6533b834fe1ef96bd129ce71

RESEARCH PRODUCT

Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

Simona TalamancaGiovanni SavettieriPaolo RagoneseValeria TerrusoGerlando QuintiniMarianna Lo ReMarco D'amelioMaria Antonietta MazzolaPaolo Aridon

subject

Malemedicine.medical_specialtyPathologyNeurologyThrombotic thrombocytopenic purpuraAnti-Inflammatory AgentsDermatologyMethylprednisolonehemic and lymphatic diseasesmedicineHumansmedicine.diagnostic_testbusiness.industryMagnetic resonance imagingGeneral MedicinePlasmapheresisMiddle Agedmedicine.diseaseReversible posterior leukoencephalopathy Thrombotic thrombocytopenic purpura ADAMTS-13Plasma exchangeADAMTS13SchistocyteSurgeryHydrocephalusPsychiatry and Mental healthPurpuraTreatment OutcomeMethylprednisolonePurpura ThrombocytopenicSettore MED/26 - NeurologiaNeurology (clinical)Posterior Leukoencephalopathy Syndromemedicine.symptombusinessmedicine.drug

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.

yearjournalcountryeditionlanguage
2011-01-01
10.1007/s10072-010-0465-4http://hdl.handle.net/10447/54452