Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

被引：6

作者：

Aridon, Paolo ^{[1
]}

Ragonese, Paolo ^{[1
]}

Mazzola, Maria Antonietta ^{[1
]}

Quintini, Gerlando ^{[2
]}

Lo Re, Marianna ^{[1
]}

Talamanca, Simona ^{[1
]}

Terruso, Valeria ^{[1
]}

D'Amelio, Marco ^{[1
]}

Savettieri, Giovanni ^{[1
]}

机构：

[1] Univ Palermo, I-90129 Palermo, Italy

[2] Univ Palermo, UO Ematol, Azienda Osped Univ Policlin Paolo Giacco, Palermo, Italy

来源：

NEUROLOGICAL SCIENCES | 2011年 / 32卷 / 03期

关键词：

Reversible posterior leukoencephalopathy; Thrombotic thrombocytopenic purpura; ADAMTS-13; Plasma exchange; ENCEPHALOPATHY SYNDROME;

D O I：

10.1007/s10072-010-0465-4

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：469 / 472

页数：4

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