Clinical and demographic features of chronic progressive external ophthalmoplegia in a large adult-onset cohort

Julia N Heighton; Lauren I Brady; Matthew C Newman; Mark A Tarnopolsky

doi:10.1016/j.mito.2017.12.006

Clinical and demographic features of chronic progressive external ophthalmoplegia in a large adult-onset cohort

Mitochondrion. 2019 Jan:44:15-19. doi: 10.1016/j.mito.2017.12.006. Epub 2017 Dec 12.

Authors

Julia N Heighton¹, Lauren I Brady¹, Matthew C Newman¹, Mark A Tarnopolsky²

Affiliations

¹ McMaster University Medical Centre, Hamilton, ON, Canada.
² McMaster University Medical Centre, Hamilton, ON, Canada. Electronic address: tarnopol@mcmaster.ca.

PMID: 29246868
DOI: 10.1016/j.mito.2017.12.006

Abstract

Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. We evaluated the impact of sex and smoking status upon knee extension strength and the phenotypic spectrum of disease in a large cohort of adult-onset CPEO patients (N=116) using retrospective chart analysis. The CPEO patients showed significantly lower knee extension strength as compared to the age- and sex-matched control population (-37%, P<0.05). Smoking also negatively impacted knee extension strength only in women with CPEO (-26%, P<0.05). We conclude that smoking and female sex interact negatively in CPEO patients.

Keywords: Chronic progressive external ophthalmoplegia; Mitochondria; Phenotypic spectrum; Smoking; Strength.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Aged
Aged, 80 and over
Female
Humans
Late Onset Disorders / epidemiology*
Late Onset Disorders / pathology*
Male
Middle Aged
Ophthalmoplegia, Chronic Progressive External / epidemiology*
Ophthalmoplegia, Chronic Progressive External / pathology*
Retrospective Studies
Risk Factors
Sex Factors
Smoking / adverse effects
Young Adult