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Logopenic dementia

Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
September 20, 2016: Journal of Alzheimer's Disease: JAD
Jonathan D Rohrer, Ione O C Woollacott, Katrina M Dick, Emilie Brotherhood, Elizabeth Gordon, Alexander Fellows, Jamie Toombs, Ronald Druyeh, M Jorge Cardoso, Sebastien Ourselin, Jennifer M Nicholas, Niklas Norgren, Simon Mead, Ulf Andreasson, Kaj Blennow, Jonathan M Schott, Nick C Fox, Jason D Warren, Henrik Zetterberg
OBJECTIVE: To investigate serum neurofilament light chain (NfL) concentrations in frontotemporal dementia (FTD) and to see whether they are associated with the severity of disease. METHODS: Serum samples were collected from 74 participants (34 with behavioral variant FTD [bvFTD], 3 with FTD and motor neuron disease and 37 with primary progressive aphasia [PPA]) and 28 healthy controls. Twenty-four of the FTD participants carried a pathogenic mutation in C9orf72 (9), microtubule-associated protein tau (MAPT; 11), or progranulin (GRN; 4)...
September 27, 2016: Neurology
Gamze Balci Camsari, Melissa E Murray, Neill R Graff-Radford
Many dementia subtypes have more shared signs and symptoms than defining ones. We review 8 cases with 4 overlapping syndromes and demonstrate how to distinguish the cases. These include focal cortical presentations of Alzheimer's disease (AD; posterior cortical atrophy and corticobasal syndrome [CBS]), fluent aphasia (semantic dementia and logopenic aphasia), late-onset slowly progressive dementia (hippocampal sclerosis and limbic predominant AD) and rapidly progressive dementia (Creutzfeldt-Jakob disease and limbic encephalitis)...
August 2016: Neurologic Clinics
Tim Van Langenhove, Cristian E Leyton, Olivier Piguet, John R Hodges
BACKGROUND: Differentiating between primary progressive aphasia (PPA) variants based on the profile of language deficits can be difficult in a proportion of patients. Further, little is presently know about the pattern of longitudinal changes in behavior in PPA variants. OBJECTIVE: To determine the presence of behavioral changes in the main variants of PPA: semantic (sv-PPA), nonfluent/agrammatic (nfv-PPA), and logopenic (lv-PPA), and establish the course of these changes over time...
June 18, 2016: Journal of Alzheimer's Disease: JAD
Samrah Ahmed, Ian Baker, Sian Thompson, Masud Husain, Christopher R Butler
INTRODUCTION: Existing literature suggests that the presence or absence of apraxia and associated parietal deficits may be clinically relevant in differential diagnosis of dementia syndromes. AIM: This study investigated the profile of these features in Alzheimer's disease (AD) and frontotemporal dementia (FTD) spectrum disorders, at first presentation. METHODS: Retrospective case note analysis was undertaken in 111 patients who presented to the Oxford Cognitive Disorders Clinic, Oxford, UK, including 29 amnestic AD, 12 posterior cortical atrophy (PCA), 12 logopenic primary progressive aphasia (lvPPA), 20 behavioural variant FTD (bvFTD), 7 non-fluent variant PPA (nfvPPA), 6 semantic variant PPA (svPPA) and 25 patients with subjective cognitive impairment (SCI)...
June 1, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
Young Kyoung Jang, Seongbeom Park, Hee Jin Kim, Hanna Cho, Chul Hyoung Lyoo, Sang Won Seo, Duk L Na
BACKGROUND: Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). PATIENTS AND METHODS: We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA...
January 2016: Case Reports in Neurology
Rik Ossenkoppele, Daniel R Schonhaut, Michael Schöll, Samuel N Lockhart, Nagehan Ayakta, Suzanne L Baker, James P O'Neil, Mustafa Janabi, Andreas Lazaris, Averill Cantwell, Jacob Vogel, Miguel Santos, Zachary A Miller, Brianne M Bettcher, Keith A Vossel, Joel H Kramer, Maria L Gorno-Tempini, Bruce L Miller, William J Jagust, Gil D Rabinovici
SEE SARAZIN ET AL DOI101093/BRAIN/AWW041 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: The advent of the positron emission tomography tracer (18)F-AV1451 provides the unique opportunity to visualize the regional distribution of tau pathology in the living human brain. In this study, we tested the hypothesis that tau pathology is closely linked to symptomatology and patterns of glucose hypometabolism in Alzheimer's disease, in contrast to the more diffuse distribution of amyloid-β pathology. We included 20 patients meeting criteria for probable Alzheimer's disease dementia or mild cognitive impairment due to Alzheimer's disease, presenting with a variety of clinical phenotypes, and 15 amyloid-β-negative cognitively normal individuals, who underwent (18)F-AV1451 (tau), (11)C-PiB (amyloid-β) and (18)F-FDG (glucose metabolism) positron emission tomography, apolipoprotein E (APOE) genotyping and neuropsychological testing...
May 2016: Brain: a Journal of Neurology
S Bisenius, J Neumann, M L Schroeter
Recently, diagnostic clinical and imaging criteria for primary progressive aphasia (PPA) have been revised by an international consortium (Gorno-Tempini et al. Neurology 2011;76:1006-14). The aim of this study was to validate the specificity of the new imaging criteria and investigate whether different imaging modalities [magnetic resonance imaging (MRI) and fluorodeoxyglucose positron emission tomography (FDG-PET)] require different diagnostic subtype-specific imaging criteria. Anatomical likelihood estimation meta-analyses were conducted for PPA subtypes across a large cohort of 396 patients: firstly, across MRI studies for each of the three PPA subtypes followed by conjunction and subtraction analyses to investigate the specificity, and, secondly, by comparing results across MRI vs...
April 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Siddharth Ramanan, Emma Flanagan, Cristian E Leyton, Victor L Villemagne, Christopher C Rowe, John R Hodges, Michael Hornberger
Diagnostic distinction of primary progressive aphasias (PPA) remains challenging, in particular for the logopenic (lvPPA) and nonfluent/agrammatic (naPPA) variants. Recent findings highlight that episodic memory deficits appear to discriminate these PPA variants from each other, as only lvPPA perform poorly on these tasks while having underlying amyloid pathology similar to that seen in amnestic dementias like Alzheimer's disease (AD). Most memory tests are, however, language based and thus potentially confounded by the prevalent language deficits in PPA...
2016: Journal of Alzheimer's Disease: JAD
Sheilla de Medeiros Correia Marin, Paulo Henrique Ferreira Bertolucci, Luis Fabiano Marin, Fabricio Ferreira de Oliveira, Jose Roberto Wajman, Valéria Santoro Bahia, Letícia Lessa Mansur
BACKGROUND: Few studies have described characteristics of swallowing in primary progressive aphasia (PPA) and its variants. OBJECTIVE: To describe and characterize swallowing and eating behaviors of patients with PPA, as well as their correlates with neuropsychiatric symptoms and patterns of communication. METHODS: We studied 16 patients with PPA and 16 their caregivers. PPA was subdivided in agrammatic variant (PPA-G), semantic variant (PPA-S) and logopenic variant (PPA-L)...
2016: NeuroRehabilitation
Sicong Tu, Cristian E Leyton, John R Hodges, Olivier Piguet, Michael Hornberger
BACKGROUND: Clinico-pathological distinction of primary progressive aphasia (PPA) can be challenging at clinic presentation. In particular, cross-sectional neuroimaging signatures across the logopenic (lvPPA) and semantic (svPPA) variants are difficult to establish, with longitudinal profiles showing greater divergence. OBJECTIVE: Assess longitudinal propagation of white matter degradation in lvPPA and svPPA to determine disease progression over time, and whether this reflects distinct underlying pathology...
2015: Journal of Alzheimer's Disease: JAD
Ajay Madhavan, Christopher G Schwarz, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Daniel A Drubach, Kejal Kantarci, Scott A Przybelski, Robert I Reid, Matthew L Senjem, Jeffrey L Gunter, Liana G Apostolova, Val J Lowe, Ronald C Petersen, Clifford R Jack, Keith A Josephs, Jennifer L Whitwell
BACKGROUND: Different clinical syndromes can arise from Alzheimer's disease (AD) neuropathology, including dementia of the Alzheimer's type (DAT), logopenic primary progressive aphasia (lvPPA), and posterior cortical atrophy (PCA). OBJECTIVE: To assess similarities and differences in patterns of white matter tract degeneration across these syndromic variants of AD. METHODS: Sixty-four subjects (22 DAT, 24 lvPPA, and 18 PCA) that had diffusion tensor imaging and showed amyloid-β deposition on PET were assessed in this case-control study...
2015: Journal of Alzheimer's Disease: JAD
Rik Ossenkoppele, Brendan I Cohn-Sheehy, Renaud La Joie, Jacob W Vogel, Christiane Möller, Manja Lehmann, Bart N M van Berckel, William W Seeley, Yolande A Pijnenburg, Maria L Gorno-Tempini, Joel H Kramer, Frederik Barkhof, Howard J Rosen, Wiesje M van der Flier, William J Jagust, Bruce L Miller, Philip Scheltens, Gil D Rabinovici
Alzheimer's disease (AD) can present with distinct clinical variants. Identifying the earliest neurodegenerative changes associated with each variant has implications for early diagnosis, and for understanding the mechanisms that underlie regional vulnerability and disease progression in AD. We performed voxel-based morphometry to detect atrophy patterns in early clinical stages of four AD phenotypes: Posterior cortical atrophy (PCA, "visual variant," n=93), logopenic variant primary progressive aphasia (lvPPA, "language variant," n=74), and memory-predominant AD categorized as early age-of-onset (EOAD, <65 years, n=114) and late age-of-onset (LOAD, >65 years, n=114)...
November 2015: Human Brain Mapping
Carl Taswell, Victor L Villemagne, Paul Yates, Hitoshi Shimada, Cristian E Leyton, Kirrie J Ballard, Olivier Piguet, James R Burrell, John R Hodges, Christopher C Rowe
UNLABELLED: Alzheimer disease is the cause of up to one-third of cases of primary progressive aphasia or corticobasal syndrome. The primary objective of this study was to determine the accuracy of 18F-FDG PET metabolic imaging for the detection of Alzheimer disease in patients with primary progressive aphasia or corticobasal syndrome. METHODS: A cohort of patients (n=94), including those with an expert clinical diagnosis of logopenic (n=19), nonfluent (n=16), or semantic (n=13) variants of primary progressive aphasia, corticobasal syndrome (n=14), or Alzheimer disease (n=24), underwent 18F-FDG metabolic and 11C-labeled Pittsburgh compound B (11C-PiB) amyloid PET brain imaging...
October 2015: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Alon Seifan, Stephanie Assuras, Edward D Huey, Jesse Mez, Angeliki Tsapanou, Elise Caccappolo
OBJECTIVE: To further our understanding of the association between self-reported childhood learning disabilities (LDs) and atypical dementia phenotypes (Atypical Dementia), including logopenic primary progressive aphasia (L-PPA), Posterior Cortical Atrophy (PCA), and Dysexecutive-type Alzheimer's Disease (AD). METHODS: This retrospective case series analysis of 678 comprehensive neuropsychological assessments compared rates of self-reported LD between dementia patients diagnosed with Typical AD and those diagnosed with Atypical Dementia...
2015: PloS One
Hugo Botha, Joseph R Duffy, Jennifer L Whitwell, Edythe A Strand, Mary M Machulda, Christopher G Schwarz, Robert I Reid, Anthony J Spychalla, Matthew L Senjem, David T Jones, Val Lowe, Clifford R Jack, Keith A Josephs
The consensus criteria for the diagnosis and classification of primary progressive aphasia (PPA) have served as an important tool in studying this group of disorders. However, a large proportion of patients remain unclassifiable whilst others simultaneously meet criteria for multiple subtypes. We prospectively evaluated a large cohort of patients with degenerative aphasia and/or apraxia of speech using multidisciplinary clinical assessments and multimodal imaging. Blinded diagnoses were made using operational definitions with important differences compared to the consensus criteria...
August 2015: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
Shun Shimohama
Dementia manifests during the presenile and senile periods in many diseases and pathological conditions. Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD) are the two main diseases in which cortical dementia is observed. FTLD includes frontotemporal dementia (FTD), progressive non-fluent aphasia (PNFA), and semantic dementia (SD). Cortical dementia has also been described in posterior cortical atrophy (CPA) and primary progressive non-fluent or logopenic aphasia (PNFLA). This article describes and discusses historical changes in the disease concept; diagnostic characteristics, including pathology and imaging analysis; clinical symptoms based on local cognitive function in the cerebral cortex; and other recent topics on dementia...
April 2015: Brain and Nerve, Shinkei Kenkyū No Shinpo
Chrem Mendez Patricio, Cohen Gabriela, Russo Maria Julieta, Fernandez Suarez Marcos, Nahas Federico, Russo Griselda, Wierszylo Claudio, Paz Santiago, Tabaschi Leonardo, Campos Jorge, Amengual Alejandra, Kremer Janus, Guinjoan Salvador, Leiguarda Ramon, Sevlever Gustavo, Vázquez Silvia, Allegri Ricardo
INTRODUCTION: Today, ligands that bind to fibrillar β-amyloid are detectable by Positron Emission Tomography (PET) allowing for in vivo visualization for Abeta burden. However, amyloid plaques detection per se does not establish Alzheimer's Disease diagnosis. In this sense, the utility of amyloid imaging to improve clinical diagnosis was settled only for specific clinical scenarios and few studies have assessed amyloid molecular neuroimaging in a broader clinical setting. The aim of this study is to determine the frequency of PiB amyloid findings in different diagnostic syndromes grouped into high and low probability pre- test categories, taking into account pre-test clinical assumption of the presence of AD related pathology...
September 2015: American Journal of Alzheimer's Disease and Other Dementias
Eoin P Flanagan, Matthew C Baker, Ralph B Perkerson, Joseph R Duffy, Edythe A Strand, Jennifer L Whitwell, Mary M Machulda, Rosa Rademakers, Keith A Josephs
BACKGROUND: Mutations in three genes [chromosome 9 open-reading-frame 72 (C9ORF72); microtubule-associated protein tau (MAPT) and progranulin (GRN)] account for the vast majority of familial, and a proportion of sporadic, frontotemporal dementia (FTD) cases. Progressive apraxia of speech (PAOS) is a type of FTD characterized by speech production deficits without a known cause. METHODS: We therefore assessed for genetic mutations in C9ORF72, MAPT and GRN in 40 prospectively recruited PAOS patients...
2015: Dementia and Geriatric Cognitive Disorders
Jennifer L Whitwell, David T Jones, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Scott A Przybelski, Prashanthi Vemuri, Brian E Gregg, Jeffrey L Gunter, Matthew L Senjem, Ronald C Petersen, Clifford R Jack, Keith A Josephs
We aimed to determine whether network-level functional connectivity differs in 2 clinical variants of Alzheimer's disease: logopenic primary progressive aphasia (lvPPA) and dementia of the Alzheimer's type (DAT). Twenty-four lvPPA subjects with amyloid deposition on positron emission tomography and task-free functional magnetic resonance imaging were matched to 24 amyloid-positive DAT subjects and 24 amyloid-negative controls. Independent-component analysis and spatial-temporal dual regression were used to assess functional connectivity within the language network, left and right working memory networks, and ventral default mode network...
March 2015: Neurobiology of Aging
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