Objective: To investigate survival and its determinants in a Tunisian cohort of Progressive Supranuclear Palsy (PSP).

Background: PSP is a neurodegenerative tauopathy characterized by faster progression and earlier mortality compared to Parkinson’s disease. However, the predictors of survival are still controversial.

Method: We conducted a retrospective study over a period of 17 years (2004 – 2020) in the Department of Neurology of Razi University Hospital, in Tunis, Tunisia, including a group of PSP patients diagnosed and classified into phenotypes according to the 2017 MDS-PSP criteria. Survival statistics were analyzed considering demographic data, phenotypes (PSP-RS, PSP-cortical phenotypes: PSP-F + PSP-SL + PSP-CBS + PSP-PLS;PSP-subcortical phenotypes: PSP-P + PSP-PGF + PSP-PI + PSP-OM + PSP-C), early motor and cognitive signs (during the first 3 years) and ApoE genotypes.

Results: We collected 50 patients (SR=1.4, mean age= 68.2±7.4 years, mean age of onset= 61.6±7.9 years).The mean survival time from symptom onset was 6.6±5.2 years. Older age at onset (>65 years), RS and cortical phenotypes, early dysphagia, early spastic dysarthria and E3E4 genotype were predictors of shorter survival. However, sex, extrapyramidal signs, cognitive impairment did not predictthe outcome.

Conclusion: Our results support the fact that early recognition of the PSP phenotype and identification of the ApoE genotype would help predict survival in PSP patients. Larger prospective studies are needed to better investigate the various survival determinants.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/predictors-of-survival-in-progressive-supranuclear-palsy-in-a-tunisian-cohort/