Abstract In this case report we discuss the lack of emotional reactivity and evasive motor motivation to nociceptive stimuli presented by a patient with frontotemporal degenerative disease and apathy as a predominant behavioral symptom.

Abstract The differential diagnosis between frontotemporal dementia (FTD) and Alzheimer's disease (AD) is sometimes difficult. Objectives: To verify the accuracy of neuropsychological tests and a behavioral disorders scale in the differential diagnosis between FTLD and AD. Methods: Retrospective data on 12 FTD patients and 12 probable AD patients were analyzed. The scores on neuropsychological tests (MMSE score, reverse digit span, delayed recall for drawings, semantic fluency of animals) and the Neuropsychiatric Inventory (NPI) in both groups were compared. Results: Both groups had similar performance on neuropsychological tests. All FTD patients and 50% of AD patients had neuropsychiatric abnormalities. The NPI score was 58.0±19.3 for the FTD patients, and 3.6±4.7 for the AD patients (p<0.01). Using a NPI cut-off score of 13, the sensitivity and specificity were 100% in this sample. The four most common neuropsychiatric disturbances in FTD patients were: apathy, aberrant motor behavior, disinhibition and eating abnormalities. Apathy and dysphoria/depression were the most common behavioral symptoms among the AD patients. Conclusions: In this study, NPI was found to be a useful tool for the differential diagnosis between FTD and AD. The neuropsychological tests commonly used in the medical office were unable to distinguish between the two groups.

ABSTRACT Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Three main clinical variants are widely recognized within the FTLD spectrum: the behavioural variant of frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD represents a highly heterogeneous group of neurodegenerative disorders which are best classified according to the main protein component of pathological neuronal and glial inclusions. The most common pathological class of FTLD is associated with the TDP-43 protein (FTLD-TDP), while FTLD-Tau is considered slightly less common while the FTLD-FUS (Fused in sarcoma protein) pathology is rare. In this review, these three major pathological types of FTLD are discussed.

ABSTRACT Frontal lobe lesions are associated with behavioral abnormalities and executive dysfunction. When these lesions occur early in life, the symptoms are even more severe as the anatomical and functional substrates underlying personality and behavior are damaged, distorting normal modulation by interaction with the psychosocial environment. We present a case of a 40-year-old man who suffered a frontal lobe lesion at the age of nine years and developed impulsivity, disinhibition and inappropriate behaviors while showing some preservation of insight. Brain MRI revealed lesions to bilateral orbitofrontal cortex, ventromedial prefrontal cortex, anterior cingulate gyri and genu of the corpus callosum , which were more extensive on the right side. The right prefrontal dorsolateral cortex was severely damaged, whereas the right ventrolateral prefrontal cortex was spared. We will discuss the correlation of the damaged pre frontal regions with the symptoms presented by the patient.

Abstract Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative disorder that involves the frontal and anterior temporal lobes. It is characterized by prominent behavioral and language disorders. There are three variants of FTLD: frontotemporal dementia (FTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD may be mistaken for AD (Alzheimer's disease) or psychiatric disorders especially in the early stages. Objectives: To describe the demographic characteristics of patients with FTLD, assessed at the Behavioral and Cognitive Neurology Unit, and to show that FTLD is commonly clinically under-diagnosed. Methods: All patients diagnosed with FTLD (Consensus Criteria for FTLD), and who were seen at the Behavioral and Cognitive Neurology Unit of Hospital das Clínicas, in São Paulo, Brazil from January 2004 to August 2007 were included in the analyses. Results: Sixteen patients with FTLD (11 women) were included in this study. There were 12 patients with FTD, two with PNFA and two with SD. The mean duration of the illness until diagnosis of FTLD was 4.1±2.3 years, ranging from one to seven years. Nine patients had been initially seen by psychiatrists, five by neurologists, and two by general physicians. The first diagnosis was psychiatric disorder in six patients, AD in four, dementia in two, FTD in two, and stroke and hydrocephalus for one patient each. Conclusion: The diagnosis of FTLD can be difficult and many patients may be misdiagnosed in Brazil, especially in the initial stages. Educational programs on FTLD for the medical community are warranted.

Abstract The differential diagnosis between frontotemporal lobar degeneration (FTLD) and Alzheimer's disease (AD) is often challenging. Objectives: To verify the usefulness of behavioral and activities of daily living inventories in the differential diagnosis between FTLD and AD. Methods: Caregivers of 12 patients with FTLD (nine with frontotemporal dementia, two with semantic dementia and one with progressive non-fluent aphasia) and of 12 patients with probable AD were interviewed. The Brazilian version of the Frontal Behavioral Inventory (FBI) and Disability Assessment for Dementia (DAD ) were used. Results: The mean of the MMSE score was 12.4±10.7 for patients with FTLD and 11.9±6.2 for patients with AD (p=0.93). Mean scores on the DAD were 33.7±27.7 in patients with FTLD and 55.6±29.7 in patients with AD (p=0.06), while for the FBI they were 42.6±10.0 for FTLD and 16.7±11.7 for AD (p<0.01). Conclusions: In this study, FBI was found to be a helpful tool for the differential diagnosis between FTLD and AD. Although the DAD was not useful in differential diagnosis in our sample we believe it to be important for measuring the severity of the disease through quantitative and qualitative assessment of functional deficits of the patients.

Abstract Functional communication is crucial for independent and efficient communicative behavior in response to every day activities. In the course of dementia progression, cognitive losses may impair these abilities. For this reason, functional communication assessment should be part of a formal assessment to quantify and qualify the impact of deficiency on patients' lives. Objective: To compare functional communication abilities in fronto-temporal lobar degeneration (FLTD) and Alzheimer's disease (AD). Methods: Six AD patients (mean age: 82.50±2.66 years; mean education: 5.67±3.61 years), and eight FTLD patients (mean age: 57.13±9.63 years; mean education: 10.86±6.91 years) had their close relatives answer the Functional Assessment of Communication Skills for Adults (Asha-facs) . Statistical analyses correlated the performance on each of the Asha-facs domains (social communication, communication of basic needs; reading, writing, number concept and daily planning) between both groups. Results: Analyses showed that functional communication was similar for AD and FTLD patients. Only two items had statistical difference, namely 'Comprehension of inference' (AD 6.7±1.33; FTLD 2.43±2.30, p=0.017) and 'capacity to make basic money transactions' (AD 2.17±2.04; FTLD 4.00±0.90, p=0.044). Comparison among the four domains' mean scores revealed no significant difference. Conclusion: The Asha-facs is a useful instrument to characterize functional communication abilities in both FTLD and AD. Nevertheless, the analysis presented for this sample showed that the Asha-facs could not discriminate which aspects of the FTLD and AD differed.

OBJETIVO: analisar as características clínicas e as condições mórbidas (CM) associados em uma amostra de pacientes com demência vascular (DV). MÉTODOS: foram estudados retrospectivamente 25 pacientes com diagnóstico de DV, estabelecidos com base critérios do grupo State of California Alzheimer´s Disease Diagnostic and Treatment Centers (ADDTC). Os dados clínicos e de neuroimagem e os exames laboratoriais foram computados para caracterização da amostra. RESULTADOS: a média da faixa etária foi de 68,7 ± 14,6 anos (64,0% homens), com escolaridade média de 5,2 ± 4,4 anos. A instalação súbita do quadro foi observada em 48,0% dos pacientes e a evolução em degraus e o curso flutuante, em 4,0% e 16,0% dos casos, respectivamente. Apresentavam déficit neurológico focal como sintoma inicial 48,0%, sendo constatado déficit ao exame em 80,0%. As principais CM foram: hipertensão arterial sistêmica (92,0%); hipercolesterolemia (64,0%); insuficiência coronariana (40,0%); tabagismo (40,0%); hipertrigliceridemia (36,0%); diabete melito (32,0%); doença de Chagas (8,0%). CONCLUSÕES: observou-se forte correlação entre DV e hipertensão e hipercolesterolemia. A presença de dois pacientes com doença de Chagas sugere que esta doença possa constituir possível fator de risco regional.

ABSTRACT Several studies have addressed visuospatial and executive skills in artistic activities in Frontotemporal Lobar Degeneration (FTLD) and Alzheimer's disease (AD). Objective: To investigate the performance of FTLD patients compared to controls on two artistic tasks. Methods: Four FTLD patients with mean age of 57 (8.7) years and schooling of 12.2 (4.5) years plus 10 controls with mean age of 62.9 (8.6) years and schooling of 12.3 (4.6) years, were assessed using the Lowenstein Occupational Therapy Cognitive Assessment (LOTCA) and by a three-stage artistic protocol including visual observation, copying and collage, based on a Sisley painting. Results: FTLD patients had lower scores than controls on Visuospatial Perception, Copy, Collage, Examiner's Observation, and Total, showing distinct patterns of performance according to FTLD sub-type: semantic PPA, nonfluent PPA and bvFTD. Conclusion: FTLD patients presented impairment in the visuospatial and executive skills required to perform artistic tasks. We demonstrated that the application of the instrument as a complimentary method for assessing cognitive skills in this group of patients is possible. Further studies addressing larger and more homogeneous samples of FTLD patients as well as other dementias are warranted.

OBJECTIVES: To verify the diagnostic accuracy of the Brazilian version of the Mattis Dementia Rating Scale (DRS) in the diagnosis of patients with mild dementia in Alzheimer's disease (AD); to verify the interference of the variables age and schooling on the performance of the DRS. METHOD: The DRS was administered to 41 patients with mild AD and to 60 controls. In order to analyze the effects of age and schooling on the performance of the tests, patients and controls were separated into three age groups and three levels of schooling. RESULTS: The cutoff score of 122 showed a sensitivity of 91.7 % and specificity of 87.8 %. Age and schooling interfered in the DRS total score and in the scores of its subscales. CONCLUSION: The DRS showed good diagnostic accuracy in the discrimination of patients with mild AD from the control individuals. In the sample examined, the effects of schooling were more marked than age.