title: Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura.
abstract: 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.
authors.affiliation: Biomedicina Sperimentale e Neuroscienze Cliniche, University of Palermo, Via Gaetano La Loggia 1, 90129 Palermo, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Aridon
authors.forename: Paolo
authors.initials: P
authors.lastname: Ragonese
authors.forename: Maria Antonietta
authors.initials: MA
authors.lastname: Mazzola
authors.forename: Gerlando
authors.initials: G
authors.lastname: Quintini
authors.forename: Marianna
authors.initials: M
authors.lastname: Lo Re
authors.forename: Simona
authors.initials: S
authors.lastname: Talamanca
authors.forename: Valeria
authors.initials: V
authors.lastname: Terruso
authors.forename: Marco
authors.initials: M
authors.lastname: D'Amelio
authors.forename: Giovanni
authors.initials: G
authors.lastname: Savettieri
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010956:Plasmapheresis / Q000379:methods; D054038:Posterior Leukoencephalopathy Syndrome / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy*; D011696:Purpura, Thrombocytopenic / Q000150:complications* / Q000188:drug therapy / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone