Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis

Abstract

(2017) Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. Journal of Neurology, Neurosurgery and Psychiatry, 88 (16). This version is available from Sussex Research Online: http://sro.sussex.ac.uk/67644/ This document is made available in accordance with publisher policies and may differ from the published version or from the version of record. If you wish to cite this item you are advised to consult the publisher's version. Please see the URL above for details on accessing the published version. Copyright and reuse:Sussex Research Online is a digital repository of the research output of the University.Copyright and all moral rights to the version of the paper presented here belong to the individual author(s) and/or other copyright owners. To the extent reasonable and practicable, the material made available in SRO has been checked for eligibility before being made available.Copies of full text items generally can be reproduced, displayed or performed and given to third parties in any format or medium for personal research or study, educational, or not-for-profit purposes without prior permission or charge, provided that the authors, title and full bibliographic details are credited, a hyperlink and/or URL is given for the original metadata page and the content is not changed in any way. ABSTRACTObjective: To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).Methods: Publications of at least 10 patients with a likely or confirmed diagnosis of PSP or MSA were eligible for inclusion. Methodological quality was rated using a modified version of the Quality in Prognostic Studies tool. For frequently examined prognostic factors, hazard ratios (HR) derived by univariate and multivariate analysis were pooled in separate subgroups; other results were synthesised narratively and HRs could not be reported here. Results:Thirty-seven studies presenting findings on 6193 patients (1911 PSP, 4282 MSA) fulfilled the inclusion criteria. We identified the following variables as unfavourable predictors of survival: In PSP: PSP-Richardson's phenotype (univariate HR: 2.53; 95%CIs: 1.69, 3.78), early dysphagia and early cognitive symptoms. In MSA: severe dysautonomia and early development of combined autonomic and motor features but not MSA phenotype (multivariate HR: 1.22; 95%CIs: 0.83, 1.80).In PSP and MSA survival was predicted by early falls (multivariate HR: 2.32; 95%CIs: 1.94, 2.77), the NNIPPS Parkinson plus score and the Clinical Global Impression disease severity score but not sex (multivariate HR: 0.93; 95%CIs: 0.67, 1.28). There was conflicting evidence regarding the prognostic effect of age at onset and stridor. Conclusion:Several clinical variables were strongly associated with shorter survival in PSP and MSA. Results on most prognostic factors were consistent across methodologically diverse studies;...

Neuropathological validation of the MDS-PSP criteria with PSP and other frontotemporal lobar degeneration

“…Dementia, dysphagia and supranuclear gaze palsy have been variably reported to be clinical predictors of death in PSP-RS. 30, 31 In our PSP-F cohort, both dysphagia and supranuclear gaze palsy were over-expressed compared to bvFTD patients, suggesting a possible role of either symptom on survival. However, dysphagia was the only symptom not consistently over-expressed in PSP-SL and PSP-CBS cases.…”

Diagnosis of multiple system atrophy

“…Indeed, the diagnosis of probable or possible MSA according to the 2008 consensus criteria (Table 1) relies on either the presence of OH or urinary dysfunction indicating pathological involvement of autonomic neurons (Gilman et al, 2008). Early and severe autonomic failure appears to be associated with poorer prognosis (Coon et al, 2015; Glasmacher et al, 2017). Bladder and sexual dysfunction (erectile dysfunction in men and dyspareunia in women) as well as orthostatic dizziness, lightheadedness or syncope due to neurogenic OH were the earliest signs of the disease in up to 50% of patients, years before the emergence of motor signs (Kaufmann et al, 2017b).…”

The Role of Rehabilitation in Patients With Progressive Supranuclear Palsy: A Narrative Review

“…Given that most disabling disorders in PSP are due to balance deficit, walking impairment and falls with related injuries, all studies predominantly focused on these functional domains, in particular balance and gait impairment, apart from one study that addressed voice and speech phonation [26]. Indeed, recent studies have shown that postural instability with early falls, Richardson‐type PSP, and dementia are the main predictive negative factors [32,33]. With regard to balance, all case reports showed an improvement of this functionality with a reduction in falls.…”