Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Paolo Aridon • Paolo Ragonese • Maria Antonietta Mazzola • Gerlando Quintini • Marianna Lo Re • Simona Talamanca • Valeria Terruso • Marco D’Amelio
- چاپ و سال / کشور: 2011
Description
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.
Neurol Sci (2011) 32:469–472 DOI 10.1007/s10072-010-0465-4 Received: 21 February 2010 / Accepted: 24 November 2010 / Published online: 14 January 2011
