Thrombotic thrombocytopenic purpura with reversible splenial lesion syndrome: a case report

Chunming Yong,Ang Xing,Lingyan Zhou,Shuhao Liu,Xiaomeng Wang,Chunxiao Fei,Junqing Zhang,Song Hu,Xinyue Hou

doi:10.1186/s12883-020-01696-2

Abstract

BackgroundReversible splenial lesion syndrome (RESLES) is known to cause severe psychiatric symptoms but is also a very rare clinical disease in which the specific aetiology is unknown. According to current reports, there are major causes of the disease, including viral or bacterial infection, epilepsy, anti-epileptic drug withdrawal, high-altitude cerebral oedema, and metabolic disorders such as hypoglycaemia and hypernatraemia. In this article, we report a patient with thrombotic thrombocytopenic purpura (TTP) who presented with RESLES.Case presentationA 34-year-old female patient who presented with fever and progression of disorder of consciousness was eventually diagnosed with RESLES based on brain imaging. Moreover, clinical features and peripheral smears demonstrating schistocytes and thrombocytopenia confirmed a diagnosis of TTP. RESLES can be improved by plasma exchange therapy.ConclusionThis rare case highlights the occurrence of RESLES as a presenting feature of the expanding list of unusual neurological manifestations of TTP.

Highlights

Reversible splenial lesion syndrome (RESLES) is known to cause severe psychiatric symptoms but is a very rare clinical disease in which the specific aetiology is unknown
This rare case highlights the occurrence of RESLES as a presenting feature of the expanding list of unusual neurological manifestations of thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura (TTP) is an invasive thrombotic microangiopathy classically defined by the pentad of fever, renal dysfunction, severe thrombocytopenia, haemolytic anaemia, terminal organ dysfunction and CNS involvement [1]

Summary

Introduction

Reversible splenial lesion syndrome (RESLES) is known to cause severe psychiatric symptoms but is a very rare clinical disease in which the specific aetiology is unknown. TTP can cause neurological symptoms such as consciousness disorders in 80% of patients and can have a 90% mortality rate. Multiple brain imaging abnormalities have been reported in patients with TTP [1]. We report a patient with TTP who presented with reversible splenial lesion syndrome (RESLES).

Results

Conclusion