caa a22 4500 44535609X CHVBK 20180317142900.0 cr unu---uuuuu 170323e20110601xx s 000 0 eng 10.1007/s10072-010-0465-4 doi (NATIONALLICENCE)springer-10.1007/s10072-010-0465-4 Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura [Elektronische Daten] [Paolo Aridon, Paolo Ragonese, Maria Mazzola, Gerlando Quintini, Marianna Lo Re, Simona Talamanca, Valeria Terruso, Marco D'Amelio, Giovanni Savettieri] Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by fever, microangiopathic haemolytic anaemia, renal insufficiency, and thrombocytopenia. Neurological involvement, a prominent component of TTP, is characterised by a variety of brain lesions which include reversible cerebral oedema or magnetic resonance imaging (MRI) features of reversible posterior leukoencephalopathy syndrome (RPLS). TTP is frequently associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13. Here, we report a case of TTP with severe acute encephalopathy. Posterior leukoencephalopathy and brainstem oedema with triventricular hydrocephalus were observed on MRI. The low activity of ADAMTS13 was not observed and ADAMTS-13 antibodies were absent. Neurological symptoms and patient's condition were completely resolved by plasma exchange therapy in addition to high dose of methylprednisolone. Springer-Verlag, 2010 Reversible posterior leukoencephalopathy nationallicence Thrombotic thrombocytopenic purpura nationallicence ADAMTS-13 nationallicence Plasma exchange nationallicence Aridon Paolo Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Ragonese Paolo Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Mazzola Maria Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Quintini Gerlando U.O. di Ematologia, Azienda Ospedaliera Universitaria Policlinico Paolo Giaccone dell'Università degli Studi di Palermo, Palermo, Italy aut Lo Re Marianna Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Talamanca Simona Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Terruso Valeria Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut D'Amelio Marco Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Savettieri Giovanni Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut Neurological Sciences Springer Milan 32/3(2011-06-01), 469-472 1590-1874 32:3<469 2011 32 10072 https://doi.org/10.1007/s10072-010-0465-4 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10072-010-0465-4 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Aridon Paolo Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- Ragonese Paolo Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- Mazzola Maria Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- Quintini Gerlando U.O. di Ematologia, Azienda Ospedaliera Universitaria Policlinico Paolo Giaccone dell'Università degli Studi di Palermo, Palermo, Italy aut NATIONALLICENCE 700 1- Lo Re Marianna Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- Talamanca Simona Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- Terruso Valeria Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- D'Amelio Marco Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 700 1- Savettieri Giovanni Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy aut NATIONALLICENCE 773 0- Neurological Sciences Springer Milan 32/3(2011-06-01), 469-472 1590-1874 32:3<469 2011 32 10072 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer