caa a22 4500 445808187 CHVBK 20180317145203.0 cr unu---uuuuu 170323e20110701xx s 000 0 eng 10.1007/s00415-011-5914-3 doi (NATIONALLICENCE)springer-10.1007/s00415-011-5914-3 Hypogonadism in DM1 and its relationship to erectile dysfunction [Elektronische Daten] [Giovanni Antonini, Alessandro Clemenzi, Elisabetta Bucci, Emanuela De Marco, Stefania Morino, Antonella Di Pasquale, Pamela Latino, Gilda Ruga, Andrea Lenzi, Nicola Vanacore, Antonio Radicioni] Myotonic dystrophy type 1 (DM1) is characterized by both a premature appearance of age-related phenotypes and multiple organ involvement, which affects skeletal and smooth muscle as well as the eye, heart, central nervous system, and endocrine system. Although erectile dysfunction (ED) is a frequent complaint in patients with DM1, it has not been investigated in great depth. Hypogonadism, which is reported to be one of the physical causes of ED in the general population, frequently occurs in DM1. We planned this case-control study to evaluate the relationship between hypogonadism, as defined by the sexual hormone profile (FSH, LH, testosterone (T) and prolactin) and ED, as assessed by means of an internationally validated self-administered questionnaire (IIEF). DM1 patients had significantly increased mean levels of both gonadotropins (FSH and LH) (p<0.0001) and a reduced mean level of T (p<0.0001) when compared to controls. Twelve patients were eugonadic (normal LH, T, and FSH), while 18 displayed hormonal evidence of hypogonadism, characterized by tubular failure (increased FSH) in all the subjects and associated with interstitial failure in 14 subjects: seven with primary hypogonadism (increased LH and reduced T) and seven with compensated hypogonadism (increased LH and normal T). Patients with hormonal evidence of interstitial failure had a larger CTG expansion (p=0.008), longer disease duration (p=0.013), higher grade of disease (p=0.004) and lower erectile function score (p=0.02) than eugonadic patients. Impotence occurred in 13/14 hypogonadic patients with interstitial failure and in 5/12 eugonadic patients (p=0.017, OR=18.2). Springer-Verlag, 2011 Myotonic dystrophy nationallicence Erectile dysfunction nationallicence Hypogonadism nationallicence Antonini Giovanni Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut Clemenzi Alessandro Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut Bucci Elisabetta Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut De Marco Emanuela Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut Morino Stefania Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut Di Pasquale Antonella Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut Latino Pamela Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut Ruga Gilda Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut Lenzi Andrea Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut Vanacore Nicola National Institute of Health, Rome, Italy aut Radicioni Antonio Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut Journal of Neurology Springer-Verlag 258/7(2011-07-01), 1247-1253 0340-5354 258:7<1247 2011 258 415 https://doi.org/10.1007/s00415-011-5914-3 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-5914-3 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Antonini Giovanni Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut NATIONALLICENCE 700 1- Clemenzi Alessandro Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut NATIONALLICENCE 700 1- Bucci Elisabetta Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut NATIONALLICENCE 700 1- De Marco Emanuela Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut NATIONALLICENCE 700 1- Morino Stefania Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut NATIONALLICENCE 700 1- Di Pasquale Antonella Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut NATIONALLICENCE 700 1- Latino Pamela Department of Neurology, II School of Medicine, University of Rome "Sapienza”, c/o Ospedale Sant'Andrea, Via Grottarossa 1015, 00189, Rome, Italy aut NATIONALLICENCE 700 1- Ruga Gilda Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut NATIONALLICENCE 700 1- Lenzi Andrea Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut NATIONALLICENCE 700 1- Vanacore Nicola National Institute of Health, Rome, Italy aut NATIONALLICENCE 700 1- Radicioni Antonio Department of Experimental Medicine, I School of Medicine, University of Roma "Sapienza”, Rome, Italy aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/7(2011-07-01), 1247-1253 0340-5354 258:7<1247 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer