Dataset Information

Neuropsychiatric Aspects in a Patient Diagnosed with Frontotemporal Dementia: Clinical Case of Low Incidence and Prevalence Disease in Colombia.

ABSTRACT: Frontotemporal dementia (FTD) is a neuropsychiatric pathology characterized by dysfunctions in the frontal lobe of the brain, especially in planning, execution, and inhibition tasks, with an inability to make decisions and handle different sequences, also affecting the temporal lobe. The patient presents alterations to store, consolidate, and recall information. These neurocognitive deficits are accompanied by neurobehavioral disorders such as depression, anxiety, and apathy that contribute to the worsening of the quality of life, with a high impact on the individual, social, and family level. To identify the neurobehavioral disorders and neurocognitive deterioration that present a patient diagnosed with FTD: clinical case of low incidence and prevalence disease in Colombia. A 40-year-old man, with progressive deterioration of his immediate verbal memory, low verbal fluency, aberrant motor behavior, sexual impulsivity, alterations in his executive functions, especially in planning tasks, decision-making, and inhibition was found to have a lesser degree of affectation in his visuospatial functioning and visuoconstructive abilities. It was found that the patient presents a severe dysexecutive syndrome associated with a clinical picture of FTD, correlated with an inability to process and recall information, accompanied by disorders such as depression, anxiety, and apathy. It is necessary to generate a functional neurorehabilitation plan that aims to improve the quality of life in these patients. In the same way, it is necessary to create new lines of research and intervention that have the purpose to create a greater field of heuristics or new questions in this type of neurodegenerative pathologies.

SUBMITTER: Vergel Hernandez J

PROVIDER: S-EPMC8299406 | biostudies-literature | 2021 May-Aug

REPOSITORIES: biostudies-literature

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Publications

Neuropsychiatric Aspects in a Patient Diagnosed with Frontotemporal Dementia: Clinical Case of Low Incidence and Prevalence Disease in Colombia.

Vergel Hernández Juliana J Barrera Robledo María Eugenia ME Hurtado González Carlos Alberto CA Marmolejo Escobar Carlos Steven CS Ospina Otalvaro Sebastián S Sánchez Tobón Juan David JD Arango de la Pava Pablo Miguel PM Alvarez Juan José JJ

Case reports in neurology 20210501 2

Frontotemporal dementia (FTD) is a neuropsychiatric pathology characterized by dysfunctions in the frontal lobe of the brain, especially in planning, execution, and inhibition tasks, with an inability to make decisions and handle different sequences, also affecting the temporal lobe. The patient presents alterations to store, consolidate, and recall information. These neurocognitive deficits are accompanied by neurobehavioral disorders such as depression, anxiety, and apathy that contribute to t ...[more]

PMID: 34326755

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Project description:ImportanceBehavioral disturbances are core features of frontotemporal dementia (FTD); however, symptom progression across the course of disease is not well characterized in genetic FTD.ObjectiveTo investigate behavioral symptom frequency and severity and their evolution and progression in different forms of genetic FTD.Design, setting, and participantsThis longitudinal cohort study, the international Genetic FTD Initiative (GENFI), was conducted from January 30, 2012, to May 31, 2019, at 23 multicenter specialist tertiary FTD research clinics in the United Kingdom, the Netherlands, Belgium, France, Spain, Portugal, Italy, Germany, Sweden, Finland, and Canada. Participants included a consecutive sample of 232 symptomatic FTD gene variation carriers comprising 115 with variations in C9orf72, 78 in GRN, and 39 in MAPT. A total of 101 carriers had at least 1 follow-up evaluation (for a total of 400 assessments). Gene variations were included only if considered pathogenetic.Main outcomes and measuresBehavioral and neuropsychiatric symptoms were assessed across disease duration and evaluated from symptom onset. Hierarchical generalized linear mixed models were used to model behavioral and neuropsychiatric measures as a function of disease duration and variation.ResultsOf 232 patients with FTD, 115 (49.6%) had a C9orf72 expansion (median [interquartile range (IQR)] age at evaluation, 64.3 [57.5-69.7] years; 72 men [62.6%]; 115 White patients [100%]), 78 (33.6%) had a GRN variant (median [IQR] age, 63.4 [58.3-68.8] years; 40 women [51.3%]; 77 White patients [98.7%]), and 39 (16.8%) had a MAPT variant (median [IQR] age, 56.3 [49.9-62.4] years; 25 men [64.1%]; 37 White patients [94.9%]). All core behavioral symptoms, including disinhibition, apathy, loss of empathy, perseverative behavior, and hyperorality, were highly expressed in all gene variant carriers (>50% patients), with apathy being one of the most common and severe symptoms throughout the disease course (51.7%-100% of patients). Patients with MAPT variants showed the highest frequency and severity of most behavioral symptoms, particularly disinhibition (79.3%-100% of patients) and compulsive behavior (64.3%-100% of patients), compared with C9orf72 carriers (51.7%-95.8% of patients with disinhibition and 34.5%-75.0% with compulsive behavior) and GRN carriers (38.2%-100% with disinhibition and 20.6%-100% with compulsive behavior). Alongside behavioral symptoms, neuropsychiatric symptoms were very frequently reported in patients with genetic FTD: anxiety and depression were most common in GRN carriers (23.8%-100% of patients) and MAPT carriers (26.1%-77.8% of patients); hallucinations, particularly auditory and visual, were most common in C9orf72 carriers (10.3%-54.5% of patients). Most behavioral and neuropsychiatric symptoms increased in the early-intermediate phases and plateaued in the late stages of disease, except for depression, which steadily declined in C9orf72 carriers, and depression and anxiety, which surged only in the late stages in GRN carriers.Conclusions and relevanceThis cohort study suggests that behavioral and neuropsychiatric disturbances differ between the common FTD gene variants and have different trajectories throughout the course of disease. These findings have crucial implications for counseling patients and caregivers and for the design of disease-modifying treatment trials in genetic FTD.

Project description:ImportancePrior research suggests there are racial disparities in the presentation of dementia, but this has not been investigated in the context of frontotemporal dementia (FTD).ObjectiveTo explore racial disparities in dementia severity, functional impairment, and neuropsychiatric symptoms in individuals with a diagnosis of FTD.Design, setting, and participantsThis exploratory cross-sectional study of National Alzheimer's Coordinating Center (NACC) data collected between June 2005 to August 2021 evaluated Asian, Black, and White individuals with a diagnosis of FTD (behavioral variant FTD or primary progressive aphasia). Excluded were races with limited data, including American Indian or Alaska Native (n = 4), Native Hawaiian or other Pacific Islander (n = 3), other (n = 13), and unknown (n = 24), and participants with symptom duration more than 4 SDs above the mean.Main outcomes and measuresRacial differences at initial NACC visit were examined on Clinical Dementia Rating Dementia Staging Instrument plus NACC Frontotemporal Lobar Degeneration Behavior & Language Domains (FTLD-CDR), Functional Assessment Scale, and Neuropsychiatric Inventory using regression models. Matching was also performed to address the imbalance between racial groups.ResultsThe final sample comprised 2478 individuals, of which 59 (2.4%) were Asian, 63 (2.5%) were Black, and 2356 (95.1%) were White. The mean (SD) age at initial visit was 65.3 (9.4) years and symptom duration at initial visit was 67.5 (35.6) months. Asian and Black individuals were considerably underrepresented, comprising a small percent of the sample. Black individuals had a higher degree of dementia severity on FTLD-CDR (β = 0.64; SE = 0.24; P = .006) and FTLD-CDR sum of boxes (β = 1.21; SE = 0.57; P = .03) and greater functional impairment (β = 3.83; SE = 1.49; P = .01). There were no differences on FTLD-CDR and Functional Assessment Scale between Asian and White individuals. Black individuals were found to exhibit a higher frequency of delusions, agitation, and depression (delusions: odds ratio [OR], 2.18; 95% CI, 1.15-3.93; P = .01; agitation: OR, 1.73; 95% CI, 1.03-2.93; P = .04; depression: OR, 1.75; 95% CI, 1.05-2.92; P = .03). Asian individuals were found to exhibit a higher frequency of apathy (OR, 1.89; 95% CI, 1.09-3.78; P = .03), nighttime behaviors (OR, 1.72; 95% CI, 1.01-2.91; P = .04), and appetite/eating (OR, 1.99; 95% CI, 1.17-3.47; P = .01) compared to White individuals.Conclusions and relevanceThis exploratory study suggests there are racial disparities in dementia severity, functional impairment, and neuropsychiatric symptoms. Future work must address racial disparities and their underlying determinants as well as the lack of representation of racially minoritized individuals in nationally representative dementia registries.

Dataset Information

Neuropsychiatric Aspects in a Patient Diagnosed with Frontotemporal Dementia: Clinical Case of Low Incidence and Prevalence Disease in Colombia.

Publications

Neuropsychiatric Aspects in a Patient Diagnosed with Frontotemporal Dementia: Clinical Case of Low Incidence and Prevalence Disease in Colombia.

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