Background and Aims:
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by reduced activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13). It is characteriszed by platelet aggregation, which causes microangiopathic haemolytic anaemia and thrombocytopenia. Clinically, there is a variety of symptoms. TTP is a rare cause of stroke. Untreated it has a mortality up to 90%.

 
Methods:
A 29-year-old woman presented with a numb feeling and paresis of the right arm and hand. Initial laboratory findings were unremarkable except for high D-dimer (1721 ug/L). CT-brain showed small subarachnoid haemorrhage in the left frontal lobe. Brain-MRI showed multiple areas of recent ischemia and suggestion of subarachnoid blood in the left central sulcus. With working hypothesis cortical vein thrombosis, she was treated with fraxiparin and advised to quit smoking and using oral contraceptives.

Six weeks later she developed new stroke symptoms, extreme fatigue, disorientation and headache. Physical examination showed petechiae. Laboratory testing showed thrombocytopenia (5*109/L), microangiopathic haemolytic anaemia (Hb 3.4 mmol/l, reticulocytes 323) and light renal failure. Clotting factors were unremarkable. Activity of ADAMTS13 was <5%. She was diagnosed with TTP and treated with plasma exchange (PEX). 
Results:
After treatment with PEX her neurological deficits improved and laboratory findings returned to normal.
Conclusions:
Thrombotic thrombocytopenic purpura is a rare cause of neurologic symptoms. In this case typical laboratory findings were found after a delay of a few weeks after first presentation with stroke. TTP has to be considered a potential diagnosis in young patients presenting with stroke, even if initial laboratory findings are normal.

STROKE AND DELAYED THROMBOTIC THROMBOCYTOPENIC PURPURA IN A YOUNG WOMAN