Frontal presentation in progressive supranuclear palsy

Kaat, Laura Donker; Boon, Agnita J.W.; Kamphorst, Wouter; Ravid, Rivka; Duivenvoorden, H.J.; Swieten, John van

doi:10.1212/01.wnl.0000267643.24870.26

Cited by 163 publications

(93 citation statements)

References 23 publications

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“…Two atypical parkinsonian disorders, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), are clinically and pathologically related to FTLD (Kertesz & Munoz, 2003) Both disorders are neurodegenerative diseases that present with behavioral symptoms and frontal-executive difficulties (Belfor et al, 2006;Kaat et al, 2007) and are tauopathies, although the pathological molecule is 4R tau. In addition, patients with PNFA frequently progress to PSP and CBS, even in the absence of early motor findings (Ferrer et al, 2003;Josephs et al, 2006).…”

Section: Etiology Of Ftldmentioning

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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Section: Etiology Of Ftldmentioning

confidence: 99%

The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia

et al. 2008

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“…bvFTD has a broad differential diagnosis of both neurodegenerative and psychiatric disorders, resulting in a similar late-onset behavioral syndrome [4,5,6,7,8,9,10,11,12,13]. Although biomarkers take a prominent position in the present FTDC criteria, it is still unclear how they can distinguish between bvFTD and a representative control group [3].…”

Section: Introductionmentioning

confidence: 99%

Impact of Imaging and Cerebrospinal Fluid Biomarkers on Behavioral Variant Frontotemporal Dementia Diagnosis within a Late-Onset Frontal Lobe Syndrome Cohort

Krudop¹,

Dols

Kerssens

et al. 2015

Dement Geriatr Cogn Disord

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Background: The criteria for behavioral variant frontotemporal dementia (bvFTD) incorporate MRI and [¹⁸F]-FDG-PET. Cerebrospinal fluid (CSF) analysis is merely advised for excluding Alzheimer's disease. Aims: We aimed to assess the impact of biomarkers on diagnostic certainty and contingent changes of bvFTD diagnosis within the clinically relevant neuropsychiatric differential diagnosis of subjects with a late-onset frontal lobe syndrome (LOF). Methods: We included 137 patients with LOF, aged 45-75 years, 72% males. Biomarker disclosure was considered contributing after any substantial difference in diagnostic certainty or a diagnostic change. Percentages of contributing biomarkers were compared between three major diagnostic groups (bvFTD, psychiatry, other neurological disorders). Certainty levels in stable diagnostic groups were compared to those with a diagnostic change. Results: Biomarkers contributed in 53, 60 and 41% of the LOF patients for MRI, [¹⁸F]-FDG-PET and CSF, respectively. Biomarkers changed the diagnosis in 14% of cases towards bvFTD and in 13% from bvFTD into an alternative. Those that changed had a lower level of a priori diagnostic certainty compared to stable diagnoses. Conclusion: Our study not only supports the widely accepted use of MRI and [¹⁸F]-FDG-PET in diagnosing or excluding bvFTD, but also shows that CSF biomarkers aid clinicians in the diagnostic process.

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“…Such collaborative studies, which have been ongoing for more than 15 years, include research studies on Alzheimer's disease (AD) [13,14,15,16,17,18,19,20,21,22,23,24,25,26,27], Parkinson's disease [13,28,29,30,31,32], frontotemporal dementia (FTD) [37,38,39,40,41,42,43,44,45,46,47], multiple sclerosis [37,38,39,40,41,42,43,44,45,46,47], amyotrophic lateral sclerosis, motor neuron disease and psychiatric disorders, including depression and schizophrenia [48,49,50,51]. …”

Section: Results Of Btb-bank Networking and Collaborationsmentioning

confidence: 99%

The Uniqueness of Biobanks for Neurological and Psychiatric Diseases: Potentials and Pitfalls

Ravid¹

2014

Pathobiology

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Objectives: Central nervous system (CNS) biobanks are facing difficult and specific challenges due to the sensitive issue of collecting specimens of the CNS, and especially the brain. At present, there is no global network/central database to serve researchers, clinicians and pharma companies, or to supply the special specimens and the accompanying data in sufficient numbers and detail, respectively. The main challenge/objective is to standardize and harmonize all the facets involved in CNS biobanking in order to maximize efficient sample collection. Methods: Since the number of CNS biospecimens stored in existing biobanks is relatively limited and the accompanying data are not always readily available and hard to identify, we propose using optimal procedures for handling and storage of these specimens, and the global standardization of the cliniconeuropathological diagnostic criteria. Results: One of the prominent achievements of the current global activity in brain tissue biobanks (BTB-banks) is the development of an inventory of international standards, available specimens and concomitant data, and national registries. Conclusions: Taking into consideration the huge variety of the specimens stored in different repositories and the enormous differences in medicolegal systems and ethics regulations in different countries, we strongly recommend that healthcare systems and institutions who host BTB-banks make efforts to secure adequate funding for the infrastructure and daily activities. BTB-banks will refine standard operating procedures and their internal guides of best practices/codes of conduct. This in turn will enable the BTB-banks to share the collected specimens and data with the largest possible number of researchers, aiming at maximal scientific spin-off and advance of public health research.

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Frontal presentation in progressive supranuclear palsy

Abstract: There exists a subgroup of patients with progressive supranuclear palsy (PSP) with a predominant frontal presentation, who progressed into typical PSP over the course of the disease.

Cited by 163 publications

References 23 publications

The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia

The Early Neuropsychological and Behavioral Characteristics of Frontotemporal Dementia

Impact of Imaging and Cerebrospinal Fluid Biomarkers on Behavioral Variant Frontotemporal Dementia Diagnosis within a Late-Onset Frontal Lobe Syndrome Cohort

The Uniqueness of Biobanks for Neurological and Psychiatric Diseases: Potentials and Pitfalls

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