drawing Test, Boston Naming Test, Neuropsychiatric Inventory, Hospital Anxiety and Depression Scale (HADS) and Beck Depression Inventory. PD without complaints presented lower total score on Scales for outcome of Parkinson's disease-cognition as compared to controls (p = 0.048). PD with complaints group showed higher scores on HADS (p = 0.011). PD without complaints group showed poorer cognitive performance compared to controls, but was similar to the PD with complaints group. Moreover, this group was different from the PD without complaints and control groups concerning mood.

Presence or absence of cognitive complaints in Parkinson's disease: mood disorder or anosognosia? / Presença ou ausência de queixas cognitivas na doença de Parkinson: transtorno de humor ou anosognosia?

ABSTRACT We intended to evaluate whether non-demented Parkinsons’s disease (PD) patients, with or without subjective cognitive complaint, demonstrate differences between them and in comparison to controls concerning cognitive performance and mood. We evaluated 77 subjects between 30 and 70 years, divided as follows: PD without cognitive complaints (n = 31), PD with cognitive complaints (n = 21) and controls (n = 25). We applied the following tests: SCOPA-Cog, Trail Making Test-B, Phonemic Fluency, Clock Drawing Test, Boston Naming Test, Neuropsychiatric Inventory, Hospital Anxiety and Depression Scale (HADS) and Beck Depression Inventory. PD without complaints presented lower total score on Scales for outcome of Parkinson’s disease-cognition as compared to controls (p = 0.048). PD with complaints group showed higher scores on HADS (p = 0.011). PD without complaints group showed poorer cognitive performance compared to controls, but was similar to the PD with complaints group. Moreover, this group was different from the PD without complaints and control groups concerning mood.

RESUMO Avaliar se pacientes com doença de Parkinson (DP) sem demência, com ou sem queixa cognitiva subjetiva, demonstram diferenças entre eles e comparativamente aos controles relativos a desempenho cognitivo e humor. Avaliados 77 indivíduos entre 30 e 70 anos: PD sem queixas cognitivas (n = 31), PD com queixas cognitivas (n = 21) e controles (n = 25). Testes aplicados: SCOPA-cognição, Trail Making Test-B, Fluência Fonêmica, Teste do Relógio, Teste Nominativo de Boston, Inventário Neuropsiquiátrico, Escala Hospitalar de Depressão e Ansiedade (HADS)e Inventário de Depressão de Beck. PD sem queixas apresentaram menor pontuação total na SCOPA-cognição, comparativamente aos controles (p = 0,048). Por outro lado, PD com queixas apresentaram maior pontuação no HADS (p = 0,011) em comparação aos controles. O grupo PD sem queixas mostrou pior desempenho cognitivo em comparação aos controles, mas foi semelhante ao PD com queixas. Este grupo foi diferente dos grupos PD sem queixas e controle em relação ao humor.

Cognitive and olfactory deficits in Machado-Joseph disease: a dopamine transporter study.

Cognitive and olfactory impairments have been demonstrated in patients with Machado-Joseph disease (MJD), and a possible relationship with dopaminergic dysfunction is implicated. However, there is still controversy regarding the pattern of striatal dopaminergic dysfunction in patients with MJD. In this study, we investigated whether these patients had different dopamine transporter (DAT) densities as compared to healthy subjects, and correlated these data with cognitive performance and sense of smell. Twenty-two MJD patients and 20 control subjects were enrolled. The neuropsychological assessment comprised the spatial span, symbol search, picture completion, stroop color word test, trail making test and phonemic verbal fluency test. The 16-item Sniffin' Sticks was used to evaluate odor identification. DAT imaging was performed using the SPECT radioligand [(99m)Tc]-TRODAT-1, alongside with Magnetic Resonance imaging. Patients with MJD showed significantly lower DAT density in the caudate (1.34 ± 0.27 versus 2.02 ± 0.50, p < 0.001), posterior putamen (0.81 ± 0.32 versus 1.32 ± 0.34, p < 0.001) and anterior putamen (1.10 ± 0.31 versus 1.85 ± 0.45, p < 0.001) compared with healthy controls. The putamen/caudate ratio was also significantly lower in patients compared with controls (0.73 ± 0.038 versus 0.85 ± 0.032, p = 0.027). Even though we had only two patients with parkinsonism, we detected striatal dopaminergic deficits in those patients. No significant correlations were detected between DAT density and cognitive performance or Sniffin' Sticks scores. The data suggests that striatal dopamine deficit is not involved in cognitive or sense of smell deficits. This finding raises the possibility of extra-striatal dopamine and other neurotransmitter system involvement or of cerebellum neurodegeneration exerting a direct influence on cognitive and sensorial information processing in MJD.

Cognitive deficits in Machado-Joseph disease correlate with hypoperfusion of visual system areas.

Cognitive and olfactory impairments have previously been demonstrated in patients with spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD)-SCA3/MJD. We investigated changes in regional cerebral blood flow (rCBF) using single-photon emission computed tomography (SPECT) imaging in a cohort of Brazilian patients with SCA3/MJD. The aim of the present study was to evaluate the correlation among rCBF, cognitive deficits, and olfactory dysfunction in SCA3/MJD. Twenty-nine genetically confirmed SCA3/MJD patients and 25 control subjects were enrolled in the study. The severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. Psychiatric symptoms were evaluated by the Hamilton Anxiety Scale and Beck Depression Inventory. The neuropsychological assessment consisted of Spatial Span, Symbol Search, Picture Completion, the Stroop Color Word Test, Trail Making Test (TMT), and Phonemic Verbal Fluency. Subjects were also submitted to odor identification evaluation using the 16-item Sniffin' Sticks. SPECT was performed using ethyl cysteine dimer labeled with technetium-99m. SCA3/MJD patients showed reduced brain perfusion in the cerebellum, temporal, limbic, and occipital lobes compared to control subjects (pFDR <0.001). A significant positive correlation was found between the Picture Completion test and perfusion of the left parahippocampal gyrus and basal ganglia in the patient group as well as a negative correlation between the TMT part A and bilateral thalamus perfusion. The visuospatial system is affected in patients with SCA3/MJD and may be responsible for the cognitive deficits seen in this disease.

Cerebellar cognitive affective syndrome in Machado Joseph disease: core clinical features.

The cerebellum is no longer considered a purely motor control device, and convincing evidence has demonstrated its relationship to cognitive and emotional neural circuits. The aims of the present study were to establish the core cognitive features in our patient population and to determine the presence of Cerebellar Cognitive Affective Syndrome (CCAS) in this group. We recruited 38 patients with spinocerebellar ataxia type 3 (SCA3) or Machado­Joseph disease (MJD)-SCA3/MJD and 31 controls. Data on disease status were recorded (disease duration, age, age at onset, ataxia severity, and CAG repeat length). The severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. The neuropsychological assessment consisted of the Mini-Mental State Examination, Clock Drawing Test, Wechsler Adult Intelligence Scale, Rey­Osterrieth Complex Figure, Wisconsin Card Sorting Test, Stroop Color­Word Test, Trail-Making Test, Verbal Paired Associates, and verbal fluency tests. All subjects were also submitted to the Hamilton Anxiety Scale and Beck Depression Inventory. After controlling for multiple comparisons, spatial span, picture completion, symbol search, Stroop Color­Word Test, phonemic verbal fluency, and Trail-Making Tests A and B were significantly more impaired in patients with SCA3/MJD than in controls. Executive and visuospatial functions are impaired in patients with SCA3/MJD, consistent with the symptoms reported in the CCAS. We speculate on a possible role in visual cortical processing degeneration and executive dysfunction in our patients as a model to explain their main cognitive deficit.

[Brazilian version of the Cornell depression scale in dementia]. / Versão brasileira da Escala Cornell de depressão em demência (Cornell depression scale in dementia).

OBJECTIVE: Translating and adapting the Cornell scale for depression in dementia to the Portuguese language and verifying the interrater and test-retest reliability of the translated and adapted version. METHOD: The Cornell scale was translated into Portuguese and back translated into English. Divergences of translation were identified and discussed, resulting in a version which was submitted to a pre-test for cross-cultural adaptation. The final version was administered to a sample of 29 patients with probable AD and to their caregivers. RESULTS: The Cornell Scale presented good interrater (Kappa=0,77; p<0,001) and test-retest reliability (Kappa=0,76; p<0,001). The final version was easy to administer and well understood by the caregivers. CONCLUSION: The Brazilian version of the Cornell Scale is an instrument with good reliability to evaluate depression in patients with dementia. This tool will contribute to the evaluation and follow-up of depressed patients with dementia in our population and may also be used in multicentric studies with Brazilian population.

[Cross-cultural adaptation of the Disability Assessment for Dementia (DAD)]. / Adaptação transcultural da escala de avaliação de incapacidade em demência (Disability Assessment For Dementia--DAD).

The original version of the Disability Assessment for Dementia (DAD) was translated into Portuguese and back translated to English. The divergences of translation were identified and discussed, resulting in a version that was used in a preliminary investigation for cross-cultural adaptation. The final version was administered to 29 patients with mild to moderate probable Alzheimer's disease. The correlation coefficients of DAD were 0.929 and 0.932 for the inter-examiner and test-retest evaluations respectively. The reliability indexes were also high (Kappa 0.72 p<0.001 inter-examiners and 0.85 p<0.001 test-retest). The Brazilian version of DAD was easy to administer and had good reliability to assess the functional status of demented patients. It will contribute to the follow-up of these patients in our population. Moreover, it can be used in transcultural studies on functional abilities in dementia.

Versão brasileira da Escala Cornell de depressão em demência (Cornell depression scale in dementia) / Brazilian version of the Cornell depression scale in dementia

OBJETIVO: Tradução e adaptação da escala Cornell de depressão em demência e verificação da confiabilidade entre e intra-examinadores da versão na língua portuguesa. MÉTODO: A versão original da Escala Cornell foi traduzida para o português por firma especializada em tradução de textos médicos e retrotraduzida para o inglês por outros dois tradutores independentes. As divergências de tradução foram identificadas e discutidas, chegando-se à versão que foi submetida à pré-teste para adaptação sócio-cultural. Após esta adaptação, obteve-se a versão final que foi administrada a amostra de 29 pacientes com doença de Alzheimer provável e aos seus cuidadores. RESULTADOS: A versão final da escala mostrou-se de fácil aplicação e obteve boa confiabilidade intra-examinador (Kappa=0,77; p<0,001) e entre-examinadores (Kappa=0,76; p<0,001). CONCLUSÃO: A versão brasileira da Escala Cornell é um instrumento que pode ser utilizado para avaliação e acompanhamento de depressão em pacientes com demência.

OBJECTIVE: Translating and adapting the Cornell scale for depression in dementia to the Portuguese language and verifying the interrater and test-retest reliability of the translated and adapted version. METHOD: The Cornell scale was translated into Portuguese and back translated into English. Divergences of translation were identified and discussed, resulting in a version which was submitted to a pre-test for cross-cultural adaptation. The final version was administered to a sample of 29 patients with probable AD and to their caregivers. RESULTS: The Cornell Scale presented good interrater (Kappa=0,77; p<0,001) and test-retest reliability (Kappa=0,76; p<0,001). The final version was easy to administer and well understood by the caregivers. CONCLUSION: The Brazilian version of the Cornell Scale is an instrument with good reliability to evaluate depression in patients with dementia. This tool will contribute to the evaluation and follow-up of depressed patients with dementia in our population and may also be used in multicentric studies with Brazilian population.

Adaptação transcultural da escala de avaliação de incapacidade em demência (Disability Assessment For Dementia - DAD) / Cross-cultural adaptation of the disability assessment for dementia (DAD)

A versão original da Escala de Avaliação de Incapacidade em Demência (Disability Assessment for Dementia, DAD) foi traduzida para a língua portuguesa e retrotraduzida para o inglês. Divergências de tradução foram identificadas e discutidas, chegando-se a uma versão que foi submetida a pré-teste para adaptação sócio-cultural. A versão final foi administrada a amostra de 29 pacientes com doença de Alzheimer provável de leve a moderada. Os coeficientes de correlação da DAD foram 0,929 e 0,932 nas avaliações inter e intra-examinadores respectivamente. Os índices de confiabilidade também foram elevados (Kappa=0,72; p<0,001 inter-examinadores e Kappa=0,85; p<0,001 intra-examinadores). A versão brasileira da escala DAD mostrou-se um instrumento de fácil aplicação e boa confiabilidade para avaliação funcional de pacientes com demência e poderá contribuir para o acompanhamento desses pacientes em nosso meio. Esta versão também poderá ser utilizada em estudos transculturais sobre habilidades funcionais de pacientes com demência.

The original version of the Disability Assessment for Dementia (DAD) was translated into Portuguese and back translated to English. The divergences of translation were identified and discussed, resulting in a version that was used in a preliminary investigation for cross-cultural adaptation. The final version was administered to 29 patients with mild to moderate probable Alzheimer's disease. The correlation coefficients of DAD were 0.929 and 0.932 for the inter-examiner and test-retest evaluations respectively. The reliability indexes were also high (Kappa 0.72 p<0.001 inter-examiners and 0.85 p<0.001 test-retest). The Brazilian version of DAD was easy to administer and had good reliability to assess the functional status of demented patients. It will contribute to the follow-up of these patients in our population. Moreover, it can be used in transcultural studies on functional abilities in dementia.

Comparison between neuropsychological evaluation instruments for severe dementia.

OBJECTIVE: To study the correlation between two tools for cognitive evaluation, Mini-Mental State Examination-severe (MMSE-s) and Severe Impairment Battery (SIB), and the Bristol Daily Activities Functional Scale. METHOD: 50 patients from the Behavioral Neurology Section--EPM-UNIFESP--were evaluated. Mean age was 76.8+/-7.9 (range 57 to 95); 32% were males; mean education was 5.0+/-2.3 years (range 4 to 15); mean disease duration was 3.9+/-1.5 years (range 2 to 10). RESULTS: Preliminary results in a small sample drawn from the study group do indicate a difference between the three cognitive scales. SIB and MMSE-s had a better correlation with functional score than MMSE, and MMSE-s had a correlation slightly better than SIB. CONCLUSION: These data indicate that it is possible to follow dementia patients up to severe stage as long as adequate instruments are used, and that there may be differences between scales with regard to sensitivity.

Comparison between neuropsychological evaluation instruments for severe dementia / Comparação de instrumentos de avaliação neuropsicológica para a demência grave

OBJECTIVE: To study the correlation between two tools for cognitive evaluation, Mini-Mental State Examination-severe (MMSE-s) and Severe Impairment Battery (SIB), and the Bristol Daily Activities Functional Scale. METHOD: 50 patients from the Behavioral Neurology Section - EPM-UNIFESP - were evaluated. Mean age was 76.8±7.9 (range 57 to 95); 32 percent were males; mean education was 5.0±2.3 years (range 4 to 15); mean disease duration was 3.9±1.5 years (range 2 to 10). RESULTS: Preliminary results in a small sample drawn from the study group do indicate a difference between the three cognitive scales. SIB and MMSE-s had a better correlation with functional score than MMSE, and MMSE-s had a correlation slightly better than SIB. CONCLUSION: These data indicate that it is possible to follow dementia patients up to severe stage as long as adequate instruments are used, and that there may be differences between scales with regard to sensitivity.

OBJETIVO: Avaliar a correlação entre dois instrumentos de avaliação cognitiva, o Mini-Exame do Estado Mental-grave (MEEM-g) e a Bateria para Comprometimento Grave (SIB), e compara-los com a Escala Funcional de Atividades Diárias Bristol. MÉTODO: Foram avaliados 50 pacientes provenientes do Setor de Neurologia do Comportamento - UNIFESP-EPM - com idade entre 57 e 95 anos (média 76,84 anos, desvio-padrão 7,94 anos e mediana 77,00 anos); 16 (32,0 por cento) eram do sexo masculino; tempo de escolaridade era 4 a 15 anos (média 5,08±2,31 anos e mediana 4,00 anos). RESULTADOS: Foi possível observar aspectos de sensibilidade entre o MEEM tradicional e os outros dois instrumentos de avaliação para fases avançadas. CONCLUSÃO: É possível seguir acompanhando o paciente demenciado, mesmo em fases avançadas da doença, em relação às avaliações cognitiva e funcional.

Memoria de figuras com conteudo emocional em pacientes com doenca de Alzheimer / Figure memories with emotional content in patients with Alzheimer's disease

O presente trabalho consiste de um relato preliminar de um projeto de estudo sobre o processamento de estimulos visuais com conteudo emocional em pacientes com demencia do tipo Alzheimer. Foram avaliados 14 pacientes com provavel demencia de Alzheimer e 14 sujeitos controles pareados de acordo com a idade e anos de escolaridade. Foram apresentadas 16 figuras-alvo com diferentes conteudos emocionais (paisagens, pessoas mutiladas, figuras com criancas, figuras de sexo e figuras geometricas, entre outras); imediatamente apos a apresentacao das figuras foi dada uma tarefa distratora de 30 minutos, e em seguida foi feito o reconhecimento das figuras-alvo dispersas entre outras 34; de cada figura-alvo reconhecida foi solicitada a identificacao e classificacao como agradavel, desagradavel ou indiferente...

Discinesia tardia: aspectos clínicos; fisiopatologia e tratamento / Tardive dyskinesia: clinical aspects;physiopathology and treatment

A discinesia tardia (DT), uma complicaçäo do uso da medicaçäo antipsicótica por longo período em pacientes psiquiátricos, apresenta inúmeros fatores de risco, que podem a influenciar na prevalência. Embora sua fisiopatologia näo esteja bem esclarecida foram demonstrados distúrbios na neurotransmissäo dopaminérgica no estriado envolvendo vários mecanismos e neurotransmissores. Apesar de todas as tentativas terapêuicas, até o momento näo se encontrou uma medicaçäo que pudesse reverter totalmente o quadro hoje exitente