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Previous studies demonstrated cognitive impairments in spinocerebellar ataxia type 3 (SCA3/MJD); however, there is no consensus about the cognitive domains affected and the correlation with structural brain abnormalities. We investigated the neuropsychological profile and 3T-MRI findings, including high-resolution T1-images, diffusion tensor imaging and magnetic resonance spectroscopy of 32 patients with SCA3/MJD and 32 age-, gender- and educational level-matched healthy controls. We reviewed patients' clinical history and CAG repeat length, and performed assessment and rating of ataxia (SARA)-Brazilian version and the neuropsychiatric inventory. Patients presented worse performance in episodic and working memory and Beck inventories (depression and anxiety). SCA3/MJD patients had a reduction of gray matter volume (GM) in the cerebellum, putamen, cingulum, precentral and parietal lobe. A positive correlation was identified between the cognitive findings and GM of temporal, frontal, parietal, culmen and insula. We observed positive correlation between the brainstem's fractional anisotropy and digit span-forward. The following cerebellar metabolite groups (measured relative to creatine) were reduced in patients: N-acetyl-aspartate (NAA), NAA + N-acetyl-aspartate-glutamate and glutamate + glutamine (Glx). We found a positive correlation between Corsi's block-tapping task forward with Glx; semantic verbal fluency with phosphorylcholine and glycerophosphorylcholine; digits span-forward with NAA. The cognitive impairments in SCA3/MJD are associated not only with cerebellar and brainstem abnormalities, but also with neuroimaging evidence of diffuse neuronal and axonal dysfunction, particularly in temporal, frontal, parietal and insular areas.

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UNLABELLED: The objective of this research was to assess the occurrence of cognitive impairment in 32 individuals (average age: 67.2 years old) with Parkinson' disease (PD). PROCEDURES: clinical-neurological assessment; modified Hoehn and Yahr staging scale (HYS); standard neuropsychological battery of CERAD (Consortium to Establish a Registry for Alzheimer' Disease); Pfeffer questionnaire; and Clinical Dementia Rating. A comparison was made with a control group (CG), consisting of 26 individuals with similar age and educational level but without cognitive impairment. The PD patients showed an inferior performance in the CERAD battery when compared to the CG. Three PD sub-groups were characterised according to cognition: no cognitive impairment--15 cases; mild cognitive impairment--10; dementia--7 cases. There was a significant association between motor disability (HYS) and the occurrence of dementia. Dementia and mild cognitive impairment frequently occur in PD patients and should be investigated in a routine way.

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The objective of this research was to assess the occurrence of cognitive impairment in 32 individuals (average age: 67.2 years old) with Parkinson' disease (PD). Procedures: clinical-neurological assessment; modified Hoehn and Yahr staging scale (HYS); standard neuropsychological battery of CERAD (Consortium to Establish a Registry for Alzheimer' Disease); Pfeffer questionnaire; and Clinical Dementia Rating. A comparison was made with a control group (CG), consisting of 26 individuals with similar age and educational level but without cognitive impairment. The PD patients showed an inferior performance in the CERAD battery when compared to the CG. Three PD sub-groups were characterised according to cognition: no cognitive impairment - 15 cases; mild cognitive impairment - 10; dementia - 7 cases. There was a significant association between motor disability (HYS) and the occurrence of dementia. Dementia and mild cognitive impairment frequently occur in PD patients and should be investigated in a routine way.

O objetivo desta pesquisa foi avaliar a ocorrência de déficits cognitivos em 32 indivíduos (idade média: 67,2 anos) com doença de Parkinson (DP). Procedimentos: avaliação clínico-neurológica, escala de Hoehn and Yahr modificada (EHY), bateria neurospicológica do CERAD (Consortium to Establish a Registry for Alzheimer' Disease), questionário de Pfeffer e escore clínico da demência (Clinical Dementia Rating). Foi feita comparação com grupo controle (GC) de 26 indivíduos sem declínio cognitivo, com idade e nível educacional similares. Os pacientes com DP tiveram desempenho inferior na bateria CERAD, quando comparados ao do GC. Foram caracterizados 3 subgrupos com PD segundo a cognição: sem déficits cognitivos - 15 casos; transtorno cognitivo leve - 10; demência - 7 casos. Houve associação entre comprometimento motor e ocorrência de demência. Demência e transtorno cognitivo leve são freqüentes em pacientes com DP e devem ser investigados de modo rotineiro.

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Além das alterações léxico-semânticas, transtornos discursivos também têm sido freqüentemente relacionados as demências. Eles, entretanto, não estão plenamente determinados, principalmente com relação à doença de Alzheimer (DA) e à demência fronto-temporal (DFT), porque a maioria dos estudos não têm examinado sistematicamente a produção discursiva destes pacientes de acordo com diferentes níveis de análise. O objetivo desta pesquisa foi investigar a habilidade discursivo-narrativa e seus correlatos neuropsicológicos em pacientes com DA e com a variante frontal da DFT. Para tanto nós estudamos 47 sujeitos, divididos em quatro grupos: (1) pacientes com DA; (2) pacientes com DFT; (3) pacientes com lesão cerebral restrita aos lobos frontais; e (4) sujeitos-controle não neurológicos. Todos realizaram avaliação neuropsicológica (incluindo o Teste de Vigilância de Strub & Black, subtestes da Escala de Inteligência para Adultos e da Bateria de Memória de Wechsler, Teste de Fluência Verbal, Teste de Nomeação de Boston, Teste de Token, Wisconsin Card Sorting Test, Teste de Stroop, Teste de Organização Visual de Hooper, e Teste de Lista de Palavras) e avaliação discursiva propondo duas tarefas de geração de história: narração baseada em figuras temáticas (seriadas e únicas) e narração espontânea. As produções foram transcritas e quantificadas de acordo com três níveis de análise: microestrutural (organização superficial da narrativa), macroestrutural (organização lógica da narrativa) e pragmática (relacionada à situação comunicativa). A avaliação cognitiva mostrou...

Narrative discourse inabilities are often found in degenerative dementia, additionally to lexical semantic impairment. However, they are not fully established, especially regarding differences between Alzheimer's disease (AD) and frontotemporal dementia (FTD) since most studies have not systematically examined the discourse production of those patients according to the different levels of analysis. The objective of this research was to investigate the narrative discourse abilities and its neuropsychological correlates in patients with AD and FTD. For that purpose, we studied 47 subjects, divided into four groups: (1) AD group; (2) FTD group; (3) frontal lobe lesion group; and (4) non neurological controls. All subjects were submitted to neuropsychological evaluation (including Strub and Black vigilance test, subtests of the Wechsler Adult Intelligence Scale and the Memory Battery, Verbal Fluency Test, Boston Naming Test, Token Test, Wisconsin Card Sorting Test, Stroop Test, Hooper Organization Visual Test, and Word List Test) and evaluation of narrative discourse, consisting of two tasks for story generation: narration based on thematic figures (sequential and single), and spontaneous narration. Productions were transcribed and quantified according to three categories of analysis: microstrucrural (surface narrative organization), macrostructural (logical narrative organization) and pragmatic (related to communicative situation). The cognitive...

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