Alzheimer's disease (AD) and frontotemporal dementia (FTD) are the most common neurodegenerative early-onset dementias. Despite the fact that both conditions have a very distinctive clinical pattern, they present with an overlap in their cognitive and behavioral features that may lead to misdiagnosis or delay in diagnosis. The current review intends to summarize briefly the main differences at the clinical, neuropsychological, and behavioral levels, in an attempt to suggest which aspects would facilitate an adequate diagnosis in a clinical setting, especially in Latin American and low-and middle-income countries, where the resources needed for a differential diagnosis (such as MRI or biomarkers) are not always available. A timely diagnosis of AD and FTD have significant implications for the medical management and quality of life of patients and careers.

The Neuropsychiatric Inventory Questionnaire (NPI-Q) is an informant-based instrument that measures the presence and severity of 12 Neuropsychiatric Symptoms (NPS) in patients with dementia, as well as informant distress.ObjectiveTo measure the psychometric properties of the NPI-Q and the prevalence of NPS in patients with Alzheimer's disease (AD) in Chile.Methods53 patients with AD were assessed. Subjects were divided into two different groups: mild AD (n=26) and moderate AD (n=27). Convergent validity was estimated by correlating the outcomes of the NPI-Q with Neuropsychiatric Inventory (NPI) scores and with a global cognitive efficiency test (Addenbrooke's Cognitive Examination - Revised - ACE-R). Reliability of the NPI-Q was analysed by calculating its internal consistency. Prevalence of NPS was estimated with both the NPI and NPI-Q.ResultsPositive and significant correlations were observed between the NPI-Q, the NPI, and the ACE-R (r=0.730; p<0.01 and 0.315; p<0.05 respectively). The instrument displayed an adequate level of reliability (Cronbach's alpha=0.783). The most prevalent NPS were apathy/indifference (62.3%) and dysphoria/depression (58.5%).ConclusionThe NPI-Q exhibited acceptable validity and reliability indicators for patients with AD in Chile, indicating that it is a suitable instrument for the routine assessment of NPS in clinical practice.

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Background: Memory impairment in behavioral variant frontotemporal dementia (bvFTD) is traditionally considered to be mild and attributed to prefrontal cortex dysfunction. Recent studies, however, indicated that some patients can present with a memory impairment of the hippocampal type, showing storage and consolidation deficits in addition to the more executive/prefrontal related encoding and strategic difficulties.

ObjectiveTo compare social cognition performance between patients with amyotrophic lateral sclerosis (ALS) and those patients with behavioural variant frontotemporal dementia (bvFTD).MethodsWe included 21 participants with ALS, 20 with bvFTD and 21 healthy controls who underwent a comprehensive cognitive battery, including the short version of the Social Cognition and Emotional Assessment (Mini-SEA), which comprises the faux pas test and Facial Emotion Recognition Test (FERT); Mini-Mental State Examination; Frontal Assessment Battery; lexical fluency (F-A-S), category fluency (animals/minute), digit span (direct and backwards) tests and the Hayling test. A post hoc analysis was conducted with the patients with ALS divided into two subgroups: patients without cognitive impairment (ALScn; n=13) and patients with cognitive impairment (ALSci; n=8).ResultsNo significant difference was noted between participant groups in terms of the age, sex and education. ALS-total group and patients with bvFTD had similar disease durations. Patients with ALSci performed poorly when compared with controls with regard to the FERT (p<0.001), the faux pas (p<0.004) and the Mini-SEA (p<0.002) total scores. Moreover, patients with bvFTD performed poorly in comparison with controls in executive and social cognition tests. The performance of patients with ALSci was similar to that of patients with bvFTD, while the performance of patients with ALScn was similar to that of controls.DiscussionOur findings support a cognitive continuum between ALS and bvFTD and shed light on the cognitive heterogeneity of ALS, expanding its possible neuropsychological profiles.

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Background Decades of researches aiming to unveil truths about human neuropsychology may have instead unveil facts appropriate to only a fraction of the world’s population: those living in western educated rich democratic nations (Muthukrishna et al., 2020 Psych Sci). So far, most studies were conducted as if education and cultural assumptions on which neuropsychology is based were universals and applied everywhere in the world. The importance given to sociological or cultural factors is thus still relatively ignored. With the growth of international clinical studies on dementia, we believe that documenting the potential inter‐cultural differences at stake in a common neuropsychological assessment is an essential topic. This study thus aimed to explore these potential variations in two classical tasks used in neuropsychology that are composing the mini‐SEA (Bertoux et al., 2012 JNNP), i.e. a reduced version of the well‐known Ekman faces (FER), where one has to recognize facial emotions, and a modified version of the Faux Pas test (mFP), where one has to detect and explain social faux. Method The data of 573 control participants were collected through the Social Cognition & FTLD Network, an international consortium investigating social cognitive changes in dementia covering 3 continents (18 research centres in 12 countries). Impact of demographic factors and the effect of countries on performance (mini‐SEA, FER, mFP) were explored through linear mixed‐effects models. Result Age, education and gender were found to significantly impact the performance of the mini‐SEA subtests. Significant and important variations across the countries were also retrieved, with England having the highest performance for all scores. When controlling for demographical factors, differences within countries explained between 14% (mFP) and 24% (FER) of the variance at the mini‐SEA. These variations were not explained by any economical or sociological metrics. Conclusion Important variations of performance were observed across the 12 countries of the consortium, showing how cultural differences may critically impact neuropsychological performance in international studies.

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