caa a22 4500 445355417 CHVBK 20180317142858.0 cr unu---uuuuu 170323e20110201xx s 000 0 eng 10.1007/s10072-010-0423-1 doi (NATIONALLICENCE)springer-10.1007/s10072-010-0423-1 Mixed connective tissue disease presenting as a peculiar myositis with poor muscle regeneration [Elektronische Daten] [Giorgio Tasca, Massimiliano Mirabella, Alfredo Berrettini, Mauro Monforte, Pietro Tonali, Enzo Ricci] Mixed connective tissue disease (MCTD) is a rheumatological disease which has to be distinguished from other entities causing inflammatory myopathy. The usual clinical presentation of inflammatory myopathy associated with connective tissue disease is not different from isolated polymyositis or dermatomyositis, i.e., subacute onset of proximal weakness affecting both upper and lower girdle with high serum CK level. Here we report a patient with MCTD/myositis overlap syndrome displaying an uncommon clinical presentation and a distribution of muscle weakness involving facial, neck and arm muscles with sparing of lower limbs. We also describe the scarcity of muscle regeneration signs on the muscle biopsy with complete absence of alkaline phosphatase positivity in the endomysial and permysial connective tissue as a novel finding of this condition. Springer-Verlag, 2010 Alkaline phosphatase staining nationallicence Inflammatory myopathy nationallicence Mixed connective tissue disease nationallicence Muscle regeneration nationallicence Tasca Giorgio Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut Mirabella Massimiliano Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut Berrettini Alfredo Department of Urology, Pediatric Urology Unit, University Hospital of Padua, Padua, Italy aut Monforte Mauro Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut Tonali Pietro Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut Ricci Enzo Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut Neurological Sciences Springer Milan 32/1(2011-02-01), 171-174 1590-1874 32:1<171 2011 32 10072 https://doi.org/10.1007/s10072-010-0423-1 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10072-010-0423-1 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Tasca Giorgio Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut NATIONALLICENCE 700 1- Mirabella Massimiliano Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut NATIONALLICENCE 700 1- Berrettini Alfredo Department of Urology, Pediatric Urology Unit, University Hospital of Padua, Padua, Italy aut NATIONALLICENCE 700 1- Monforte Mauro Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut NATIONALLICENCE 700 1- Tonali Pietro Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut NATIONALLICENCE 700 1- Ricci Enzo Institute of Neurology, Catholic University School of Medicine, Largo A. Gemelli 8, 00168, Rome, Italy aut NATIONALLICENCE 773 0- Neurological Sciences Springer Milan 32/1(2011-02-01), 171-174 1590-1874 32:1<171 2011 32 10072 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer