Frontotemporal lobar degeneration

Grossman, Murray; Seeley, William W.; Boxer, Adam L.; Hillis, Argye E.; Knopman, David S.; Ljubenov, Peter A.; Miller, Bruce; Piguet, Olivier; Rademakers, Rosa; Whitwell, Jennifer L.; Zetterberg, Henrik; van Swieten, John C.

doi:10.1038/s41572-023-00447-0

Cited by 39 publications

(22 citation statements)

References 294 publications

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“…Thirteen treatments are under investigation for dementia with Lewy bodies . Current investigational medications for frontotemporal degeneration have been focused on people with identifiable genetic variations ( GRN , C9ORF72 ), as well as on various mechanisms to suppress expression and pathological dysregulation of tau and progranulin pathways …”

Section: Disease Treatmentmentioning

confidence: 99%

Dementia Prevention and Treatment

Reuben,

Kremen,

Maust

2024

JAMA Intern Med

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ImportanceDementia affects 10% of those 65 years or older and 35% of those 90 years or older, often with profound cognitive, behavioral, and functional consequences. As the baby boomers and subsequent generations age, effective preventive and treatment strategies will assume increasing importance.ObservationsPreventive measures are aimed at modifiable risk factors, many of which have been identified. To date, no randomized clinical trial data conclusively confirm that interventions of any kind can prevent dementia. Nevertheless, addressing risk factors may have other health benefits and should be considered. Alzheimer disease can be treated with cholinesterase inhibitors, memantine, and antiamyloid immunomodulators, with the last modestly slowing cognitive and functional decline in people with mild cognitive impairment or mild dementia due to Alzheimer disease. Cholinesterase inhibitors and memantine may benefit persons with other types of dementia, including dementia with Lewy bodies, Parkinson disease dementia, vascular dementia, and dementia due to traumatic brain injury. Behavioral and psychological symptoms of dementia are best treated with nonpharmacologic management, including identifying and mitigating the underlying causes and individually tailored behavioral approaches. Psychotropic medications have minimal evidence of efficacy for treating these symptoms and are associated with increased mortality and clinically meaningful risks of falls and cognitive decline. Several emerging prevention and treatment strategies hold promise to improve dementia care in the future.Conclusions and RelevanceAlthough current prevention and treatment approaches to dementia have been less than optimally successful, substantial investments in dementia research will undoubtedly provide new answers to reducing the burden of dementia worldwide.

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Section: Disease Treatmentmentioning

confidence: 99%

Dementia Prevention and Treatment

Reuben,

Kremen,

Maust

2024

JAMA Intern Med

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“…Quality control was carried out on all MRI data according to previous described procedures 1 , and data management and processing were done through our in house database system 2 . As the registration process is particularly challenging in patients with significant brain atrophy, this process was visually inspected for each subject included in the study (which can be reviewed in supplementary material: Registration_All.pdf).…”

Section: Image Quality Controlmentioning

confidence: 99%

“…Behavioural variant frontotemporal dementia (bvFTD) is a syndrome of a neurodegenerative disease commonly affecting the anterior frontal, temporal and insular cortices. 1 Symptoms of bvFTD include a combination of changes in behaviour (disinhibition, lack of motivation/ apathy, repetitive behaviour and altered eating habits), and cognitive dysfunction (mainly in social cognition, executive functions and language), concomitant to a lack of insight. 1,2 Important drivers of the changes in behaviour are loss of social cognitive functions such as emotional recognition, understanding of social norms, mentalization (“theory of mind”), moral reasoning, and empathic function, which occur together to varying degrees (for reviews see, 3,4 ).…”

Section: Introductionmentioning

confidence: 99%

Altered empathy processing in frontotemporal dementia A task-based fMRI study

Lindberg,

Li,

Lind

et al. 2024

Preprint

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A lack of empathy, and particularly its affective components, is a core symptom of behavioural variant frontotemporal dementia (bvFTD). Visual exposure to images of a needle pricking a hand (pain condition) and Q-tips touching a hand (control condition) is an established functional magnetic resonance imaging (fMRI) paradigm used to investigate empathy for pain (EFP; pain condition minus control condition). EFP has been associated with increased blood oxygen level dependent (BOLD) signal in regions known to become atrophic in the early stages in bvFTD, including the anterior insula and the anterior cingulate. We therefore hypothesized that patients with bvFTD would display altered empathy processing in the EFP paradigm. Here we examined empathy processing using the EFP paradigm in 28 patients with bvFTD and 28 sex and age matched controls. Participants underwent structural MRI, task-based and resting-state fMRI. The Interpersonal Reactivity Index (IRI) was used as a measure of different facets of empathic function outside the scanner. The EFP paradigm was analysed at a whole brain level and using two regions-of-interest approaches, one based on a metanalysis of affective perceptual empathy versus cognitive evaluative empathy and one based on the control's activation pattern. In controls, EFP was linked to an expected increase of BOLD signal that displayed an overlap with the pattern of atrophy in the bvFTD patients (insula and anterior cingulate). Additional regions with increased signal were the supramarginal gyrus and the occipital cortex. These latter regions were the only ones that displayed increased BOLD signal in bvFTD patients. BOLD signal increase under the affective perceptual empathy but not the cognitive evaluative empathy region of interest was significantly greater in controls than in bvFTD patients. The control's rating on their empathic concern subscale of the IRI was significantly correlated with the BOLD signal in the EFP paradigm, as were an informant's ratings of the patient's empathic concern subscale. This correlation was not observed on other subscales of the IRI or when using the patient's self-ratings. Finally, controls and patients showed different connectivity patterns in empathy related networks during resting-state fMRI, mainly in nodes overlapping the ventral attention network. Our results indicate that reduced neural activity in regions typically affected by pathology in bvFTD is associated with reduced empathy processing, and a predictor of patient's capacity to experience affective empathy.

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“…However, In the second edition, in 2000, there is an 83-page chapter on aging, Alzheimer's disease, and dementia. 25 Many of the neurologists who were previously dedicated to the study of neuropsychology migrated to the field of dementia, bringing with them the clinical and anatomical knowledge that has been so important to recognize or better redefine forms of degenerative dementia, such as Primary Progressive Aphasia, 26 Behavioral Variant of Frontal Dementia, 27,28 or new signs for the clinical diagnosis of these conditions. 29,30 ARE AD AND SENILE DEMENTIA ONLY ONE OR TWO DIFFERENT DISEASES?…”

Section: The Relatively Recent Pastmentioning

confidence: 99%

The past, present and future of Alzheimer's disease – part 1: the past

Nitrini

2023

Arq Neuropsiquiatr

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Background Alzheimer's disease (AD) was described in 1907, and since then it changed from a relatively rare condition to one of the most prevalent diseases. Objective To describe the evolution of the notions of dementias and AD, and to investigate the reasons for the increase in scientific interest in AD. Methods A historical analysis was carried out on knowledge about dementia, the site of mental activity, the relationships between brain diseases and mental activity, and on the advances in research about AD, since its discovery until the publication of the amyloid cascade hypothesis in 1992. A search was carried out in the National Library of Medicine (PubMed) for scientific articles that included the terms dementia or AD over 50 years, from 1972 to 2021. Results The scientific research on AD increased from 615 papers with the term AD in the first decade (1972-1981), to 100,028 papers in the last decade (2012-2021): an increase of 162.6 times whereas publications with the term dementia increased 28.6 times in the same period. In the 1960s and 1970s, a consensus was reached that AD is responsible for the majority of cases of dementia previously known as senile dementia. In the 1980s, beta-amyloid peptide was identified in the core of the senile plaque, hyperphosphorylated tau protein was found in neurofibrillary tangles, and a mutation was discovered in a hereditary form of AD. Conclusion The expansion of the concept of AD to include senile dementia, and the discoveries that occurred in the 1980s greatly expanded research in AD.

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Frontotemporal lobar degeneration

Cited by 39 publications

References 294 publications

Dementia Prevention and Treatment

Dementia Prevention and Treatment

Altered empathy processing in frontotemporal dementia A task-based fMRI study

The past, present and future of Alzheimer's disease – part 1: the past

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