caa a22 4500 445811277 CHVBK 20180317145212.0 cr unu---uuuuu 170323e20111101xx s 000 0 eng 10.1007/s00415-011-6060-7 doi (NATIONALLICENCE)springer-10.1007/s00415-011-6060-7 Nature and frequency of respiratory involvement in chronic progressive external ophthalmoplegia [Elektronische Daten] [Bart Smits, Yvonne Heijdra, Femke Cuppen, Baziel van Engelen] Chronic progressive external ophthalmoplegia (CPEO) is a relatively common mitochondrial disorder. Weakness of the extra-ocular, limb girdle and laryngeal muscles are established clinical features. Respiratory muscle involvement however has never been studied systematically, even though respiratory complications are one of the main causes of death. We therefore determined the prevalence and nature of respiratory muscle involvement in 23 patients with genetically confirmed CPEO. The main finding was decreased respiratory muscle strength, both expiratory (76.8% of predicted, p=0.002) and inspiratory (79.5% of predicted, p=0.004). Although the inspiratory vital capacity (92.5% of predicted, p=0.021) and the forced expiratory volume in 1s (89.3% of predicted, p=0.002) were below predicted values, both were still within the normal range in the majority of patients. Expiratory weakness was associated with a decreased vital capacity (ρ=0.502, p=0.015) and decreased peak expiratory flow (ρ=0.422, p=0.045). Moreover, expiratory muscle strength was lower in patients with limb girdle weakness (62.6±26.1% of predicted vs. 98.9±22.5% in patients with normal limb girdle strength, p=0.003), but was not associated with other clinical features, subjective respiratory complaints, disease severity or disease duration. Since respiratory involvement in CPEO is associated with severe morbidity and mortality, the present data justify periodic assessment of respiratory functions in all CPEO patients. The Author(s), 2011 Chronic progressive external ophthalmoplegia nationallicence Kearns-Sayre syndrome nationallicence Respiratory muscles nationallicence Pneumonia nationallicence Polymerase gamma nationallicence Smits Bart Neuromuscular Centre Nijmegen, Department of Neurology, Radboud University Nijmegen Medical Centre, Reinier Postlaan 4, 6500 HB, Nijmegen, The Netherlands aut Heijdra Yvonne Department of Pulmonary Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands aut Cuppen Femke Department of Pulmonary Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands aut van Engelen Baziel Neuromuscular Centre Nijmegen, Department of Neurology, Radboud University Nijmegen Medical Centre, Reinier Postlaan 4, 6500 HB, Nijmegen, The Netherlands aut Journal of Neurology Springer-Verlag 258/11(2011-11-01), 2020-2025 0340-5354 258:11<2020 2011 258 415 https://doi.org/10.1007/s00415-011-6060-7 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-6060-7 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Smits Bart Neuromuscular Centre Nijmegen, Department of Neurology, Radboud University Nijmegen Medical Centre, Reinier Postlaan 4, 6500 HB, Nijmegen, The Netherlands aut NATIONALLICENCE 700 1- Heijdra Yvonne Department of Pulmonary Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands aut NATIONALLICENCE 700 1- Cuppen Femke Department of Pulmonary Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands aut NATIONALLICENCE 700 1- van Engelen Baziel Neuromuscular Centre Nijmegen, Department of Neurology, Radboud University Nijmegen Medical Centre, Reinier Postlaan 4, 6500 HB, Nijmegen, The Netherlands aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/11(2011-11-01), 2020-2025 0340-5354 258:11<2020 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer