Executive Dysfunction Is the Primary Cognitive Impairment in Progressive Supranuclear Palsy

Gerstenecker, Adam; Mast, Benjamin T.; Duff, Kevin; Ferman, Tanis J.; Litvan, Irene

doi:10.1093/arclin/acs098

Cited by 85 publications

(51 citation statements)

References 34 publications

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“…Finally, the third cluster in PSP covered a region of left Crus I/II and lobule VIIb that has been implicated in the ECN, which fits with executive dysfunction being the most common cognitive symptom in the disease 62. Based on these findings, the cerebellum may be involved in motor symptoms of PSP and in cognitive-affective changes.…”

Section: Discussionmentioning

confidence: 63%

Cerebellar atrophy in neurodegeneration—a meta-analysis

Gellersen

Guo

O’Callaghan

et al. 2017

J Neurol Neurosurg Psychiatry

127

100

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Section: Discussionmentioning

confidence: 63%

Cerebellar atrophy in neurodegeneration—a meta-analysis

Gellersen

Guo

O’Callaghan

et al. 2017

J Neurol Neurosurg Psychiatry

127

100

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“…Paviour et al [7] showed also that lexical fluency and motor series subscores from FAB correctly classified 70% of the PSP, MSA and PD patients. In Gerstenecker et al's study [4] lexical fluency mean scores were also the lowest subscores obtained by patients with PSP. Language contribution to FAB score and its discriminant validity seems prominent.…”

Section: Discussionmentioning

confidence: 81%

“…Neuropsychological profile of PSP is supposed to be dominated by executive dysfunction: difficulties with initiation, planning multi-stage activities, impulsivity and perseveration [4]. However, a subset of PSP patients develop speech and language deficits that may be as severe as corresponding to non-fluent progressive aphasia and/or progressive apraxia of speech (PSP-PNFA), which is in line with PSP as a part of Pick Complex spectrum [3], but may also be only an additional feature.…”

Section: Introductionmentioning

confidence: 99%

Utility of Frontal Assessment Battery in detection of neuropsychological dysfunction in Richardson variant of progressive supranuclear palsy

Sitek

Konkel

Dąbrowska

et al. 2015

Neurologia i Neurochirurgia Polska

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Progressive supranuclear palsy is characterized by motor, cognitive and behavioral features. In Richardson's syndrome of PSP (PSP-RS) executive dysfunction is quite prominent. Frontal Assessment Battery (FAB) is one of the most popular screening tests in the differential diagnosis of bradykinetic rigid syndromes. The study aimed at analyzing FAB subscores in relation to neuropsychological assessment results. Twenty patients with PSP-RS (12 with probable and eight with possible diagnosis) participated in the study. Sixteen PSP-RS patients scored below 15 on FAB. Among four patients having scored above cut-off (12 points) on FAB, two demonstrated both executive and language deficits, while the other two presented with only selective executive deficits on comprehensive neuropsychological evaluation. FAB is a useful screening measure in PSP, but it may not detect subtle executive deficits. Moreover, language performance seems to contribute significantly to FAB scores. Thus, FAB should be treated as "frontal" rather than "executive" screening task, in line with its name.

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“…Executive function is often the only reported neuropsychological deficit in the typical PSP (Richardson's) syndrome, [17-19] and individuals with worse color trails scores, a measure of executive function, declined at a faster rate. Surprisingly, the broader RBANS neuropsychological battery which includes tests of memory, attention, language and visuospatial function was also sensitive to change over one year, and individuals with more global cognitive impairments on RBANS at baseline had higher rates of annual PSPRS change.…”

Section: Discussionmentioning

confidence: 99%

Predicting disease progression in progressive supranuclear palsy in multicenter clinical trials

Investigators

2016

Parkinsonism & Related Disorders

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Introduction Clinical and MRI measurements can track disease progression in PSP, but many have not been extensively evaluated in multicenter clinical trials. We identified optimal measures to capture clinical decline and predict disease progression in multicenter PSP trials. Methods Longitudinal clinical rating scales, neuropsychological test scores, and volumetric MRI data from an international, phase 2/3 clinical trial of davunetide for PSP (intent to treat population, n=303) were used to identify measurements with largest effect size, strongest correlation with clinical change, and best ability to predict dropout or clinical decline over one year as measured by PSP Rating Scale (PSPRS). Results Baseline cognition as measured by Repeatable Battery for Assessing Neuropsychological Status (RBANS) was associated with attrition, but had only a small effect. PSPRS and Clinical Global Impression (CGI) had the largest effect size for measuring change. Annual change in CGI, RBANS, color trails, and MRI midbrain and ventricular volumes were most strongly correlated with annual PSPRS and had the largest effect sizes for detecting annual change. At baseline, shorter disease duration, more severe depression, and lower performance on RBANS and executive function tests were associated with faster worsening of the PSPRS in completers. With dropouts included, SEADL, RBANS, and executive function tests had significant effect on PSPRS trajectory of change. Conclusion Baseline cognitive status and mood influence the rate of disease progression in PSP. Multiple clinical, neuropsychological, and volumetric MRI measurements are sensitive to change over one year in PSP and appropriate for use in multicenter clinical trials.

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Executive Dysfunction Is the Primary Cognitive Impairment in Progressive Supranuclear Palsy

Cited by 85 publications

References 34 publications

Cerebellar atrophy in neurodegeneration—a meta-analysis

Cerebellar atrophy in neurodegeneration—a meta-analysis

Utility of Frontal Assessment Battery in detection of neuropsychological dysfunction in Richardson variant of progressive supranuclear palsy

Predicting disease progression in progressive supranuclear palsy in multicenter clinical trials

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