Pupillographic findings in chronic progressive external ophthalmoplegia

Background: Chronic progressive external ophthalmoplegia (CPEO) as a mitochondrial disease entity is characterized by slowly progressive ocular immobility and ptosis and may be associated with other neurological or systemic symptoms (CPEOplus). Pupil involvement is usually not reported to be a feature of CPEO. However, systematic data on pupillary function in CPEO does not exist to date. We aimed to characterize the pattern of pupillary function in a clinically homogeneous series of genetically proven CPEO patients. Methods: Infrared video pupillography was performed in 15 CPEOplus patients and 22 age- and sex-m…