Document Detail


Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura.
MedLine Citation:
PMID:  21234778     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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:
Paolo Aridon; Paolo Ragonese; Maria Antonietta Mazzola; Gerlando Quintini; Marianna Lo Re; Simona Talamanca; Valeria Terruso; Marco D'Amelio; Giovanni Savettieri
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-14
Journal Detail:
Title:  Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology     Volume:  -     ISSN:  1590-3478     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-1-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100959175     Medline TA:  Neurol Sci     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Biomedicina Sperimentale e Neuroscienze Cliniche (BIONEC), University of Palermo, Via Gaetano La Loggia 1, 90129, Palermo, Italy.
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