Cognitive deficits in progressive supranuclear palsy on the Repeatable Battery for the Assessment of Neuropsychological Status

Duff, Kevin; McDermott, Dana; Luong, Dan; Randolph, Christopher; Boxer, Adam L.

doi:10.1080/13803395.2019.1572073

Cited by 12 publications

(7 citation statements)

References 35 publications

(34 reference statements)

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“…No baseline differences were found between groups on other clinical measures of severity, including SEADL, CGI-S, and CDR-SB. Baseline cognitive impairment was assessed with the RBANS, a brief tool validated in patients with PSP-RS with a normative index score of 100 (SD, 15), 11 and scores were comparable at baseline. Additionally, no differences were noted on MMSE or GDS, a measure of depressive symptoms.…”

Section: Resultsmentioning

confidence: 99%

“…Currently, a variety of experimental therapeutic approaches to PSP have been proposed, mainly focused on tau protein as a target, but relatively few late-phase clinical trials have been conducted. 1,[4][5][6][7][8] Large, international clinical trials and multicenter, longitudinal, observation studies have revealed striking consistency in rates of change of clinical rating scales of PSP symptomatology, 9 particularly on the Progressive Supranuclear Palsy Rating Scale (PSPRS) and Schwab and England Activity of Daily Living scale (SEADL), 10 the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), 11 volumetric MRI measures of midbrain atrophy, 12 and cerebrospinal fluid (CSF) neurofilament light chain (NfL) concentrations. 13 This suggests that natural history data from earlier longitudinal studies could be used to gauge the potential promise of novel therapeutic interventions, before the initiation of larger, placebo-controlled trials or in situations where such trials are not practical or possible, including commonly used therapeutics, lifestyle interventions that are widely available, or other desired off-label interventions by patients and families.…”

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confidence: 99%

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Open‐Label Phase 1 Futility Studies of Salsalate and Young Plasma in Progressive Supranuclear Palsy

VandeVrede

Dale

Fields

et al. 2020

Movement Disord Clin Pract

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Background Progressive supranuclear palsy (PSP) is a neurodegenerative disease without approved therapies, and therapeutics are often tried off‐label in the hope of slowing disease progression. Results from these experiences are seldom shared, which limits evidence‐based knowledge to guide future treatment decisions. Objectives To describe an open‐label experience, including safety/tolerability, and longitudinal changes in biomarkers of disease progression in PSP‐Richardson's syndrome (PSP‐RS) patients treated with either salsalate or young plasma and compare to natural history data from previous multicenter studies. Methods For 6 months, 10 PSP‐RS patients received daily salsalate 2,250 mg, and 5 patients received monthly infusions of four units of young plasma. Every 3 months, clinical severity was assessed with the Progressive Supranuclear Palsy Rating Scale (PSPRS), and MRI was obtained for volumetric measurement of midbrain. A range of exploratory biomarkers, including cerebrospinal fluid levels of neurofilament light chain, were collected at baseline and 6 months. Interventional data were compared to historical PSP‐RS patients from the davunetide clinical trial and the 4‐Repeat Tauopathy Neuroimaging Initiative. Results Salsalate and young plasma were safe and well tolerated. PSPRS change from baseline (mean ± standard deviation [SD]) was similar in salsalate (+5.6 ± 9.6), young plasma (+5.0 ± 7.1), and historical controls (+5.6 ± 7.1), and change in midbrain volume (cm3 ± SD) did not differ between salsalate (–0.07 ± 0.03), young plasma (–0.06 ± 0.03), and historical controls (–0.06 ± 0.04). No differences were observed between groups on any exploratory endpoint. Conclusions Neither salsalate nor young plasma had a detectable effect on disease progression in PSP‐RS. Focused open‐label clinical trials incorporating historical clinical, neuropsychological, fluid, and imaging biomarkers provide useful preliminary data about the promise of novel PSP‐directed therapies.

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Section: Resultsmentioning

confidence: 99%

mentioning

confidence: 99%

Open‐Label Phase 1 Futility Studies of Salsalate and Young Plasma in Progressive Supranuclear Palsy

VandeVrede

Dale

Fields

et al. 2020

Movement Disord Clin Pract

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“…Selection of clinically relevant outcomes has been facilitated by several multicenter clinical and observational trials showing striking consistency in several clinical endpoints [194], including the PSP Rating Scale [195], the Schwab and England Activity of Daily Living scale (SEADL) [196], t h e R e p e a t a b l e B a t t e r y f o r t h e A s s e s s m e n t o f Neuropsychological Status (RBANS) [197], and neurofilament light chain (NfL) concentrations in the blood and cerebrospinal fluid [198]. And although the current tau positron emission tomography (PET) radiotracers do not appear sensitive for 4R-tau deposits [199], several more recent and promising efforts may yet yield a 4R-tau imaging biomarker that could replace the current use of volumetric MRI measures of midbrain atrophy to detect PSP-RS.…”

Section: Discussionmentioning

confidence: 99%

Four-Repeat Tauopathies: Current Management and Future Treatments

et al. 2020

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Four-repeat tauopathies are a neurodegenerative disease characterized by brain parenchymal accumulation of a specific isoform of the protein tau, which gives rise to a wide breadth of clinical syndromes encompassing diverse symptomatology, with the most common syndromes being progressive supranuclear palsy-Richardson's and corticobasal syndrome. Despite the lack of effective disease-modifying therapies, targeted treatment of symptoms can improve quality of life for patients with 4-repeat tauopathies. However, managing these symptoms can be a daunting task, even for those familiar with the diseases, as they span motor, sensory, cognitive, affective, autonomic, and behavioral domains. This review describes current approaches to symptomatic management of common clinical symptoms in 4-repeat tauopathies with a focus on practical patient management, including pharmacologic and nonpharmacologic strategies, and concludes with a discussion of the history and future of disease-modifying therapeutics and clinical trials in this population. Keywords 4R-tauopathy (4R-tau) . progressive supranuclear palsy (PSP) . Richardson's syndrome (PSP-RS) . corticobasal syndrome (CBS) . corticobasal degeneration (CBD) . atypical parkinsonism

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“…For example, this battery is not only proven to have a sufficiently high accuracy diagnosis-wise [ 12 ], but it is also useful for detecting HIV-associated neurocognitive disorders [ 13 , 14 ]. The RBANS’ areas of application also extend to other causes of cognitive impairment, such as alcohol dependence [ 15 ], post-traumatic stress disorder [ 16 ], and progressive supranuclear palsy [ 17 ].…”

Section: Introductionmentioning

confidence: 99%

Mini-Mental State Examination: Optimal Cut-Off Levels for Mild and Severe Cognitive Impairment

2023

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Considering the need to intercept neurocognitive damage as soon as possible, it would be useful to extend cognitive test screening throughout the population. Here, we propose differential cut-off levels that can be used to identify mild and severe cognitive impairment with a simple and widely used first-level neurocognitive screening test: the Mini-Mental State Examination (MMSE). We studied a population of 262 patients referred for cognitive impairment testing using the MMSE and Repeatable Battery for the Assessment of Neuropsychological Status (RBANS), a neuropsychological battery. The sample consisted of 262 participants with mean age 73.8 years (60–87), of whom 154 (58.8%) women. No significant gender-related differences in cognitive ability were identified. The two tests (MMSE and RBANS) showed a moderate correlation in identifying cognitive deficit. We used RBANS as a categorial variable to identify different degrees of cognitive impairment. Youden’s J indexes were used to consider the better sensitivity/specificity balance in the 24-point cut-off score for severe cognitive deficit, 29.7-point score for mild cognitive deficit, and 26.1-point score for both mild and severe cognitive deficit. The study shows that the MMSE does not identify early cognitive impairment. Though different cut-offs are needed to discriminate different impairment degrees, the 26.1-point score seems to be preferable to the others.

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Cognitive deficits in progressive supranuclear palsy on the Repeatable Battery for the Assessment of Neuropsychological Status

Cited by 12 publications

References 35 publications

Open‐Label Phase 1 Futility Studies of Salsalate and Young Plasma in Progressive Supranuclear Palsy

Open‐Label Phase 1 Futility Studies of Salsalate and Young Plasma in Progressive Supranuclear Palsy

Four-Repeat Tauopathies: Current Management and Future Treatments

Mini-Mental State Examination: Optimal Cut-Off Levels for Mild and Severe Cognitive Impairment

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