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Neurology
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
This is defined as an acquired, progressive cognitive impairment that interferes with activities of daily living. The four most common causes are Alzheimer’s Disease, Vascular Dementia, Dementia with Lewy Bodies and Frontotemporal Lobar Degeneration.
Degenerative Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James A. Mastrianni, Elizabeth A. Harris
For a diagnosis of bvFTD with definite frontotemporal lobar degeneration (FTLD) pathology, the following must be present: Criteria for possible or probable bvFTD.Evidence of FTD-related pathology (biopsy or postmortem) and/or a known pathogenic mutation.
A case study of an emerging visual artist with frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Published in Howard J. Rosen, Robert W. Levenson, Neurocase, 2020
Anli Liu, Kelly Werner, Subhojit Roy, John Q. Trojanowski, Ursula Morgan-Kane, Bruce L. Miller, Katherine P. Rankin
Frontotemporal lobar degeneration is a progressive neurodegenerative disorder disproportionately affecting the frontal and anterior temporal lobes (Neary et al., 1998). Some clinical subtypes of FTLD help illuminate the nature of artistic creativity. Previous case studies of patients with left-sided FTLD syndromes, whether temporally predominant (semantic dementia) or frontally predominant (progressive non-fluent aphasia) reveal that a new preoccupation with art, greater attention to visual stimuli, and increased visual creativity sometimes occur during the early stages of these kinds of dementia. In contrast, Alzheimer’s disease (AD), which typically first affects the posterior parietal and medial temporal areas, is associated with decreased visuo-constructive ability (Miller & Hou, 2004; Miller et al., 1998). Creativity in FTLD has been observed in visual art, as well as music, mechanics, and mathematics, but not in writing or poetry (Mendez, 2004; Miller, Boone, Cummerings, Read, & Mishkin, 2000).
Validity of the Wechsler Abbreviated Scale of Intelligence, Second Edition (WASI-II) as an Indicator of Neurological Disease/Injury: A Pilot Study
Published in Brain Injury, 2021
Joseph J. Ryan, David S. Kreiner, Gordon Teichner, Samuel T. Gontkovsky
This investigation demonstrated that the WASI-II is sensitive to the biological condition of the brain. Based on group data, the meaningful differences between patients with neurological disorders and individuals with psychiatric and age-related concerns were purely in terms of level of performance. The Psychiatric Control group scored significantly better on all WASI-II variables relative to patients with brain disorders. As mentioned previously, there was no evidence that patient groups differed in terms of subtest pattern or the relationship between the VCI and PRI. This latter finding, however, should be accepted for mean score analyses only. When ability test scores are averaged, there is always the possibility that the uniqueness of some profiles will be lost to a statistical approach focused on the group mean and standard deviation. The Gontkovsky (2017) case study of a patient afflicted with frontotemporal lobar degeneration is a prime example of this possibility (6).
Epigenetic regulatory modifications in genetic and sporadic frontotemporal dementia
Published in Expert Review of Neurotherapeutics, 2018
Chiara Fenoglio, Elio Scarpini, Daniela Galimberti
Frontotemporal Dementia (FTD) is the second most common cause of dementia after Alzheimer’s disease (AD), affecting people from 45 to 65 years. The prevalence has been estimated as 3–26% worldwide in people more than 65 years old [1]. The term FTD encompasses three clinically distinct syndromes: behavioral variant (bv) FTD, Progressive Non-Fluent Aphasia (PNFA), and Semantic Dementia (SD) [2]. Clinical features of FTD are different according to the different subtype of disease. In general, typical features of bvFTD are the presence of behavioral disturbances, aggressiveness, lack of empathy, decline in social conduct, often associated with cognitive and executive impairment, whereas in PNFA and SD language impairment is the most prominent feature [2]. At the pathological level, all syndromes described are collectively grouped as Frontotemporal Lobar Degeneration (FTLD). At the histopathological level, based on the type of protein depositing, FTLD is classified into FTLD-Tau, FTLD-TAR DNA Binding protein (TDP)43, and FTLD fused in Sarcoma (FUS) [3].
The changing landscape of neuroimaging in frontotemporal lobar degeneration: from group-level observations to single-subject data interpretation
Published in Expert Review of Neurotherapeutics, 2022
Mary Clare McKenna, Aizuri Murad, William Huynh, Jasmin Lope, Peter Bede
The neuroimaging signature of frontotemporal lobar degeneration (FTLD) has been refined by robust computational imaging studies in recent years. This has led to the characterization of phenotype- [1–16] and genotype-associated [17–23] patterns of anatomical involvement and trajectories of longitudinal progression. These findings have contributed important academic insights to our understanding of FTLD biology. However, the practical clinical utility of group-level observations is contingent on the reliable interpretation of individual MRI scans. In recent years, a multitude of classification models have been trialed to capitalize on group-level traits and categorize single-subject MRI data into diagnostic and prognostic subgroups.