Severity dependent distribution of impairments in PSP and CBS: Interactive visualizations

Brittain, Clare; McCarthy, Andrew; Irizarry, Michael C.; McDermott, Dana; Biglan, Kevin; Ser, Teodoro del; Williams, David R.; Lafontaine, Anne; Marras, Connie; Jog, Mandar; Panisset, Michael; Lang, Anthony E.; Parker, Lesley H.; Stewart, Alistair; Corvol, Jean‐Christophe; Azulay, Jean‐Philippe; Couratier, Philippe; Mollenhauer, Brit; Lorenzl, Stefan; Benecke, R.; Woitalla, Dirk; Chan, Dennis; Zermansky, Adam; Lees, Andrew J.; Gozes, Illana; Lobach, Iryna; Honig, Lawrence S.; Zamrini, Edward; Pahwa, Rajesh; Driver-Dunkley, Erika; Lessig, Stephanie; Lew, Mark F.; Womack, Kyle; Boeve, Brad F.; Hillis, Argyle; Kaufer, Daniel I.; Kumar, Rajeev; Xie, Tao; Gunzler, Steven A.; Zesiewicz, Theresa; Dayalu, Praveen; Grossman, Murray; Jankovic, Joseph; Santiago, Anthony; Tuite, Paul; Isaacson, Stuart; Leegwater‐Kim, Julie; Knopman, David S.; Miller, Bruce L.; Schneider, Lon S.; Doody, Rachelle S.; Roberson, Erik D.; Koestler, Mary; Jack, Clifford R.; Deerlin, Viviana Van; Randolph, Christopher; Whitaker, Steve; Hirman, Joe; Gold, Michael; Morimoto, Bruce H.; G, Georg Nuebling; Hensler, Mira; Paul, Sabine; Zwergal, Andreas; Heuer, Hilary W.; Tartaglia, Maria Carmela; McGinnis, Scott; Dickerson, Bradford C.; Kornak, John; Schuff, Norbert; Rabinovici, Gil D.; Rosen, Howard J.; Boxer, Adam L.; Gomez, Jennifer; Tijero, Beatriz; Berganzo, Koldo; Yébenes, Justo Garcı́a de; Sendón, José Luis López; García, Gloria; Tolosa, Eduardo; Buongiorno, Maríateresa; Bargalló, Núria; Burguera, J.A.; Martinez, I. T. Arellano; Ruiz-Mart'ınez, J.; Narrativel, I.; Vivancos, Francisco; Ybot, I.; Aguilar, Miquel; Quilez, P.; Boada, Merçé; Lafuente, Asunción; Hernández, Israel; López-Lozano, Juan J.; Mata, Milena Liberal da; Kupsch, Andreas; Lipp, Axel; Ebersbach, Georg; Schmidt, Tina; Hahn, Kristine; Höglinger, Günter U.; Höllerhage, Matthias; Oertel, Wolfgang H.; Respondek, Gesine; Stamelou, María; Reichmann, Heinz; Wolz, Martin; Schneider, Caroline; Klingelhöfer, Lisa; Berg, Daniela; Maetzler, Walter; Srulijes, Karin; Ludolph, Albert C.; Kassubek, Jan; Steiger, M. J.; Tyler, Kimberly E.; Burn, David; Morris, Lorna; Ling, Huiqin; Hauser, Robert A.; McClain, Terry; Truong, Daniel D.; Jenkins, Sem; Litvan, Irene; Houghton, David; Ferrara, Joseph M.; Bordelon, Yvette; Gratiano, A.; Golbe, Lawrence I.; Mark, Margery H.; Uitti, Ryan J.; Gerpen, J. Ven

doi:10.1016/j.parkreldis.2018.08.025

Cited by 7 publications

(4 citation statements)

References 20 publications

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“…A recent analysis supports our choice to use only dysphagia and gait/balance in formulating the staging system. 22 It calculated the relative normalized contributions of the 6 PSPRS components to the total PSPRS as the total worsened over time. It found that the "gait" component progressed the fastest relative to the other 5, with 90% of patients reaching the "severe" state in the gait-related items (ie, the worst of the 3 states available in that model) by the time the total PSPRS score reached 40 (of 100).…”

Section: Discussionmentioning

confidence: 99%

A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy

Golbe

Ohman‐Strickland

Beisser

et al. 2020

Movement Disord Clin Pract

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Background Progressive supranuclear palsy (PSP) causes major disability, shortens life, and as yet has no disease‐modifying and little symptomatic treatment. A convenient prognostic tool is needed to assist patients, families, and clinicians in planning care. Objectives We calculated times to acquisition of certain disease milestones and death. Methods We followed a cohort of 417 patients with PSP–Richardson syndrome from 1995 to 2016, applying the Progressive Supranuclear Palsy Rating Scale (PSPRS) at each visit. We generated median times to acquisition of 13 milestones using the input variables of sex, onset age, rate of disease progression from motor symptom onset to initial visit, and PSPRS score at the baseline. Of the outcome milestones, 5 were stages of a new, provisional PSP staging system. The other 8 milestones comprised death and disabling levels of cognitive loss, gaze palsy, dysarthria, dysphagia, and gait/balance impairment. Results We derived median times to milestones, with 25th and 75th percentiles and 95% confidence intervals of the median for baseline PSPRS scores from 25 to 65 (scale range, 0–100). Sex and initial progression velocity significantly influenced the death milestone, but not most of the others. Median time to death ranged from 4.8 years for a man with PSPRS score of 25 and a slow progression velocity from onset to initial visit of 0.51 PSPRS points/month to 1.8 years for a woman with PSPRS 65 and rapid initial velocity of 2.25 points/month. Conclusions We have created a convenient, inexpensive, noninvasive reference for counseling patients with PSP–Richardson syndrome on approximate time to encountering 13 life‐altering disease milestones.

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Section: Discussionmentioning

confidence: 99%

A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy

Golbe

Ohman‐Strickland

Beisser

et al. 2020

Movement Disord Clin Pract

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“…It is typified by rapid progression and a short survival 2 . Characteristic clinical features of PSP include unintelligible speech, akinesia, bulbar impairments, cognitive dysfunction, supranuclear gaze palsy, and postural instability 3,4 . The impairment of these functions is measured using the Progressive Supranuclear Palsy Rating Scale (PSPRS) 5 and the Movement Disorder Society criteria (MDS‐PSP).…”

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confidence: 99%

“… 2 Characteristic clinical features of PSP include unintelligible speech, akinesia, bulbar impairments, cognitive dysfunction, supranuclear gaze palsy, and postural instability. 3 , 4 The impairment of these functions is measured using the Progressive Supranuclear Palsy Rating Scale (PSPRS) 5 and the Movement Disorder Society criteria (MDS‐PSP) . 6 Although developed for Parkinson's disease (PD), the Movement Disorders Society‐Unified Parkinson's Disease Rating Scale (MDS‐UPDRS) 7 , 8 may also be useful in evaluating the motor features of PSP.…”

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confidence: 99%

Longitudinal Monitoring of Progressive Supranuclear Palsy using Body‐Worn Movement Sensors

Sotirakis

Conway

et al. 2022

Movement Disorders

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Background We have previously shown that wearable technology and machine learning techniques can accurately discriminate between progressive supranuclear palsy (PSP), Parkinson's disease, and healthy controls. To date these techniques have not been applied in longitudinal studies of disease progression in PSP. Objectives We aimed to establish whether data collected by a body‐worn inertial measurement unit (IMU) network could predict clinical rating scale scores in PSP and whether it could be used to track disease progression. Methods We studied gait and postural stability in 17 participants with PSP over five visits at 3‐month intervals. Participants performed a 2‐minute walk and an assessment of postural stability by standing for 30 seconds with their eyes closed, while wearing an array of six IMUs. Results Thirty‐two gait and posture features were identified, which progressed significantly with time. A simple linear regression model incorporating the three features with the clearest progression pattern was able to detect statistically significant progression 3 months in advance of the clinical scores. A more complex linear regression and a random forest approach did not improve on this. Conclusions The reduced variability of the models, in comparison to clinical rating scales, allows a significant change in disease status from baseline to be observed at an earlier stage. The current study sheds light on the individual features that are important in tracking disease progression. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society

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“…A task force report15 recommended clinical tools to assess PSP disability and progression, including motor, cognitive, behavioural and functional measures. While these measures have proved to effectively measure clinical symptoms and to report natural history of the disease,7 16 very few studies17 have explored the longitudinal change in different symptom domains. The effectiveness of these clinical scales to detect changes in different PSP features is still a matter of discussion in the literature.…”

Section: Introductionmentioning

confidence: 99%

Longitudinal changes of early motor and cognitive symptoms in progressive supranuclear palsy: the OxQUIP study

Pereira

Buchanan²,

Höglinger

et al. 2022

BMJ Neurol Open

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BackgroundProgressive supranuclear palsy (PSP) is a rare neurodegenerative condition characterised by a range of motor and cognitive symptoms. Very little is known about the longitudinal change in these symptoms over time. Moreover, the effectiveness of clinical scales to detect early changes in PSP is still a matter of debate.ObjectiveWe aimed to determine longitudinal changes in PSP features using multiple closely spaced follow-up time points over a period of 2 years.Methods28 healthy control and 28 PSP participants, with average time since onset of symptoms of 1.9 years, were prospectively studied every 3 months for up to 24 months. Changes from baseline scores were calculated at each follow-up time point using multiple clinical scales to identify longitudinal progression of motor and cognitive symptoms.ResultsThe Montreal Cognitive Assessment, but not the Mini-Mental State Examination, detected cognitive decline at baseline. Both scales revealed poor longitudinal sensitivity to clinical change in global cognitive symptoms. Conversely, the Movement Disorders Society Unified Parkinson’s disease Rating Scale – part III and the PSP Rating Scale (PSPRS) reliably detected motor decline less than 2 years after disease onset. The ‘Gait/Midline’ PSPRS subscore consistently declined over time, with the earliest change being observed 6 months after baseline assessment.ConclusionWhile better cognitive screening tools are still needed to monitor cognitive decline in PSP, motor decline is consistently captured by clinical rating scales. These results support the inclusion of multiple follow-up time points in longitudinal studies in the early stages of PSP.

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Severity dependent distribution of impairments in PSP and CBS: Interactive visualizations

Abstract: The PSP Rating Scale captures disease severity in both PSP and CBS. Modelling how domains change in relation to one other at varying disease severities may facilitate detection of therapeutic effects in future clinical trials.

Cited by 7 publications

References 20 publications

A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy

A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy

Longitudinal Monitoring of Progressive Supranuclear Palsy using Body‐Worn Movement Sensors

Longitudinal changes of early motor and cognitive symptoms in progressive supranuclear palsy: the OxQUIP study

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