scholarly journals Analysis of a case series of behavioral variant frontotemporal dementia: Emphasis on diagnostic delay

2013 ◽  
Vol 7 (1) ◽  
pp. 55-59
Author(s):  
Henrique Cerqueira Guimarães ◽  
Thiago Cardoso Vale ◽  
Victor Pimentel ◽  
Nayara Carvalho de Sá ◽  
Rogério Gomes Beato ◽  
...  

ABSTRACT Introduction: Despite many advances in the characterization of the behavioral variant of frontotemporal dementia (bvFTD), the diagnosis of this syndrome poses a significant challenge, while delays or diagnostic mistakes may impact the proper clinical management of these patients. Objective: To describe the clinical profile at first evaluation of a sample of patients with bvFTD from a specialized outpatient neurological unit, with emphasis on the analysis of the delay between the onset of symptoms and diagnosis. Methods: We selected 31 patients that fulfilled international consensus criteria for possible or probable bvFTD. Patients' medical admission sheets were thoroughly reviewed. Results: Patients' mean age was 67.9±8.2 years; 16 (51.6%) were men. Mean number of years of formal education was 7.7±4.0 years. Mean age at onset was 62.2±7.7 years, indicating a mean of 5.8 years of diagnostic delay. Thirteen patients (41.9%) presented with initial behavioral complaints only, eleven patients (35.5%) had mixed behavioral and memory complaints, five patients (16.1%) presented with memory complaints only, and two patient (6.4%) had behavioral and speech problems. Nine patients (29%) were admitted with alternative diagnoses. Mean and standard deviation scores for the mini-mental state examination, animal category fluency and memory test for drawings (five-minute delayed recall) were 19.3±6.3, 8.3±4.1 and 3.7±2.7, respectively. Conclusion: Most patients from this sample were evaluated almost six years after the onset of symptoms and performed poorly on both cognitive screening tests and functional evaluation measures.

2016 ◽  
Vol 14 (3) ◽  
pp. 398-402 ◽  
Author(s):  
Patrícia Romano Opasso ◽  
Simone dos Santos Barreto ◽  
Karin Zazo Ortiz

ABSTRACT Objective: To establish normative parameters for the F-A-S form of the phonemic verbal fluency test, in a population of Brazilian Portuguese speaking adults with high-level literacy. Methods: The sample comprised 40 male and female volunteers aged 19 to 59 years, and at least 8 years of formal education. Volunteers were first submitted to the Mini-Mental State Examination and the Clock Drawing cognitive screening tests, then to the F-A-S Verbal Phonemic Fluency Test; in this test, examinees were given 60 seconds to generate as many words as possible beginning with each of the three test letters. Results: The means for number of words beginning the letters F, A and S and for total number of words beginning with either letter generated per minute corresponded to 15.3, 14.4, 13.9 and 43.5, respectively. Conclusion: Reference values obtained from young adults with high levels of literacy submitted to the F-A-S Verbal Phonemic Fluency Test in this study were similar to those reported in the international literature. These reference values can be used for clinical assessment of language disorder and neuropsychological evaluation.


2008 ◽  
Vol 2 (4) ◽  
pp. 315-320 ◽  
Author(s):  
Regina Miksian Magaldi ◽  
Alexandre Leopold Busse ◽  
Venceslau Antonio Coelho ◽  
Daniel Apolinário ◽  
Leonardo da Costa Lopes ◽  
...  

Abstract Memory complaints are common in elderly, and may be associated with many clinical problems. Objective: To identify clinical conditions and possible factors related to memory complaints in elderly outpatients presenting at a tertiary unit. Methods: Patients with memory complaints and normal cognitive screening tests were submitted to clinical and laboratorial testing. Radiological evaluation was performed as needed for diagnosis. Results: One hundred and seventy-seven subjects were initially evaluated, 12 were excluded because of poor and inconsistent memory complaints. Of the remainder, seventeen had criteria for dementia diagnosis. Ninety-two (55.4%) had one or more comorbid conditions possibly related to their complaints. Major depression was present in 26.0%. Sixteen patients (9.6%) had vitamin B12 deficiency, 8 were in use of inappropriate medications, and 7 (4.2%) had hypothyroidism. Other conditions diagnosed were: generalized anxiety disorder, obstructive sleep apnea syndrome, hyperparathyroidism, normal pressure hydrocephalus. Three patients had severe hearing loss (in 22 with hearing complaints); one had severe visual impairment (in 22 with visual complaints). Conclusions: Comprehensive evaluation was able to identify treatable conditions possibly related to memory complaints.


2019 ◽  
Author(s):  
Ashita S. Gurnani ◽  
Shayne S.-H. Lin ◽  
Brandon E Gavett

Objective: The Colorado Cognitive Assessment (CoCA) was designed to improve upon existing screening tests in a number of ways, including enhanced psychometric properties and minimization of bias across diverse groups. This paper describes the initial validation study of the CoCA, which seeks to describe the test; demonstrate its construct validity; measurement invariance to age, education, sex, and mood symptoms; and compare it to the Montreal Cognitive Assessment (MoCA). Method: Participants included 151 older adults (MAge = 71.21, SD = 8.05) who were administered the CoCA, MoCA, Judgment test from the Neuropsychological Assessment Battery (NAB), 15-item version of the Geriatric Depression Scale (GDS-15), and 10-item version of the Geriatric Anxiety Scale (GAS-10). Results: A single factor confirmatory factor analysis model of the CoCA fit the data well, CFI = 0.955; RMSEA = 0.033. The CoCA’s internal consistency reliability was .84, compared to .74 for the MoCA. The CoCA had stronger disattenuated correlations with the MoCA (r = .79) and NAB Judgment (r = .47) and weaker correlations with the GDS-15 (r = -.36) and GAS-10 (r = -.15), supporting its construct validity. Finally, when analyzed using multiple indicators, multiple causes (MIMIC) modeling, the CoCA showed no evidence of measurement non-invariance, unlike the MoCA. Conclusions: These results provide initial evidence to suggest that the CoCA is a valid cognitive screening tool that offers numerous advantages over the MoCA, including superior psychometric properties and measurement non-invariance. Additional validation and normative studies are warranted.


2020 ◽  
Vol 78 (2) ◽  
pp. 537-541
Author(s):  
Jordi A. Matias-Guiu ◽  
Vanesa Pytel ◽  
Jorge Matías-Guiu

We aimed to evaluate the frequency and mortality of COVID-19 in patients with Alzheimer’s disease (AD) and frontotemporal dementia (FTD). We conducted an observational case series. We enrolled 204 patients, 15.2% of whom were diagnosed with COVID-19, and 41.9% of patients with the infection died. Patients with AD were older than patients with FTD (80.36±8.77 versus 72.00±8.35 years old) and had a higher prevalence of arterial hypertension (55.8% versus 26.3%). COVID-19 occurred in 7.3% of patients living at home, but 72.0% of those living at care homes. Living in care facilities and diagnosis of AD were independently associated with a higher probability of death. We found that living in care homes is the most relevant factor for an increased risk of COVID-19 infection and death, with AD patients exhibiting a higher risk than those with FTD.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii324-iii324
Author(s):  
Derek Hanson ◽  
Nicolas Andre ◽  
Susan Chi ◽  
Mariella Filbin ◽  
Michael Fisher ◽  
...  

Abstract Embryonal tumors with multilayer rosettes (ETMR) are rare and highly-aggressive central nervous system (CNS) neoplasms which occur primarily in young children and carry a dismal prognosis. To date, no large clinical investigations have been conducted to determine the optimal therapy for ETMR. Data from retrospective case series suggest that our most aggressive standard therapies are not sufficient for cure in the majority of cases. New treatment approaches incorporating pre-clinical data and the known biology of ETMR are therefore urgently needed. A German drug screen using the patient-derived ETMR BT183 cell line and its xenograft revealed anti-tumor activity of topotecan, doxorubicin, and actinomycin D; three agents used infrequently for treating infant CNS tumors. Additional results from a small series of ETMR patients suggest that optimization of induction chemotherapy using these active agents may improve response and survival outcomes. In 2019, an international panel of pediatric neuro-oncology experts convened to advance therapy for ETMR. A consensus protocol was developed incorporating maximal safe surgical resection, induction chemotherapy with active pre-clinical agents, intrathecal chemotherapy, radiotherapy, and high-dose chemotherapy. This international consensus protocol represents the first prospective clinical investigation specific to ETMR and will be available through a treatment registry globally and as a clinical trial at select centers. The study aims to improve survival by providing aggressive, optimized therapy for ETMR and will serve as a platform to explore new biologically-promising agents. The investigation will also provide valuable prospective outcome data and correlative biological studies to serve as baseline comparators for future clinical trials.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1272.1-1272
Author(s):  
M. Ben Majdouba ◽  
S. Boussaid ◽  
S. Rekik ◽  
S. Jemmali ◽  
H. Ajlani ◽  
...  

Background:Work productivity of patients with spondyloarthritis is frequently affected by their disease.Objectives:We aim to identify disease-related factors associated with poor work productivity in patients with spondyloarthritis.Methods:A cross-sectional study was performed in patients with spondyloarthritis. Data on disease characteristics were collected as well as specific indices: Visual analogue scale (VAS) for fatigue and pain, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score with CRP (ASDAS-CRP), Bath Ankylosing Spondylitis Functionnel Index (BASFI) and Bath Ankylosing Spondylitis Metrology Index (BASMI). EuroQol-5D (EQ5D) was used to assess health-related quality of life. Work productivity was assessed by the Work Productivity and Activity Impairment scale (WPAI:SpA). Factors associated with presenteeism, absenteeism and work productivity loss were evaluated.Results:One hundred patients were enrolled (73 men and 27 women); mean age was 43.68 ± 10.3 years. Fifty nine percent of patients were employed, 26% were off work and 15% were retired of which 8% were in early retirement. Sixty seven percent of patients had ankylosing spondylitis, 17% had rheumatism associated with inflammatory bowel disease and 16% had psoriatic rheumatism. The average disease duration was 12.24 ± 8.73 years. Mean age at onset was 33.2 ± 10 years [18-59]. The average diagnostic delay was 2.41 ± 3 years; it was more than five years in 17% of cases. Sacroiliac pain has been noted in 69 patients, lumbar or cervical stiffness in 78 patients and peripheral joint involvement in 18 cases. Thirty one percent of patients had hip joint involvement and 49% had extra-articular manifestation. Fifty percent had inflammatory biological syndrome, 63% were treated with anti-TNFα and 58% needed symptomatic treatment regularly. The mean fatigue and pain VAS was respectively 5.58 ± 2.5 and 5.56 ± 2.9. The mean BASDAI was 4.4 ± 2.4, the average BASFI was 4.6 ± 2.7 and the average ASDAS-CRP was 2.77 ± 1.18. The mean BASMI was 4.4 ± 2.8. The mean EQ5D score was 0.485 ± 0.378. Among employed patients, mean absenteeism, presenteeism and work productivity loss was 21.8 ± 33.13%, 42 ± 32% and 46.5 ± 35.31%, respectively. These work outcomes were correlated to diagnostic delay ≥ 2 years (p<0.03), peripheral joint involvement (p=0.006), psoriasis (p=0.02), inflammatory biological syndrome (p<0.001), need of symptomatic treatment (p=0.001), fatigue and pain VAS ≥ 4 (p<0.001), BASDAI ≥ 4 (p<0.001), ASDAS-CRP ≥ 2.1 (p<0.001), BASFI ≥ 4 (p<0.001), BASMI ≥ 4 (p=0.002) and low EQ5D score (p<0.001). Work productivity loss was in addition correlated to age at onset < 25 years (p=0.03).Conclusion:Active disease, reduced physical function and poorer quality of life are associated with reduced work productivity. Early diagnosis and good disease management especially fatigue and pain can potentially improve work outcomes in patients with spondyloarthritis.Disclosure of Interests:None declared.


2013 ◽  
Vol 5 (3) ◽  
pp. 16 ◽  
Author(s):  
Fábio Henrique De Gobbi Porto ◽  
Lívia Spíndola ◽  
Maira Okada De Oliveira ◽  
Patrícia Helena Figuerêdo Do Vale ◽  
Marco Orsini ◽  
...  

It is not easy to differentiate patients with mild cognitive impairment (MCI) from subjective memory complainers (SMC). Assessments with screening cognitive tools are essential, particularly in primary care where most patients are seen. The objective of this study was to evaluate the diagnostic accuracy of screening cognitive tests and to propose a score derived from screening tests. Elderly subjects with memory complaints were evaluated using the Mini Mental State Examination (MMSE) and the Brief Cognitive Battery (BCB). We added two delayed recalls in the MMSE (a delayed recall and a late-delayed recall, LDR), and also a phonemic fluency test of letter P fluency (LPF). A score was created based on these tests. The diagnoses were made on the basis of clinical consensus and neuropsychological testing. Receiver operating characteristic curve analyses were used to determine area under the curve (AUC), the sensitivity and specificity for each test separately and for the final proposed score. MMSE, LDR, LPF and delayed recall of BCB scores reach statistically significant differences between groups (P=0.000, 0.03, 0.001 and 0.01, respectively). Sensitivity, specificity and AUC were MMSE: 64%, 79% and 0.75 (cut off &lt;29); LDR: 56%, 62% and 0.62 (cut off &lt;3); LPF: 71%, 71% and 0.71 (cut off &lt;14); delayed recall of BCB: 56%, 82% and 0.68 (cut off &lt;9). The proposed score reached a sensitivity of 88% and 76% and specificity of 62% and 75% for cut off over 1 and over 2, respectively. AUC were 0.81. In conclusion, a score created from screening tests is capable of discriminating MCI from SMC with moderate to good accurancy.


2019 ◽  
Vol 15 ◽  
pp. P1042-P1042
Author(s):  
Peter Roos ◽  
Peter Johannsen ◽  
Suzanne G. Lindquist ◽  
Jerry Brown ◽  
Jette Stokholm ◽  
...  

2009 ◽  
Vol 3 (2) ◽  
pp. 94-100 ◽  
Author(s):  
Thaís Bento Lima-Silva ◽  
Mônica Sanches Yassuda

Abstract Normal aging can be characterized by a gradual decline in some cognitive functions, such as memory. Memory complaints are common among older adults, and may indicate depression, anxiety, or cognitive decline. Objectives: To investigate the association between memory complaints and age in cognitively unimpaired older adults, and the relationship between memory complaints and memory performance. Methods: Cognitive screening tests as well as memory complaint questionnaires validated for the Brazilian population were used: the Mini-Mental State Examination (MMSE), Geriatric Depression Scale (GDS), Memory Complaint Questionnaire (MAC-Q), Memory test of 18 pictures, Forward and Backward Digit Span (WAIS-III). Fifty seven regular members of the SESC social club participated (50 women), having a mean age of 71.4 years, and 4 to 8 years of education - 34 from 4 to 7 years and 23 with 8 years of education. Results: Results revealed no significant association between cognitive complaints and age or cognitive performance. Older participants in this sample did not show worse performance or a higher level of complaints. There was no significant association between age and GDS scores. Conclusions: The studied sample constitutes a particular group of older adults whose participation in activities may be protecting them from cognitive decline, thus highlighting the impact of lifestyle on cognitive performance during the aging process.


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