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J Neurol Neurosurg Psychiatry. 2017 May;88(5):402-411. doi: 10.1136/jnnp-2016-314956. Epub 2017 Mar 1.

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

Author information

1
Department of Clinical Neuroscience, Brighton and Sussex Medical School, Brighton, UK.
2
Hurstwood Park Neurological Centre, Brighton and Sussex University Hospitals, Brighton, UK.

Abstract

OBJECTIVE:

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, HRs 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% CI 1.69 to 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% CI 0.83 to 1.80).In PSP and MSA, survival was predicted by early falls (multivariate HR 2.32; 95% CI 1.94 to 2.77), the Neuroprotection and Natural History in Parkinson Plus Syndromes Parkinson Plus Score and the Clinical Global Impression Disease Severity Score but not sex (multivariate HR 0.93; 95% CI 0.67 to 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; however, the lack of commonality of prognostic factors investigated is a significant limitation.

KEYWORDS:

Progressive supranuclear palsy; meta-analysis; multiple system atrophy; prognosis; prognostic factors; survival; systematic review

PMID:
28250027
DOI:
10.1136/jnnp-2016-314956
[Indexed for MEDLINE]

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