Functional changes in bvFTD seem to be better documented by indirect measures.

RESUMO -O autor relata três casos de pseudodemência e discute o problema dos limites entre o normal e o patológico no sujeito idoso. O envelhecimento cerebral acompanha-se de alterações mentais e neuropatológicas superponíveis às da demência de Alzheimer (DA), levando a problemas de diagnóstico diferencial. Ainda não existe um marcador biológico seguro de DA e os testes psicométricos podem dar resultados falso-positivos ou falso-negativos. No idoso normal, o declínio da memória operacional e memória secundária é maior que o das memórias primária e terciária, de modo similar ao encontrado nas fases iniciais da DA. Os testes de evocação retardada de lista de palavras, memória lógica, pensamento categórico, destreza visuo-motora-espacial e o Teste de Nomeação de Boston têm sido apontados como os mais discriminativos entre demência incipiente e normalidade. Na neuroimagem, os achados de atrofia ou hipoperfusão em regiões entorrinais-hipocampais ou temporo-parietais são sugestivos de DA, mas podem estar ausentes nas fases iniciais desta doença. O autor conclui sugerindo passos decisivos para o diagnóstico diferencial: avaliação neuropsicológica e comportamental abrangente (com levantamento do nível pré-mórbido de funcionamento cognitivo e sócio-ocupacional do paciente), bem como exames laboratoriais e de neuroimagem; e, se preciso, reavaliação após 4 a 6 meses, para verificar a consistência dos achados. PALAVRAS-CHAVE: envelhecimento cerebral, demência, testes neuropsicológicos. Brain ageing: problems of differential diagnosis between normal and pathologicABSTRACT -The author reports three cases of pseudodementia and discuss the difficulties in establishing limits between normality and illness in the elderly. The mental and neuropathological changes that accompany the normal ageing of the brain are similar to those of early Alzheimer's dementia (AD). These similarities often lead to difficulties in the differential diagnosis, hence the search for consensus criteria. The decline of working and secondary memory is greater than that of primary and tertiary memory, as is found in AD. On the other hand, tests of delayed recall of 10 to 15 unrelated words, logical memory, categorical thinking, visuo-motor-spatial skills, and Boston Naming Test have been pointed out as the most discriminative. Neuroimaging findings of atrophy or hipoperfusion in the entorhinal-hippocampal or temporo-parietal regions are suggestive of DA, but they can be lacking in the early stages of this disease. In the conclusion, the author suggests the diagnostic process should be based on a comprehensive neuropsychological and behavioral evaluation (including a survey of the subject's premorbid level of cognitive and socio-occupational functioning), supplemented by neuroimaging and laboratory tests. If inconclusive, the whole evaluation can be repeated after 4 to 6 months, to check the consistency of the findings.

Cognitive deficits and apathy are key contributors to functional disability in bvFTD but factors underlying impairment in BADLs remain unclear. Treatments targeting reduction of disability need to address apathy and cognitive impairment to ensure greater efficacy, especially in regards to IADLs.

BACKGROUNDStaging scales for dementia have been devised for grading Alzheimer's disease (AD) but do not include the specific symptoms of frontotemporal lobar degeneration (FTLD).OBJECTIVETo translate and adapt the Frontotemporal Dementia Rating Scale (FTD-FRS) to Brazilian Portuguese.METHODSThe cross-cultural adaptation process consisted of the following steps: translation, back-translation (prepared by independent translators), discussion with specialists, and development of a final version after minor adjustments. A pilot application was carried out with 12 patients diagnosed with bvFTD and 11 with AD, matched for disease severity (CDR=1.0). The evaluation protocol included: Addenbrooke's Cognitive Examination-Revised (ACE-R), Mini-Mental State Examination (MMSE), Executive Interview (EXIT-25), Neuropsychiatric Inventory (NPI), Frontotemporal Dementia Rating Scale (FTD-FRS) and Clinical Dementia Rating scale (CDR).RESULTSThe Brazilian version of the FTD-FRS seemed appropriate for use in this country. Preliminary results revealed greater levels of disability in bvFTD than in AD patients (bvFTD: 25% mild, 50% moderate and 25% severe; AD: 36.36% mild, 63.64% moderate). It appears that the CDR underrates disease severity in bvFTD since a relevant proportion of patients rated as having mild dementia (CDR=1.0) in fact had moderate or severe levels of disability according to the FTD-FRS.CONCLUSIONThe Brazilian version of the FTD-FRS seems suitable to aid staging and determining disease progression.

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