Differences Between Pick Disease and Alzheimer Disease in Clinical Appearance and Rate of Cognitive Decline

Binetti, Giuliano; Locascio, Joseph J.; Corkin, Suzanne; Vonsattel, Jean Paul; Growdon, John H.

doi:10.1001/archneur.57.2.225

Cited by 84 publications

(64 citation statements)

References 46 publications

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“…Patients with FTD declined an average of 30 points on the MDRS over 1 year, double the average 15-point drop for AD patients. This observed disparity in cognitive decline parallels recent findings using clinically diagnosed (Binetti et al, 2000) and autopsy-confirmed (Rascovsky et al, 2005) patient samples. Indeed, a recent multicenter study of autopsy-confirmed patients (Rascovsky et al, 2005) found faster rates of cognitive decline in FTD than AD when cognition was assessed with the Mini-Mental State Examination (MMSE).…”

Section: Cognitive Dysfunction In Ftd 379supporting

confidence: 86%

“…It is estimated that approximately 3-20% of all cases of dementia may be FTD (Barker et al, 2002;Gislason et al, 2003;Knopman et al, 1990;Neary, 1999;Ratnavalli et al, 2002;Rosso et al, 2003), and the disorder is particularly prevalent when the age of onset of dementia is younger than 65 years (Ratnavalli et al, 2002;Rosso et al, 2003). Recent studies suggest that FTD follows a particularly malignant course (Grasbeck et al, 2003;Hodges et al, 2003), but information as to the nature and rate of cognitive decline is still scarce (Binetti et al, 2000;Pasquier et al, 2004;Rascovsky et al, 2005).…”

Section: Introductionmentioning

confidence: 99%

“…Accurate differential diagnosis is crucial, however, given potential differences in prognosis (Rascovsky et al, 2005) and in pharmacological management strategies that might be suggested by different neurotransmitter system involvement in the two disorders Moretti et al, 2003;Pasquier et al, 2003;Qume et al, 1994;Rahman et al, 1999;Sparks & Markesbery, 1991;Swartz et al, 1997). Recent studies using batteries of neuropsychological tests suggest that FTD and AD are associated with distinct cognitive profiles that might aid in their differential diagnosis (Binetti et al, 2000;Elfgren et al, 1994;Frisoni et al, 1995;Gregory et al, 1997;Kramer et al, 2003;Lindau et al, 1998;Mendez et al, 1996;Pachana et al, 1996;Rascovsky et al, 2002;Thomas-Anterion et al, 2000). Particularly compelling are retrospective studies that have shown a double dissociation in which mildly-to-moderately demented patients with autopsy-confirmed FTD are more impaired than those with autopsy-confirmed AD on tests sensitive to frontal lobe dysfunction (e.g., word generation tasks), but less impaired on tests of memory and visuospatial abilities sensitive to dysfunction of medial temporal and parietal association cortices (e.g., Rascovsky et al, 2002).…”

Section: Introductionmentioning

confidence: 99%

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Distinct cognitive profiles and rates of decline on the Mattis Dementia Rating Scale in autopsy-confirmed frontotemporal dementia and Alzheimer's disease

Rascovsky

Salmon

Hansen

et al. 2008

J Inter Neuropsych Soc

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Neuropsychological studies have shown that patients with Frontotemporal dementia (FTD) perform worse than patients with Alzheimer's disease (AD) on tests of conceptualization and verbal fluency, but better on tests of memory and visuospatial functions. However, it is not known if these distinct cognitive profiles are robust enough to be detected using a relatively brief dementia screening instrument such as the Mattis Dementia Rating Scale (MDRS). To address this issue, the MDRS subscale profiles of patients with autopsy-confirmed FTD (n 5 17) or AD (n 5 34) were compared. Results showed distinct cognitive profiles in which FTD patients performed worse than AD patients on the Initiation0Perseveration and Conceptualization subscales while performing better on the Memory and Construction subscales. The distinct subscale profiles correctly classified 85% of AD patients and 76% of FTD patients. Profiles were maintained in a subset of mildly-to-moderately demented patients (MDRS Ն 105) and correctly classified 89% of these patients. In addition, FTD patients (mean 5 30.0 points0year) declined faster than AD patients (mean 5 14.8 points0year) on MDRS total and specific subscale scores. These results suggest that the MDRS may be a useful adjunct to other clinical measures for distinguishing FTD from AD and tracking the progression of the disorder. (JINS, 2008, 14, 373-383.)

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Section: Cognitive Dysfunction In Ftd 379supporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Distinct cognitive profiles and rates of decline on the Mattis Dementia Rating Scale in autopsy-confirmed frontotemporal dementia and Alzheimer's disease

Rascovsky

Salmon

Hansen

et al. 2008

J Inter Neuropsych Soc

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“…The most common behavioral changes include apathy, disinhibition, and inappropriate social conduct. Some studies have shown that the caretaker report of behavioral change is often the best way to differentiate between FTD and AD (Binetti, Locascio, Corkin, Vonsattel, & Growdon, 2000;Gregory et al, 1997). The hallmarked behavioral and functional changes are identified by the caretakers and quantified through their responses on measures like the NPI, Interpersonal Reactivity Index (IRI), Interpersonal Adjectives Scales, and Clinical Dementia Rating Scale (CDR).…”

Section: Differentiation Of Ftd From Admentioning

confidence: 99%

The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia

et al. 2008

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Frontotemporal lobe dementias (FTLD) represent a constellation of disorders that may be overlooked or misdiagnosed, despite being a fairly common presenile neurodegenerative diseases. Although the cognitive disorder can be difficult to document, particularly early in the dementia course, neuropsychological evaluation can assist in the diagnosis. Neuropsychologists are in an excellent position to draw from related disciplines like personality theory and social psychology to better assess the types of changes that characterize the prodromal and early phases of the disease. This review summarizes the current state of the field in the diagnosis of FTLD and discusses the emerging role of neuropsychology in elucidating the brain organization of complex processes including empathy, behavioral control and inhibition, reward systems, appetitive behaviors, emotional regulation, and goal-orientation. As this review underscores, FTD remains a powerful model for studying brain-behavior relationships.Frontotemporal lobar degeneration (FTLD) refers to a set of neurodegenerative disorders arising from relatively circumscribed frontal and temporal lobar atrophy. Characterized by troubling and progressive changes in behavior, personality, judgment, language, and cognition, the disorder compromises individuals while still in their productive years and may initially be mistaken as a psychiatric disorder. As with other degenerative dementias, a goal is to identify these disorders early with the intent of intervening before the disease has fully expressed in brain. Although no pharmacological treatments are yet available to prevent the disease progression, there are treatments that help alleviate symptoms and nonpharmacological interventions to deal with problem behaviors, coping, and adjustment. From a cognitive neuroscience perspective, the FTLD dementias with their effects on some of the core features of human personality and cognition offer an opportunity to better understand how these fundamental processes of human existence are functionally organized, both the processes involved as well as the brain systems that mediate them. This review article discusses the clinical presentation of the FTLD dementias with an emphasis on its early expression. We propose that by applying a theoretical framework of human behavior from social psychology and constructs from personality theory to the study of FTLD, neuropsychology is ideally positioned to elucidate some of the brain mechanisms underlying a range of complex human behaviors, emotions, and social cognition. Clinical and Neuropathological Expression of FTLDThe first mention of FTLD as a syndrome was in 1892 when Arnold Pick, a neurologist at the University of Prague, described the clinical and pathological features of one of his patients. He reported an aphasic presentation that progressed over three years in a 71-yearold individual. Upon autopsy, there was found to be significant frontal and temporal lobe atrophy in the diseased brain. Pick's clinical description of his patien...

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“…symptoms and early changes in behaviour. Some evidence suggests that the symptoms Some evidence suggests that the symptoms at presentation differentiate FTD from Alzat presentation differentiate FTD from Alzheimer's disease (Binetti heimer's disease (Binetti et al et al, 2000;Lindau , 2000;Lindau et al et al, 2000). Investigations are also helpful , 2000).…”

Section: Clinical Features Of Ftd Clinical Features Of Ftdmentioning

confidence: 99%

Genetics, molecular biology, neuropathology and phenotype of frontal lobe dementia

et al. 2002

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BackgroundMutations in tau have been found in a group of related disorders including the frontal lobe dementias.AimsTo describe the clinical features and molecular pathology changes in a single case of a patient with frontal lobe dementia.MethodA case report was compiled from neuropathological reports and genomic and gene expression analyses.ResultsA case with a splice-site mutation resulting in a typical frontotemporal clinical and neuropathological phenotype was found. Gene expression analysis suggests differential expression of isoforms of tau in regions in the brain.ConclusionsFrontotemporal dementia can result from gene mutations that alter splicing and expression of tau.

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Differences Between Pick Disease and Alzheimer Disease in Clinical Appearance and Rate of Cognitive Decline

Abstract: There is a characteristic cognitive profile and course of dementia in PcD. Nonetheless, cognitive test performance does not clearly distinguish PcD from AD.

Cited by 84 publications

References 46 publications

Distinct cognitive profiles and rates of decline on the Mattis Dementia Rating Scale in autopsy-confirmed frontotemporal dementia and Alzheimer's disease

Distinct cognitive profiles and rates of decline on the Mattis Dementia Rating Scale in autopsy-confirmed frontotemporal dementia and Alzheimer's disease

The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia

Genetics, molecular biology, neuropathology and phenotype of frontal lobe dementia

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