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Background: Swallowing and feeding problems may occur with the progression of behavioral variant frontotemporal dementia (bvFTD) and can impair the anticipatory and oral preparatory phases of swallowing. Objective: To characterize... more
Background: Swallowing and feeding problems may occur with the progression of behavioral variant frontotemporal dementia (bvFTD) and can impair the anticipatory and oral preparatory phases of swallowing. Objective: To characterize swallowing problems and the feeding situation of patients with bvFTD and to correlate the swallowing problems with functionality, executive functions, cognitive and behavioral features. Methods: Consecutive outpatients with bvFTD in mild, moderate and severe dementia stages were recruited along with their caregivers. Patients and caregivers were screened with the following scales: “Mini-Mental State Examination”, “Severe Mini-Mental State Examination”, “FTLD-modified Clinical Dementia Rating”, “Neuropsychiatric Inventory”, “Frontal Assessment Battery”, “Index of Independence in Activities of Daily Living”, “Swallowing Rating Scale” and “Assessment of Feeding and Swallowing Difficulties in Dementia”. Results: Overall, thirty patients with bvFTD were include...
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The Brief Cognitive Battery-Edu (BCB-Edu) contains nine tests, seven of which are related to the memory of drawings, and has good accuracy in the diagnosis of cognitive impairment. Objectives: To evaluate the influence of age, gender and... more
The Brief Cognitive Battery-Edu (BCB-Edu) contains nine tests, seven of which are related to the memory of drawings, and has good accuracy in the diagnosis of cognitive impairment. Objectives: To evaluate the influence of age, gender and educational level on the performance in tests related to memory of drawings of the BCB-Edu in healthy subjects. Methods: Participants were adult volunteers; exclusion criteria were illiteracy, neurologic or psychiatric disorders, visual or hearing impairment, untreated chronic clinical conditions, alcoholism, use of drugs, and for those aged 65 or over, an informant report of cognitive or functional impairment. We evaluated 325 individuals (207 women), with a mean age of 47.1 (±16.8) years, ranging from 19 to 81, and a mean of 9.8 (±5.0) schooling-years. Univariate analyses, correlations and logistic regression were employed (a=0.05). Results: There were significant negative correlations between age and the scores in four of the seven tests. However...
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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.
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The Informant Questionnaire on Cognitive Decline in the Elderly with the Proxy (IQCODE) was developed as a screening tool for cognition alterations. Objectives: 1) To verify the applicability of IQCODE in the elderly with limited... more
The Informant Questionnaire on Cognitive Decline in the Elderly with the Proxy (IQCODE) was developed as a screening tool for cognition alterations. Objectives: 1) To verify the applicability of IQCODE in the elderly with limited schooling, 2) To verify the reliability of the responses supplied by the aged and their proxies. Methods: Individuals of a Community Group were evaluated using the Mini-Mental State Examination (MMSE), IQCODE and Geriatric Depression Scale (GDS). The IQCODE was applied to informants and proxies. Results: We analyzed 44 individuals, aged between 58-82 years (M=66.8, SD=5.97) with mean elderly-schooling level of 3.75, SD=2.82 and 44 proxies aged 44.5 (SD=13.3), with mean schooling level of 8.25 (SD=4.3). The mean GDS was 8.22, SD=4.90 and 13 participants presented a score suggestive of depressive symptoms. The mean elderly IQCODE score was 3.26, SD=0.69 and 3.21, SD=0.65, for proxy responses. There was no statistical difference between these means. On the MMS...
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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... more
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 behav...
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Among the instruments to evaluate semantic memory, the Pyramids and Palm Trees (PPT) and the Kissing and Dancing (KDT) tests are widely used but none have a performance referential for cognitively normal and impaired Brazilian... more
Among the instruments to evaluate semantic memory, the Pyramids and Palm Trees (PPT) and the Kissing and Dancing (KDT) tests are widely used but none have a performance referential for cognitively normal and impaired Brazilian populations. Objective: [A] To study these two tests in a sample of young healthy Brazilian individuals living in São Paulo; [B] To apply the results to the evaluation of two cases diagnosed with frontotemporal lobar degeneration. Methods: We evaluated 50 normal participants (41 females and 9 males) aged between 20-63 years, with schooling level of 14-20 years. In addition, two individuals diagnosed with frontotemporal lobar degeneration were examined, one with behavioral-variant frontotemporal dementia and the other with semantic dementia. Results: On the two tests, no effects of age, gender and schooling on the performance of normal individuals were observed. According to the performance of the sample of controls, scores below 46 points on the PPT and below ...
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Mutations in GRN (progranulin) and MAPT (microtubule-associated protein tau) are among the most frequent causes of monogenic frontotemporal dementia (FTD), but data on the frequency of these mutations in regions such as Latin America are... more
Mutations in GRN (progranulin) and MAPT (microtubule-associated protein tau) are among the most frequent causes of monogenic frontotemporal dementia (FTD), but data on the frequency of these mutations in regions such as Latin America are still lacking. We aimed to investigate the frequencies of GRN and MAPT mutations in FTD cohorts from 2 Brazilian dementia research centers, the University of Sao Paulo and the Federal University of Minas Gerais medical schools. We included 76 probands diagnosed with behavioral-variant FTD (n=55), semantic-variant Primary Progressive Aphasia (PPA) (n=11), or nonfluent-variant PPA (n=10). Twenty-five percent of the cohort had at least 1 relative affected with FTD. Mutations in GRN were identified in 7 probands, and in MAPT, in 2 probands. We identified 3 novel GRN mutations (p.Q130X, p.317Afs*12, and p.K259Afs*23) in patients diagnosed with nonfluent-variant PPA or behavioral-variant FTD. Plasma progranulin levels were measured and a cutoff value of 7...
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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... more
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 p...
Research Interests: Psychology and Medicine
Background: Staging scales for dementia have been devised for grading Alzheimer's disease (AD) but do not include the specific symptoms of frontotemporal lobar degeneration (FTLD). Objective: To translate and adapt the Frontotemporal... more
Background: Staging scales for dementia have been devised for grading Alzheimer's disease (AD) but do not include the specific symptoms of frontotemporal lobar degeneration (FTLD). Objective: To translate and adapt the Frontotemporal Dementia Rating Scale (FTD-FRS) to Brazilian Portuguese. Methods: The 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). Results: The Brazilian version of the FTD-FRS seem...
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am in at io n : M o C A 6 M o n tr ea l C o g n it iv e A ss es sm en t Karolina G. Cesar, Sonia M.D. Brucki, Leonel T. Takada, Luiz Fernando C. Nascimento, Camila M.S. Gomes, Milena C.S. Almeida, Maira O. Oliveira, Fabio H.G. Porto,... more
am in at io n : M o C A 6 M o n tr ea l C o g n it iv e A ss es sm en t Karolina G. Cesar, Sonia M.D. Brucki, Leonel T. Takada, Luiz Fernando C. Nascimento, Camila M.S. Gomes, Milena C.S. Almeida, Maira O. Oliveira, Fabio H.G. Porto, Mirna L.H. Senaha, Valeria S. Bahia, Thais Bento Lima Silva, Jessica N. Ianof, Mario A. Cecchini, Luciana Cassimiro, Marcia R. Gonçalves, Jerusa Smid, Claudia S. Porto, Maria Teresa Carthery-Goulart, Mônica Sanches Yassuda, Leticia L. Mansur, Ricardo Nitrini, Sr,, University of S~ao Paulo, S~ao Paulo, Brazil; University of Taubat e, Taubat e, Brazil; Universidade Federal do ABC, S~ao Bernardo do Campo, Brazil. Contact e-mail: karolgcesar@gmail.com
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Depression is a major growing public health problem. Many population studies have found a significant relationship between depression and the presence of cognitive disorders. OBJECTIVE: To establish the correlation between the Visual... more
Depression is a major growing public health problem. Many population studies have found a significant relationship between depression and the presence of cognitive disorders. OBJECTIVE: To establish the correlation between the Visual Analogue Scale of Happiness and the Cornell Scale for Depression in Dementia in the population aged 60 years or over in the city of Tremembé, state of São Paulo, Brazil. METHODS: An epidemiological survey involving home visits was carried out in the city of Tremembé. The sample was randomly selected by drawing 20% of the population aged 60 years or older from each of the city's census sectors. In this single-phase study, the assessment included clinical history, physical and neurological examination, cognitive evaluation, and application of both the Cornell Scale and the Analogue Scale of Happiness for psychiatric symptoms. The presence of depressive symptoms was defined as scores greater than or equal to 8 points on the Cornell Scale. RESULTS: A to...
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The authors report the incidence of dementia in a community-dwelling Brazilian population. In 1997, 1656 individuals aged 65 years or more, the majority being of very low educational level, were screened at their homes in Catanduva,... more
The authors report the incidence of dementia in a community-dwelling Brazilian population. In 1997, 1656 individuals aged 65 years or more, the majority being of very low educational level, were screened at their homes in Catanduva, Brazil, and dementia was diagnosed in 118 cases. The remaining 1538 individuals were rescreened 3.25 years later applying a health questionnaire, the Mini-Mental State Examination (MMSE) and the Pfeffer Functional Activities Questionnaire (PFAQ). According to PFAQ and MMSE scores, selected subjects were submitted to clinical, neurologic, and cognitive evaluations. The subjects diagnosed with dementia underwent laboratory tests and brain computed tomography. A total of 1119 individuals were rescreened and 50 incident cases of dementia (28 with Alzheimer disease [AD]) were identified. The incidence rate of dementia was 13.8 and of AD was 7.7 per 1000 person-years for individuals aged 65 years or older. The incidence rates of dementia almost doubled with ev...
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The aim was to conduct a systematic review of studies that described the functional profile of patients with behavioral variant frontotemporal dementia (bvFTD), published between 2000 and 2013. The bibliographic search was conducted using... more
The aim was to conduct a systematic review of studies that described the functional profile of patients with behavioral variant frontotemporal dementia (bvFTD), published between 2000 and 2013. The bibliographic search was conducted using the terms “frontotemporal dementia” and “frontotemporal lobar degeneration” in combination with “independence,” “functionality,” “basic activities of daily living,” “disability,” and scales that measure functional performance: “Disability Assessment for Dementia-DAD,” “Functional Activities Questionnaire (FAQ),” “Direct Assessment of Functional Status (DAFS).” To be included in the review, the study had to mention the characterization of the functional status of patients with bvFTD in the objectives of the study, using a previously validated instrument of functional assessment. Fourteen studies met this criterion. The reviewed studies suggested that individuals with bvFTD have greater functional impairment when compared to those with other subtypes...
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OBJECTIVE: To analyze the scoring obtained by an instrument, which evaluates the ability to read and understand items in the health care setting, according to education and age. METHODS: The short version of the Test of Functional Health... more
OBJECTIVE: To analyze the scoring obtained by an instrument, which evaluates the ability to read and understand items in the health care setting, according to education and age. METHODS: The short version of the Test of Functional Health Literacy in Adults was administered to 312 healthy participants of different ages and years of schooling. The study was conducted between 2006 and 2007, in the city of São Paulo, Southeastern Brazil. The test includes actual materials such as pill bottles and appointment slips and measures reading comprehension, assessing the ability to read and correctly pronounce a list of words and understand both prose passages and numerical information. Pearson partial correlations and a multiple regression model were used to verify the association between its scores and education and age. RESULTS: The mean age of the sample was 47.3 years(sd=16.8) and the mean education was 9.7 years(sd=5; range: 1 - 17). A total of 32.4% of the sample showed literacy/numeracy...
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Primary progressive aphasia (PPA) is an intriguing syndrome, showing some peculiar aspects that differentiate it from classical aphasic pictures caused by focal cerebral lesions or dementia. The slow and progressive deterioration of... more
Primary progressive aphasia (PPA) is an intriguing syndrome, showing some peculiar aspects that differentiate it from classical aphasic pictures caused by focal cerebral lesions or dementia. The slow and progressive deterioration of language occurring in these cases provides an interesting model to better understand the mechanisms involved in the linguistic process. We describe clinical and neuroimaging aspects found in 16 cases of PPA. Our patients underwent language and neuropsychological evaluation, magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT). We observed a clear distinction in oral expression patterns; patients were classified as fluent and nonfluent. Anomia was the earliest and most evident symptom in both groups. Neuroimaging pointed to SPECT as a valuable instrument in guiding the differential diagnosis, as well as in making useful clinical and anatomical correlations. This report and a comparison to literature are an attempt to...
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Diagnosis of dementia is a challenge in populations with heterogeneous educational background. OBJECTIVE: To compare the accuracies of two delayed recall tests for the diagnosis of dementia in a community with high proportion of... more
Diagnosis of dementia is a challenge in populations with heterogeneous educational background. OBJECTIVE: To compare the accuracies of two delayed recall tests for the diagnosis of dementia in a community with high proportion of illiterates. METHOD: The delayed recall of a word list from the CERAD battery (DR-CERAD) was compared with the delayed recall of objects presented as line drawings from the Brief Cognitive Screening Battery (DR-BCSB) using ROC curves. Illiterate (23 controls and 17 patients with dementia) and literate individuals (28 controls and 17 patients with dementia) were evaluated in a community-dwelling Brazilian population. RESULTS: The DR-BCSB showed higher accuracy than the DR-CERAD in the illiterate (p=0.029), similar accuracy in the literate individuals (p=0.527), and a trend for higher accuracy in the entire population (p=0.084). CONCLUSION: the DR-BCSB could be an alternative for the diagnosis of dementia in populations with high proportion of illiterates.
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The importance of investigating the etiology for dementia lies in the possibility of treating potentially reversible dementias. The aims of this retrospective study are to determine the prevalence of potentially reversible dementias among... more
The importance of investigating the etiology for dementia lies in the possibility of treating potentially reversible dementias. The aims of this retrospective study are to determine the prevalence of potentially reversible dementias among 454 outpatients seen at the Cognitive and Behavioral Neurology Unit, Hospital das Clínicas, São Paulo University School of Medicine - Brazil, between the years of 1991 and 2001, and observe their evolution in follow-up. Among the initial 454 patients, 275 fulfilled the DSM-IV criteria for dementia. Alzheimer's disease was the most frequent diagnosis (164 cases; 59.6%). Twenty-two cases (8.0%) of potentially reversible dementia were observed, the most frequent diagnoses being neurosyphilis (nine cases) and hydrocephalus (six cases). Full recovery was observed in two patients and partial recovery in 10 patients. Two cases were not treated and eight cases were lost on follow-up. The prevalence found in the present study falls within the range repo...
Research Interests: Cognitive Science, Neurology, Dementia, Brazil, Medicine, and 15 moreHumans, Female, Male, Etiology, Ct Scan, Normal Pressure Hydrocephalus, Clinical Sciences, Mini Mental State Examination, Aged, Middle Aged, Educational Status, Alzheimer Disease, Clinical Diagnosis, Brain Tumour, and Neurosyphilis
Alzheimer&#x27;s &amp; Dementia: The Journal of the Alzheimer&#x27;s Association, Volume 4, Issue 4, Pages T443, July 2008, Authors:Sonia MD Brucki; Renato Anghinah; Renata Areza-Fegyveres; Valeria S. Bahia; Maria T.... more
Alzheimer&#x27;s &amp; Dementia: The Journal of the Alzheimer&#x27;s Association, Volume 4, Issue 4, Pages T443, July 2008, Authors:Sonia MD Brucki; Renato Anghinah; Renata Areza-Fegyveres; Valeria S. Bahia; Maria T. Carthery-Goulart; Antonio Damin; Ana P. Formigoni; Norberto Frota; ...
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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... more
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 diagnos...
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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... more
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 w...