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Frontotemporal Dementia

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The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.

Also Known As:

Dementia, Frontotemporal, with Parkinsonism; Dementia, Hereditary Dysphasic Disinhibition; Frontotemporal Dementia with Parkinsonism; Frontotemporal Dementia, Ubiquitin-Positive; Frontotemporal Lobe Dementia (FLDEM); Multiple System Tauopathy with Presenile Dementia; Dementia, Frontotemporal; Dementia, Frontotemporal Lobe (FLDEM); Dementia, Ubiquitin-Positive Frontotemporal; Dementias, Frontotemporal; Dementias, Frontotemporal Lobe (FLDEM); Dementias, Ubiquitin-Positive Frontotemporal; Frontotemporal Dementia, Ubiquitin Positive; Frontotemporal Dementias; Frontotemporal Dementias, Ubiquitin-Positive; Frontotemporal Lobe Dementias (FLDEM); Ubiquitin-Positive Frontotemporal Dementia; Ubiquitin-Positive Frontotemporal Dementias

Networked: 903 relevant articles (16 outcomes, 74 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Alzheimer Disease (Alzheimer's Disease)
2.	Dementia (Dementias)
3.	Parkinson Disease (Parkinson's Disease)
4.	Vascular Dementia (Subcortical Arteriosclerotic Encephalopathy)
5.	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)

Experts

1.	Miller, Bruce L: 27 articles (09/2015 - 11/2003)
2.	Rademakers, Rosa: 26 articles (07/2015 - 05/2004)
3.	Trojanowski, John Q: 24 articles (09/2014 - 05/2003)
4.	Kimonis, Virginia E: 22 articles (05/2015 - 09/2003)
5.	Dickson, Dennis W: 21 articles (07/2015 - 10/2003)
6.	Hodges, John R: 19 articles (10/2015 - 02/2005)
7.	Lee, Virginia M-Y: 19 articles (08/2013 - 05/2003)
8.	Josephs, Keith A: 16 articles (07/2015 - 10/2003)
9.	Wszolek, Zbigniew K: 15 articles (01/2015 - 05/2007)
10.	Gao, Fen-Biao: 15 articles (12/2014 - 09/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Frontotemporal Dementia:

1.	Proteins (Proteins, Gene)IBA 12/30/2002 - "Comparative proteomic analysis of cerebrospinal fluid (CSF) proteins was employed for studies of the pathophysiological mechanisms in frontotemporal dementia (FTD). " 01/01/2011 - "Clinical diagnosis alone has had variable success in helping to predict pathology, and is particularly poor in the diagnosis of behavioral variant frontotemporal dementia, which can be associated with all three abnormal proteins. " 09/22/2006 - "Aberrantly regulated proteins in frontotemporal dementia." 11/01/2001 - "Pathophysiological aspects of frontotemporal dementia--emphasis on cytoskeleton proteins and autoimmunity." 05/23/2000 - "Cytoskeleton proteins in CSF distinguish frontotemporal dementia from AD."
2.	Cholinesterase Inhibitors (Anticholinesterases)IBA 06/01/2009 - "This case confirms the idea that treatment with cholinesterase inhibitors can be effective in the behavioural and psychological symptoms of frontotemporal dementia." 11/01/2004 - "Efficacy of acetylcholinesterase inhibitors in frontotemporal dementia." 11/01/1998 - "It is centred on the distinction between Alzheimer's disease and other degenerative diseases still often misdiagnosed with Alzheimer's disease such as frontotemporal dementias, dementia with Lewy bodies, and some focal atrophies that do not have the same physiopathology and should not be treated with anticholinesterase drugs. " 08/01/2011 - "Cholinesterase inhibitors are not effective in frontotemporal dementia and may cause agitation (A), though selective serotonin reuptake inhibitors may help behavioural (but not cognitive) features (B). " 11/01/2008 - "Studies on Alzheimer's disease and Lewy body dementias show that cholinesterase inhibitors are still first line treatment for these diseases and memantine is indicated in moderate/severe Alzheimer's disease, whereas there is as yet no standard available treatment for frontotemporal dementias. "
3.	Memantine (Namenda)FDA Link 01/01/2015 - "There is no conclusive evidence supporting the efficacy of memantine in frontotemporal dementia (FTD). " 03/01/2013 - "To follow up on the increases we reported in normalized metabolic activity in salience network hubs from a 2-month open-label study of memantine in frontotemporal dementia (FTD). " 01/01/2013 - "To study the effect of memantine on apathy, a common symptom of behavioral variant frontotemporal dementia (bvFTD). " 01/01/2011 - "Open-label study of the short-term effects of memantine on FDG-PET in frontotemporal dementia." 07/01/2008 - "A 6-month, open-label study of memantine in patients with frontotemporal dementia."
4.	Yi-Gan SanIBA 04/01/2010 - "The aim of the present study was to investigate the efficacy of Yokukansan in improving behavioral symptoms of frontotemporal dementia. " 04/01/2010 - "This study was a prospective, open-label trial of daily Yokukansan for 4 weeks in 20 frontotemporal dementia patients. " 04/01/2010 - "The results indicate that Yokukansan can alleviate the behavioral symptoms of frontotemporal dementia. " 04/01/2010 - "Pilot study of pharmacological treatment for frontotemporal dementia: effect of Yokukansan on behavioral symptoms." 01/01/2012 - "Recent studies have shown that yokukansan might also be quite effective against BPSD occurring in association with other types of dementia, such as Alzheimer's disease, Lewy body disease, Parkinson's disease with dementia, frontotemporal dementia, and vascular dementia. "
5.	Amyloid (Amyloid Fibrils)IBA 07/01/2012 - "Cerebrospinal fluid amyloid-β 2-42 is decreased in Alzheimer's, but not in frontotemporal dementia." 01/01/2012 - "We clinically diagnosed the proband as atypical AD with frontotemporal dementia features and diagnosed the elder brother of the proband as typical AD, based on neuropsychological symptoms and a brain imaging examination including amyloid imaging data. " 01/01/2008 - "Frontotemporal dementia (FTD) is a syndrome that can be clinically difficult to distinguish from AD, but in FTD amyloid deposition is not a characteristic pathological finding. " 01/01/2008 - "In vivo amyloid imaging with PET in frontotemporal dementia." 01/09/2007 - "Finally, PS mutations have been identified in frontotemporal dementia, which lacks amyloid pathology. "
6.	alpha-SynucleinIBA 07/26/2003 - "Linkage to the alpha-synuclein gene and the Frontotemporal dementia with parkinsonism locus on chromosome 17 had previously been excluded in the families included in this study. " 03/01/2005 - "Here we report the cases of 2 brothers with familial progressive aphasia who developed features of frontotemporal dementia with predominant tau pathology but also alpha-synuclein pathology. " 03/01/2005 - "Tau and alpha-synuclein inclusions in a case of familial frontotemporal dementia and progressive aphasia." 03/01/2004 - "17q-linked frontotemporal dementia-amyotrophic lateral sclerosis without tau mutations with tau and alpha-synuclein inclusions." 07/01/2002 - "Aggregates of mutated huntingtin, alpha-synuclein, or unidentified protein in motor neuron disease type of frontotemporal dementia were always negative. "
7.	Glucose (Dextrose)FDA LinkGeneric 10/01/2007 - "The aim of this study was to investigate the MAPT haplotypes in relation to risk for, and functional alterations of glucose metabolism in, patients with frontotemporal dementia (FTD). " 01/01/2004 - "The aim of this study was to examine the pattern of glucose uptake and the changes over time of metabolic deficits in patients with frontotemporal dementia (FTD). " 01/01/2004 - "Region-specific decline of cerebral glucose metabolism in patients with frontotemporal dementia: a prospective 18F-FDG-PET study." 10/08/2013 - "In this prospective cohort study, we investigated cerebral glucose metabolism reductions on [(18)F]-fluorodeoxyglucose (FDG)-PET in progranulin (GRN) mutation carriers prior to frontotemporal dementia (FTD) onset. " 10/01/2007 - "Association of the tau haplotype H2 with age at onset and functional alterations of glucose utilization in frontotemporal dementia."
8.	Neurotransmitter Agents (Neurotransmitter)IBA 01/10/2006 - "Studies of neurotransmitter deficiencies in frontotemporal dementia (FTD) can be helpful in developing treatments. " 05/01/2009 - "[Frontotemporal dementia: neurotransmitter and clinical symptoms with focus on therapeutic targets]." 05/01/2008 - "The dopaminergic neurotransmitter system is associated with aggression and agitation in frontotemporal dementia." 01/10/2006 - "To evaluate neurotransmitter deficiencies and neurotransmitter-based treatments for frontotemporal dementia (FTD). " 01/10/2006 - "A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia."
9.	rivastigmine (Exelon)FDA LinkGeneric 06/01/2009 - "Their effectiveness in frontotemporal dementia has not been proved, since such a claim has only been backed by the publication of one open-label trial in which the behavioural and psychological symptoms of the patients treated with rivastigmine over a 12-month period improved significantly with respect to those belonging to a group that were given a placebo. " 01/01/2004 - "Rivastigmine in frontotemporal dementia: an open-label study." 06/01/2009 - "We report a case of frontotemporal dementia, Pick's disease, which improved with rivastigmine treatment. " 06/01/2009 - "[Rivastigmine in a case of autopsy proved frontotemporal dementia (Pick's disease)]." 01/01/2004 - "This preliminary open-label study aims to investigate the effects of rivastigmine, an inhibitor of acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE), in 20 patients diagnosed with frontotemporal dementia (FTD). "
10.	aripiprazole (Abilify)FDA Link 02/01/2013 - "Aripiprazole for sexually inappropriate vocalizations in frontotemporal dementia." 01/01/2007 - "Clinical improvement in a case of frontotemporal dementia under aripiprazole treatment corresponds to partial recovery of disturbed frontal glucose metabolism." 10/01/2010 - "Sequential therapeutic trials for catatonoid frontal signs in clinically-evident frontotemporal dementia (n = 2) revealed differential benefits for lorazepam, amantadine, memantine, pramipexole, aripiprazole, quetiapine, citalopram, and donepezil, although certain signs also worsened. "

Therapies and Procedures

1.	Activities of Daily Living (ADL) 03/01/2013 - "Neural correlates of activities of daily living in frontotemporal dementia." 06/01/2009 - "Emotion, social functioning and activities of daily living in frontotemporal dementia." 01/01/2009 - "Frontotemporal dementia (FTD) causes progressive change in activities of daily living (ADLs) and little is known about their rate of decline. " 01/01/2009 - "Activities of daily living in behavioral variant frontotemporal dementia: differences in caregiver and performance-based assessments." 01/01/2008 - "Zarit burden inventory and activities of daily living in the behavioral variant of frontotemporal dementia."
2.	Drug Therapy (Chemotherapy) 11/01/2008 - "This review is divided into four sections: First, a brief review of frontotemporal dementia spectrum disorders; second, a discussion of the challenges in the development of drug therapies third, a review of the current clinical trials; and finally a discussion of some recent discoveries, which have sparked new areas of investigation. " 11/01/2008 - "To discuss the development of drug therapies for frontotemporal dementia spectrum disorders, both those under current investigation and those that could be targets for future investigation. " 11/01/2008 - "Challenges and new opportunities in the investigation of new drug therapies to treat frontotemporal dementia." 03/01/2008 - "Imbalance of a serotonergic system in frontotemporal dementia: implication for pharmacotherapy." 04/01/2003 - "Drug therapy of frontotemporal dementia."
3.	Investigational Therapies (Experimental Therapy) 05/01/2013 - "The sensitivity of magnetoencephalography to cortical network changes in behavioral variant frontotemporal dementia enriches the understanding of neurocognitive systems as well as showing potential for studies of experimental therapies for neurodegenerative disease."
4.	Therapeutics 04/01/2015 - "However, recent studies show that almost all cases of ALS, as well as tau-negative frontotemporal dementia (FTD), share a common neuropathology characterized by the deposition of TAR-DNA binding protein (TDP)-43-positive protein inclusions, offering an attractive target for the design and testing of novel therapeutics. "
5.	Lasers (Laser) 01/01/2007 - "Protein profiles of human cerebrospinal fluid samples from patients with AD (n = 85), frontotemporal dementia (n = 20), and healthy controls (n = 32) were analyzed by surface-enhanced laser desorption/ionization time-of-flight mass spectrometry to verify previously discovered biomarkers. " 12/01/2005 - "This investigation describes the discovery of novel possible cerebrospinal fluid (CSF) biomarkers for frontotemporal dementia (FTD) using surface-enhanced laser desorption/ionization time-of-flight (SELDI-TOF) mass spectrometry (MS). " 05/23/2000 - "Laser light scattering was then used to measure the efficiency with which the mutant tau proteins found in frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17) form filamentous structures. "