What Is Semantic Dementia? a Cohort Study of Diagnostic Features and Clinical Boundaries
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Primary Progressive Aphasia and Kindred Disorders
Handbook of Clinical Neurology, Vol. 89 (3rd series) Dementias C. Duyckaerts, I. Litvan, Editors # 2008 Elsevier B.V. All rights reserved Primary progressive aphasias Chapter 54 Primary progressive aphasia and kindred disorders MARSEL MESULAM* AND SANDRA WEINTRAUB Cognitive Neurology and Alzheimer’s Disease Center, Northwestern University Feinberg School of Medicine, Chicago, USA 54.1. Introduction Bub, 1997; Kertesz et al., 2000, 2002; Hillis, 2002, 2004; Grossman and Moore, 2005). Terms such as The existence of progressive aphasias has been known progressive nonfluent aphasia (PNFA), semantic for more than 100 years. Pick (1892, 1904), Se´rieux dementia, aphasic variant of frontotemporal dementia (1893), Franceschi (1908), and Rosenfeld (1909) were (FTD), temporal variant of frontotemporal lobar among the first to report such patients. The current resur- degeneration (FTLD), Gogi aphasia and progressive gence of interest in this condition can be traced to a 1982 aphasia have been used, mostly implicitly, to denote report of six patients with a slowly progressive aphasia variants of PPA (Neary et al., 1998; Miller et al., and to the subsequent delineation of the primary progres- 1999). We prefer the PPA term as a root diagnosis sive aphasia (PPA) syndrome (Mesulam, 1982, 1987, for two reasons: not all progressive aphasias fulfill 2007; Mesulam and Weintraub, 1992). the PPA criteria and not all PPA cases are caused According to currently accepted criteria, the PPA by FTLD pathology. The word “primary” is a key diagnosis is made in any patient in whom a language descriptor that emphasizes the selective salience of impairment (aphasia), caused by a neurodegenerative the language disturbance and the relative preservation disease (progressive), constitutes the most salient aspect of other cognitive domains at initial stages. -
An Update on Semantic Dementia: Genetics, Imaging, and Pathology Ramon Landin-Romero1,2,3† , Rachel Tan1,2†, John R
Landin-Romero et al. Alzheimer's Research & Therapy (2016) 8:52 DOI 10.1186/s13195-016-0219-5 REVIEW Open Access An update on semantic dementia: genetics, imaging, and pathology Ramon Landin-Romero1,2,3† , Rachel Tan1,2†, John R. Hodges1,2,3 and Fiona Kumfor1,2,3* Abstract Progressive and relatively circumscribed loss of semantic knowledge, referred to as semantic dementia (SD) which falls under the broader umbrella of frontotemporal dementia, was officially identified as a clinical syndrome less than 50 years ago. Here, we review recent neuroimaging, pathological, and genetic research in SD. From a neuroimaging perspective, SD is characterised by hallmark asymmetrical atrophy of the anterior temporal pole and anterior fusiform gyrus, which is usually left lateralised. Functional magnetic resonance imaging (fMRI) studies have revealed widespread changes in connectivity, implicating the anterior temporal regions in semantic deficits in SD. Task-related fMRI have also demonstrated the relative preservation of frontal and parietal regions alongside preserved memory performance. In addition, recent longitudinal studies have demonstrated that, with disease progression, atrophy encroaches into the contralateral temporal pole and medial prefrontal cortices, which reflects emerging changes in behaviour and social cognition. Notably, unlike other frontotemporal dementia subtypes, recent research has demonstrated strong clinicopathological concordance in SD, with TDP43 type C as the most common pathological subtype. Moreover, an underlying genetic causeappearstoberelativelyrareinSD,withthemajorityof cases having a sporadic form of the disease. The relatively clear diagnosis, clinical course, and pathological homogeneity of SD make this syndrome a promising target for novel disease-modifying interventions. The development of neuroimaging markers of disease progression at the individual level is an important area of research for future studies to address, in order to assist with this endeavour. -
Oxford Handbooks Online
Anomia and Anomic Aphasia Oxford Handbooks Online Anomia and Anomic Aphasia: Implications for Lexical Processing Stacy M. Harnish The Oxford Handbook of Aphasia and Language Disorders (Forthcoming) Edited by Anastasia M. Raymer and Leslie Gonzalez-Rothi Subject: Psychology, Cognitive Neuroscience Online Publication Date: Jan DOI: 10.1093/oxfordhb/9780199772391.013.7 2015 Abstract and Keywords Anomia is a term that describes the inability to retrieve a desired word, and is the most common deficit present across different aphasia syndromes. Anomic aphasia is a specific aphasia syndrome characterized by a primary deficit of word retrieval with relatively spared performance in other language domains, such as auditory comprehension and sentence production. Damage to a number of cognitive and motor systems can produce errors in word retrieval tasks, only subsets of which are language deficits. In the cognitive and neuropsychological underpinnings section, we discuss the major processing steps that occur in lexical retrieval and outline how deficits at each of the stages may produce anomia. The neuroanatomical correlates section will include a review of lesion and neuroimaging studies of language processing to examine anomia and anomia recovery in the acute and chronic stages. The assessment section will highlight how discrepancies in performance between tasks contrasting output modes and input modalities may provide insight into the locus of impairment in anomia. Finally, the treatment section will outline some of the rehabilitation techniques for forms of anomia, and take a closer look at the evidence base for different aspects of treatment. Keywords: Anomia, Anomic aphasia, Word retrieval, Lexical processing Syndrome Description and Unique Characteristics The term anomia refers to the inability to retrieve a desired word, typically in the course of conversational sentence production. -
Semantic Dementia
cortex xxx (2012) 1e10 Available online at www.sciencedirect.com Journal homepage: www.elsevier.com/locate/cortex Research report Bringing words back to mind e Improving word production in semantic dementia Sharon A. Savage a,b, Kirrie J. Ballard c, Olivier Piguet a,b and John R. Hodges a,b,* a Neuroscience Research Australia, Sydney, Australia b Faculty of Medicine, School of Medical Sciences, University of New South Wales, Sydney, Australia c Faculty of Health Sciences, Speech Pathology, University of Sydney, Australia article info abstract Article history: Patients with semantic dementia (SD) have significant impairments in naming and Received 28 February 2012 comprehension, but demonstrate relatively intact attention, everyday memory, and Reviewed 21 June 2012 visuospatial skills. Given these preserved skills, attempts have been made to help re-build Revised 25 June 2012 vocabulary in SD patients, with promising results. Such reports, however, are generally Accepted 19 September 2012 based upon only one or two cases and have employed variable retraining methods. It is Action editor Mike Kopelman thus unclear which elements of practice are crucial to success. Over two studies, we Published online xxx assessed four patients undergoing a word training program, who ranged in severity from mild to severe impairments to semantic knowledge. All four participants showed Keywords: significant improvements in their ability to name trained items, with no changes in Semantic dementia untrained items over the same time period. Improvements were evident within 3 weeks of Cognitive training practice, and could be established from a simple, repetitive practice of word-picture Naming pairing, carried out at the participant’s home. -
Very Early Semantic Dementia with Progressive Temporal Lobe Atrophy an 8-Year Longitudinal Study
OBSERVATION Very Early Semantic Dementia With Progressive Temporal Lobe Atrophy An 8-Year Longitudinal Study Kathrin Czarnecki, MD; Joseph R. Duffy, PhD; Carissa R. Nehl, PhD; Shelley A. Cross, MD; Jennifer R. Molano, MD; Clifford R. Jack Jr, MD; Maria M. Shiung, BA; Keith A. Josephs, MD, MST; Bradley F. Boeve, MD Background: Semantic dementia is a syndrome within mal but subtle left anterior temporal lobe atrophy was the spectrum of frontotemporal lobar degenerations char- present. During the follow-up period of 8 years, she de- acterized by fluent progressive aphasia (particularly ano- veloped profound anomia and loss of word meaning as- mia) and loss of word meaning. sociated with progressive left anterior temporal lobe at- rophy, consistent with semantic dementia. In more recent Objective: To report a unique case of very early seman- years, anterograde memory impairment and mild prosop- tic dementia with a slowly progressive course, allowing agnosia evolved in association with left hippocampal at- insights into the early natural history of this disorder. rophy and subtle atrophy in the homologous gyri of the right anterior temporal lobe. She remains functionally in- Design: Case report. dependent despite her current deficits. Setting: A tertiary care center. Conclusions: Early identification of patients who will develop semantic dementia is difficult and might be missed Patient: A 62-year-old woman who presented with with standard clinical, neuropsychological, and struc- “memory loss” complaints. tural neuroimaging evaluations. Recognition of this rela- Main Outcome Measures: Clinical course, neuropsy- tively rare syndrome is important for early diagnosis and chological data, and magnetic resonance imaging results. -
An Overview on Primary Progressive Aphasia and Its Variants
1 TITLE An Overview on Primary Progressive Aphasia and its variants Serena Amici a,d *M.D., Maria Luisa Gorno -Tempini a, M.D., PhD, Jennifer M. Ogar a,b , M.S., Nina F. Dronkers a,b,c , PhD, Bruce L. Miller a, M.D. aMemory and Aging Center, Department of Ne urology , University of California, San Francisco, USA bVA Northern California Health Care System, Martinez, CA, USA cUniversity of California, Davis, Davis, CA, USA dDepartment of Neurosciences, University of Perugia, Perugia, ITALY *Corresponding author: Serena Amici 350 Parnassus Avenue, San Francisco, CA 94143 -1207 (415) 476 3572 Keywords : primary progressive aphasia, nonfluent progressive aphasia, semantic dementia, logopenic progressive aphasia, neuroimaging studies, language symptoms Words: 415 6 Tables : 2 Figures: 1 Reference s: 64 2 Abstract We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profile s of 42 PPA patients. Mesulam originally defined PPA as a progressive degenerative disorder characterized by isolated language impairment for at least two years. The most common variants of PPA are: 1) Progressive nonfluent aphasia (PNFA), 2) semantic dementia (SD), 3) logopenic progressive aphasia (LPA). PNFA is characterized by labored speech, agrammatism in production, and/or comprehension . In some cases the syndrome begins with isolated deficits in speech. SD patients typically present with loss of word and object meaning and surface dyslexia. LPA patients have word -finding difficulties, syntactically simple but accurate language output and impaired sentence comprehension. The neuropsychological data demonstrated that SD patients show the most characteristic pattern of impairment, while PNFA and LPA overlap with in many cognitive domains. -
Dewinter Cortex 2016.Pdf
cortex 82 (2016) 179e191 Available online at www.sciencedirect.com ScienceDirect Journal homepage: www.elsevier.com/locate/cortex Research report Amygdala atrophy affects emotion-related activity in face-responsive regions in frontotemporal degeneration Franc¸ois-Laurent De Winter a,b,1, Jan Van den Stock a,b,1, Beatrice de Gelder a,c, Ronald Peeters d,e, Jan Jastorff a, Stefan Sunaert d,e, * Wim Vanduffel f,g,h, Rik Vandenberghe i,j and Mathieu Vandenbulcke a,b, a Laboratory for Translational Neuropsychiatry, Research Group Psychiatry, Department of Neurosciences, KU Leuven, Leuven, Belgium b Department of Old Age Psychiatry, University Hospitals Leuven, Leuven, Belgium c Department of Cognitive Neuroscience, Maastricht University, Maastricht, The Netherlands d Department of Radiology, University Hospitals Leuven, Leuven, Belgium e Department of Imaging and Pathology, KU Leuven, Leuven, Belgium f Laboratory for Neuro- and Psychophysiology, Department of Neurosciences, KU Leuven, Leuven, Belgium g Athinoula A. Martinos Center for Biomedical Imaging, Massachusetts General Hospital, Charlestown, MA, USA h Department of Radiology, Harvard Medical School, Boston, MA, USA i Laboratory for Cognitive Neurology, Department of Neurosciences, KU Leuven, Leuven, Belgium j Department of Neurology, University Hospitals Leuven, Leuven, Belgium article info abstract Article history: In the healthy brain, modulatory influences from the amygdala commonly explain Received 3 February 2016 enhanced activation in face-responsive areas by emotional facial expressions -
Frontotemporal Dementia
About dementia 17 Frontotemporal dementia This help sheet describes a type of dementia known as frontotemporal dementia, which has different forms including behavioural-variant frontotemporal dementia, progressive non-fluent aphasia and semantic dementia. What is frontotemporal dementia? Frontotemporal dementia (FTD) is the name given to dementia when it is due to progressive damage to the frontal and/or temporal lobes of the brain. The right and left frontal lobes at the front of the brain are involved in mood, social behaviour, attention, judgement, planning and self-control. Damage can lead to reduced intellectual abilities and changes in personality, emotion and behaviour. The right and left temporal lobes at the two sides of the brain are involved in processing what we hear and understanding what we hear and see. Damage may lead to difficulty recognising objects or understanding or expressing language. FTD is sometimes called frontotemporal lobar degeneration (FTLD). It was first described 100 years ago by Arnold Pick and was previously referred to as Pick’s disease. The symptoms of FTD depend on which areas of the brain are damaged. In contrast to Alzheimer’s disease, memory often remains unaffected in FTD, especially in the early stages. When the frontal lobes are affected first, the main changes are in personality and behaviour, and this is called behavioural-variant FTD. When the temporal lobes are affected first, there is a loss of language skills. There are two types of FTD where language is impaired – progressive non-fluent aphasia and semantic dementia. For language assistance National Dementia Helpline 1800 100 500 call 131 450 About dementia 17 Behavioural-variant frontotemporal dementia In the frontal or behavioural variant of FTD, there are changes in the person’s behaviour, habits, personality and/or emotional responses. -
What Is a Rare Dementia? About Primary Progressive Aphasia (PPA)
What is a Rare Dementia? Between 5% and 15% of people living with a dementia receive a diagnosis of a rare or young-onset dementia. The diagnosis of a rare dementia brings with it a set of unique and complex challenges which can include difficulties with vision, language, movement and behavioural changes. 30% of people living with a rare dementia first receive an incorrect diagnosis and there is a widespread lack of understanding about, and resources for people living with, rare dementias. By increasing awareness about these dementias we hope to empower the healthcare sector and communities to better support those affected by a rare dementia diagnosis. This document will provide information about 6 of the rare dementias. About Primary Progressive Aphasia (PPA) The term Primary Progressive Aphasia (PPA) refers to a group of dementias, predominantly causing a loss of speech and language abilities. ‘Aphasia’ refers to a neurological language problem, ‘progressive’ being worse over time, and ‘primary’ as this condition is caused by brain tissue changes rather than an external cause. Within the PPA group, three main patterns of language loss are recognised: Semantic Dementia (SD), Progressive Nonfluent Aphasia (PNFA) and Logopenic Aphasia (LPA). PNFA and SD fall within a larger group of brain disorders collectively called Frontotemporal Dementia (FTD – see below). LPA, by contrast, is an unusual form of Alzheimer’s Disease and is usually caused by the same proteins that cause Alzheimer’s disease. Semantic Dementia (SD) The first symptoms of -
Rehabilitation in Semantic Dementia Study of the Effectiveness of Lexical Reacquisition in Three Patients
Dement Neuropsychol 2010 December;4(4):306-312 Original Article Rehabilitation in semantic dementia Study of the effectiveness of lexical reacquisition in three patients Mirna Lie Hosogi Senaha1, Sonia Maria Dozzi Brucki2, Ricardo Nitrini3 Abstract – Although language rehabilitation in patients with primary progressive aphasia (PPA) is recommended, rehabilitation studies in this clinical syndrome are scarce. Specifically, in relation to semantic dementia (SD), few studies have shown the possibility of lexical relearning. Objective: To analyze the effectiveness of rehabilitation for lexical reacquisition in SD. Methods: Three SD patients were submitted to training for lexical reacquisition based on principles of errorless learning. Comparisons between naming performance of treated items (pre and post-training) and non-treated items of the Boston Naming Test (BNT) were made. Results: All patients improved their performance in naming treated words after intervention. However, decline in performance in naming of non-treated items was observed. Case 1 named zero items at baseline while her performance post-training was 29.4% correct responses without cueing, and 90.7% correct with and without cueing. Case 2 named 6.9% of items correctly at baseline and his performance in post-training was 52.9% without cueing and 87.3%, with and without cueing. Case 3 named zero items at baseline and his performance in post-training was 100% correct responses without cueing. Considering the performance in naming the non-treated items of the BNT, the percentages of correct responses in the first evaluation and in the re-evaluation, respectively were: 16.7% and 8.3% (case 1; 14 month-interval); 26.7% and 11.6% (case 2; 18 month-interval) and 11.6% and 8.3% (case 3; 6 month-interval). -
Visual Agnosia I
Visual Agnosia I. Biran, MD, and H. B. Coslett, MD Address Clinical Variants Department of Neurology, University of Pennsylvania School of Medi- Visual agnosia can be specific to certain kinds of objects. cine, 3400 Spruce Street, Philadelphia, PA 19104, USA. Accordingly, there are agnosias for objects, agnosias for E-mail: [email protected] faces or “prosopagnosia,” agnosias for words or “pure Current Neurology and Neuroscience Reports 2003, 3:508–512 word blindness,” agnosias for colors, and agnosias for Current Science Inc. ISSN 1528–4042 Copyright © 2003 by Current Science Inc. the environment, including landmarks. Finally, the dis- order of simultanagnosia—an inability to “see” more than one object at a time—is often regarded as a type of The visual agnosias are an intriguing class of clinical phe- visual agnosia. nomena that have important implications for current theo- ries of high-level vision. Visual agnosia is defined as impaired Agnosia for objects object recognition that cannot be attributed to visual loss, Inability to recognize familiar objects is the most com- language impairment, or a general mental decline. At least mon of the agnostic syndromes. Patients are impaired in in some instances, agnostic patients generate an adequate naming regular objects and are often unable to describe internal representation of the stimulus but fail to recognize them or mimic their use. Object agnosias are further it. In this review, we begin by describing the classic works classified as either apperceptive (the percept is not fully related to the visual agnosias, followed by a description of constructed and, therefore, patients are unable to copy the major clinical variants and their occurrence in degener- drawings) or associative (the percept is relatively intact ative disorders. -
Frontotemporal Dementia (FTD) Compare and Contrast with Alzheimer Disease
Frontotemporal Dementia (FTD) Compare and contrast with Alzheimer disease • Most common cause of dementia by far ‐70% • More common with advancing age ≥ 65 YO 7 % ≥ 85 YO 30‐47 % • Insidious onset, slowly progressive course • Earliest manifestation usually STM (short term memory loss) • Other early cognitive deficits – Executive function (abstract thinking, planning, organizing) – Language ( forget words, verbal expression, comprehension of reading) • Middle‐to‐late stage manifestations – Gait instability / falls – Incontinence – BPSD ( Behavioral and Psychological Symptoms of Dementia ) – Personality changes – At best, modest / temporary improvement with CEI ( cholinesterase inhibitors ) FTD • Pathologically / clinically heterogeneous disorder with focal degeneration of frontal and/or temporal lobes • Onset typically late 50’s‐ early 60’s; mean age 58 – Onset 20‐80; unusual 40 or 75 • Earliest manifestations – Personality changes / social behavior changes (behavior variant) – Language deficits • Slowly progressive to more global dementia • Some with extrapyramidal or motor symptoms • 1/3 with FH • Pick disease behavioral variant with Pick bodies ( intracellular inclusions) • Other terms – Frontal lobe dementia – Frontal lobe degeneration – Frontotemporal lobar degeneration – Pick complex FTD Subtypes • Behavioral variant ( BV ) • Progressive Nonfluent Aphasia ( PNFA ) • Semantic Dementia ( SD ) progressive fluent aphasia • Motor Syndromes ‐ Motor Neuron Disease ( MND ) ‐ Corticobasilar Degeneration ( CBD ) ‐ Progressive Supranuclear