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Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura

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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.

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Acknowledgments

We thank the reviewers for their careful reading of the manuscript and helpful comments and Melanie Flynn for detailed and insightful corrections.

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The authors certify that there is no current or potential conflict of interest in relation to this article.

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Correspondence to Giovanni Savettieri.

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Aridon, P., Ragonese, P., Mazzola, M.A. et al. Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura. Neurol Sci 32, 469–472 (2011). https://doi.org/10.1007/s10072-010-0465-4

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  • DOI: https://doi.org/10.1007/s10072-010-0465-4

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