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frontotemporal lobar dementia

Keiko Imamura, Naruhiko Sahara, Nicholas M Kanaan, Kayoko Tsukita, Takayuki Kondo, Yumiko Kutoku, Yutaka Ohsawa, Yoshihide Sunada, Koichi Kawakami, Akitsu Hotta, Satoshi Yawata, Dai Watanabe, Masato Hasegawa, John Q Trojanowski, Virginia M-Y Lee, Tetsuya Suhara, Makoto Higuchi, Haruhisa Inoue
Mutations in the gene MAPT encoding tau, a microtubules-associated protein, cause a subtype of familial neurodegenerative disorder, known as frontotemporal lobar degeneration tauopathy (FTLD-Tau), which presents with dementia and is characterized by atrophy in the frontal and temporal lobes of the brain. Although induced pluripotent stem cell (iPSC) technology has facilitated the investigation of phenotypes of FTLD-Tau patient neuronal cells in vitro, it remains unclear how FTLD-Tau patient neurons degenerate...
October 10, 2016: Scientific Reports
Ashley N Nilson, Kelsey C English, Julia E Gerson, T Barton Whittle, C Nicolas Crain, Judy Xue, Urmi Sengupta, Diana L Castillo-Carranza, Wenbo Zhang, Praveena Gupta, Rakez Kayed
It is well-established that inflammation plays an important role in Alzheimer's disease (AD) and frontotemporal lobar dementia (FTLD). Inflammation and synapse loss occur in disease prior to the formation of larger aggregates, but the contribution of tau to inflammation has not yet been thoroughly investigated. Tau pathologically aggregates to form large fibrillar structures known as tangles. However, evidence suggests that smaller soluble aggregates, called oligomers, are the most toxic species and form prior to tangles...
October 1, 2016: Journal of Alzheimer's Disease: JAD
Amado Rivero-Santana, Daniel Ferreira, Lilisbeth Perestelo-Pérez, Eric Westman, Lars-Olof Wahlund, Antonio Sarría, Pedro Serrano-Aguilar
BACKGROUND: Differential diagnosis in dementia is at present one of the main challenges both in clinical practice and research. Cerebrospinal fluid (CSF) biomarkers are included in the current diagnostic criteria of Alzheimer's disease (AD) but their clinical utility is still unclear. OBJECTIVE: We performed a systematic review of studies analyzing the diagnostic performance of CSF Aβ42, total tau (t-tau), and phosphorylated tau (p-tau) in the discrimination between AD and frontotemporal lobar degeneration (FTLD) dementias...
October 4, 2016: Journal of Alzheimer's Disease: JAD
Ludivine Chamard, Sabrina Ferreira, Alexa Pijoff, Manon Silvestre, Eric Berger, Eloi Magnin
Objectives. To describe cognitive assessment including social cognition in SPG4 patients. Methods. We reported a series of nine patients with SPG4 mutation with an extensive neuropsychological examination including social cognition assessment. Results. None of our patients presented with mental retardation or dementia. All presented with mild cognitive impairment with a high frequency of attention deficit (100%), executive disorders (89%), and social cognition impairment (78%). An asymptomatic patient for motor skills presented with the same cognitive profile...
2016: Behavioural Neurology
H Bea Kuiperij, Alexandra A M Versleijen, Marijke Beenes, Nicolaas A Verwey, Luisa Benussi, Anna Paterlini, Giuliano Binetti, Charlotte E Teunissen, Joost Raaphorst, Helenius J Schelhaas, Benno Küsters, Yolande A L Pijnenburg, Roberta Ghidoni, Marcel M Verbeek
BACKGROUND: Frontotemporal dementia (FTD) is a heterogeneous disease both at the clinical, genetic, and pathobiological level. The underlying pathological spectrum (termed FTLD, frontotemporal lobar degeneration) is in most cases defined by accumulation of either tau (FTLD-tau) or TDP-43 proteins (FTLD-TDP). Biomarkers to differentiate these subtypes are not yet available, whereas these are essential requirements to study the natural course of disease and for homogeneous inclusion of patients in clinical studies...
September 23, 2016: Journal of Alzheimer's Disease: JAD
Joery Goossens, Jorne Laton, Jeroen Van Schependom, Jeroen Gielen, Hanne Struyfs, Sara Van Mossevelde, Tobi Van den Bossche, Johan Goeman, Peter Paul De Deyn, Anne Sieben, Jean-Jacques Martin, Christine Van Broeckhoven, Julie van der Zee, Sebastiaan Engelborghs, Guy Nagels
We investigated the power of EEG as biomarker in differential diagnosis of Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD). EEG was recorded from 106 patients with AD or FTLD, of which 37 had a definite diagnosis, and 40 controls. Dominant frequency peaks were extracted for all 19 channels, for each subject. The average frequency of the largest dominant frequency peaks (maxpeak) was significantly lower in AD than FTLD patients and controls. Based on ROC analysis, classification could be made with diagnostic accuracy of 78...
September 12, 2016: Journal of Alzheimer's Disease: JAD
R A Armstrong
Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy (FTLD-TDP). Kaplan-Meier survival analysis estimated mean survival as 7.9 years (range: 1-19 years, SD = 4.64). Familial and sporadic cases exhibited similar survival, including progranulin (GRN) gene mutation cases. No significant differences in survival were associated with sex, disease onset, Braak disease stage, or disease subtype, but higher survival was associated with lower post-mortem brain weight...
2016: Folia Neuropathologica
Alexandra M Nicholson, Rosa Rademakers
Frontotemporal lobar degeneration is a neurodegenerative disorder affecting over 50,000 people in the United States alone. The most common pathological subtype of FTLD is the presence of ubiquitinated TAR DNA binding protein 43 (TDP-43) accumulations in frontal and temporal brain regions at autopsy. While some cases of FTLD-TDP can be attributed to the inheritance of disease-causing mutations, the majority of cases arise with no known genetic cause. In 2010, the first genome-wide association study was conducted in patients with FTLD-TDP to determine potential genetic risk factors for this homogenous subgroup of dementia patients, leading to the identification of the TMEM106B locus on chromosome 7...
August 20, 2016: Acta Neuropathologica
Richard M Ransohoff
Neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and frontotemporal lobar dementia are among the most pressing problems of developed societies with aging populations. Neurons carry out essential functions such as signal transmission and network integration in the central nervous system and are the main targets of neurodegenerative disease. In this Review, I address how the neuron's environment also contributes to neurodegeneration. Maintaining an optimal milieu for neuronal function rests with supportive cells termed glia and the blood-brain barrier...
August 19, 2016: Science
Hiroaki Kazui, Kenji Yoshiyama, Hideki Kanemoto, Yukiko Suzuki, Shunsuke Sato, Mamoru Hashimoto, Manabu Ikeda, Hibiki Tanaka, Yutaka Hatada, Masateru Matsushita, Yoshiyuki Nishio, Etsuro Mori, Satoshi Tanimukai, Kenjiro Komori, Taku Yoshida, Hideaki Shimizu, Teruhisa Matsumoto, Takaaki Mori, Tetsuo Kashibayashi, Kazumasa Yokoyama, Tatsuo Shimomura, Yasunobu Kabeshita, Hiroyoshi Adachi, Toshihisa Tanaka
BACKGROUND/AIMS: Behavioral and psychological symptoms of dementia (BPSDs) negatively impact the prognosis of dementia patients and increase caregiver distress. The aims of this study were to clarify the differences of trajectories of 12 kinds of BPSDs by disease severity in four major dementias and to develop charts showing the frequency, severity, and associated caregiver distress (ACD) of BPSDs using the data of a Japan multicenter study (J-BIRD). METHODS: We gathered Neuropsychiatric Inventory (NPI) data of patients with Alzheimer's disease (AD; n = 1091), dementia with Lewy bodies (DLB; n = 249), vascular dementia (VaD; n = 156), and frontotemporal lobar degeneration (FTLD; n = 102) collected during a 5-year period up to July 31, 2013 in seven centers for dementia in Japan...
2016: PloS One
Christian Haass, Manuela Neumann
Frontotemporal dementia (FTD) is a heterogeneous clinical syndrome characterized by frontotemporal lobar degeneration (FTLD). Neuropathologically, FTLD is characterized by abnormal protein deposits and almost all cases can now be classified into three major molecular subgroups based on specific accumulating proteins with the most common being FTLD-tau and FTLD-TDP (accounting for ~40% and 50%, respectively) and FTLD-FET (accounting for ~5-10%). In this special issue, the molecular and genetic basics as well as clinical approaches and therapeutics are reviewed in a series of articles...
August 2016: Journal of Neurochemistry
Viviana Lo Buono, Francesco Corallo, Angela Marra, Rocco Salvatore Calabrò, Placido Bramanti, Silvia Marino
OBJECT: Semantic dementia is one of the main clinical variants of frontotemporal dementia and it is characterized by severe loss of conceptual knowledge with relative preservation of other cortical abilities. METHODS: We described a case of a patient affected by right temporal variant of frontotemporal lobar degeneration, characterized by prosopagnosia as an unusual disease onset. RESULTS: The neuropsychological evaluation showed a progressive deficit in face recognition and a slow decline in language in the absence of behavioral alterations...
August 4, 2016: Acta Clinica Belgica
Eneida Mioshi, Emma Flanagan, David Knopman
OBJECTIVE: To investigate the psychometric properties of the Clinical Dementia Scale-frontotemporal lobar degeneration (CDR-FTLD) psychometric properties using Rasch analysis and its sensitivity distinguishing disease progression between FTLD and Alzheimer's disease (AD). METHODS: Of 603 consecutive patients from the National Alzheimer Coordinating Center dataset (FTLD = 350; AD = 253), 120 FTLDs were included in a Rasch analysis to verify CDR-FTLD psychometric properties; 483 (FTLD = 230; AD = 253) were included to analyse disease progression, with 195 (FTLD = 82; AD = 113) followed-up (24 months)...
July 28, 2016: International Journal of Geriatric Psychiatry
Anthony Papegaey, Sabiha Eddarkaoui, Vincent Deramecourt, Francisco-Jose Fernandez-Gomez, Pierre Pantano, Hélène Obriot, Camille Machala, Vincent Anquetil, Agnès Camuzat, Alexis Brice, Claude-Alain Maurage, Isabelle Le Ber, Charles Duyckaerts, Luc Buée, Nicolas Sergeant, Valérie Buée-Scherrer
Reduction of Tau protein expression was described in 2003 by Zhukareva et al. in a variant of frontotemporal lobar degeneration (FTLD) referred to as diagnosis of dementia lacking distinctive histopathology, then re-classified as FTLD with ubiquitin inclusions. However, the analysis of Tau expression in FTLD has not been reconsidered since then. Knowledge of the molecular basis of protein aggregates and genes that are mutated in the FTLD spectrum would enable to determine whether the "Tau-less" is a separate pathological entity or if it belongs to an existing subclass of FTLD...
2016: Acta Neuropathologica Communications
Alireza Atri
This chapter reviews clinical applications and imaging findings useful in medical practice relating to neurodegenerative cognitive/dementing disorders. The preponderance of evidence and consensus guidelines support an essential role of multitiered neuroimaging in the evaluation and management of neurodegenerative cognitive/dementia syndrome that range in severity from mild impairments to frank dementia. Additionally, imaging features are incorporated in updated clinical and research diagnostic criteria for most dementias, including Alzheimer's disease (AD), Dementia with Lewy bodies (DLB), Frontotemporal Lobar Degenerations/Frontotemporal Dementia (FTD), and Vascular Cognitive Impairment (VCI)...
2016: Handbook of Clinical Neurology
Pantelis Maiovis, Panagiotis Ioannidis, Georgios Gerasimou, Anna Gotzamani-Psarrakou, Dimitrios Karacostas
Severity assessment scales for frontotemporal lobar degeneration (FTLD) have been recently introduced. In the present study, the authors examined whether the FTLD-modified Clinical Dementia Rating (FTLD-CDR) scale and the Frontotemporal Dementia Rating Scale (FRS) correlated with regional brain perfusion in Greek FTLD patients. A total of 47 behavioral variant frontotemporal dementia (bvFTD) patients and 33 primary progressive aphasia (PPA) patients were assessed for demographic data, cognitive reserve (CR), and severity of dementia and underwent brain single-photon emission computed tomography...
July 15, 2016: Journal of Neuropsychiatry and Clinical Neurosciences
Jennifer M Harris, Matthew Jones, Claire Gall, Anna M T Richardson, David Neary, Daniel du Plessis, Piyali Pal, David M A Mann, Julie S Snowden, Jennifer C Thompson
BACKGROUND/OBJECTIVES: We aimed to evaluate the co-occurrence of language and behavioural impairment in patients with frontotemporal lobar degeneration (FTLD) spectrum pathology. METHODS: Eighty-one dementia patients with pathological confirmation of FTLD were identified. Anonymized clinical records from patients' first assessment were rated for language and behavioural features from frontotemporal dementia consensus criteria, primary progressive aphasia (PPA) criteria and 1998 FTLD criteria...
May 2016: Dementia and Geriatric Cognitive Disorders Extra
Kiyotaka Nakamagoe, Kotarou Kadono, Tadachika Koganezawa, Mao Takiguchi, Makoto Terada, Fumiko Yamamoto, Tetsuya Moriyama, Kumi Yanagiha, Seitaro Nohara, Naoki Tozaka, Zenshi Miyake, Satoshi Aizawa, Kentaro Furusho, Akira Tamaoka
BACKGROUND: No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system. OBJECTIVE: The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD. METHODS: Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study...
May 2016: Dementia and Geriatric Cognitive Disorders Extra
Pan Li, Wei Quan, Yu-Ying Zhou, Yan Wang, Hui-Hong Zhang, Shuai Liu
Previous studies have focused on the curative effects of memantine in patients with mild-to-moderate frontotemporal lobar degeneration (FTLD); however, its benefits in patients with moderate-to-severe FTLD have not been investigated. The present study explores the behavioral, cognitive and functional effects of memantine on behavioral variant frontotemporal dementia (bvFTD) in patients with mild and moderate-to-severe stage bvFTD. A total of 42 patients with bvFTD completed a 6-month treatment plan of 20 mg memantine daily in an open-label, self-controlled clinical trial...
July 2016: Experimental and Therapeutic Medicine
Christopher J Holler, Georgia Taylor, Zachary T McEachin, Qiudong Deng, William J Watkins, Kathryn Hudson, Charles A Easley, William T Hu, Chadwick M Hales, Wilfried Rossoll, Gary J Bassell, Thomas Kukar
BACKGROUND: Progranulin (PGRN) is a secreted growth factor important for neuronal survival and may do so, in part, by regulating lysosome homeostasis. Mutations in the PGRN gene (GRN) are a common cause of frontotemporal lobar degeneration (FTLD) and lead to disease through PGRN haploinsufficiency. Additionally, complete loss of PGRN in humans leads to neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disease. Importantly, Grn-/- mouse models recapitulate pathogenic lysosomal features of NCL...
2016: Molecular Neurodegeneration
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