Chronic Neurological Impairment in Patients with Thrombotic Thrombocytopenic Purpura: Findings in Quantitative MRI

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening, microvascular blood disorder that affects approximately 5 people per million per year. The disorder is characterized by insufficient activity in ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats 13), which is an important enzyme in hemostasis because it prevents thrombosis. Along with blood clotting, other predominant symptoms are fever, anaemia, kidney failure, and neurological changes. Neurological changes may include confusion and decreased levels of consciousness, as well as depression and increased risk of seizures or stroke. However, little is known about the general pathology of these neurological changes and this forms the motivation for this research. An observational study using a comprehensive MRI protocol was evaluated in 13 patients and compared to results from assessments of depression and cognition. Despite prolonged remission, there is evidence of persistent neurocognitive decline as manifested in higher scores of depression and widespread white matter lesions

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