Association between reversible cerebral vasoconstriction syndrome and thrombotic thrombocytopenic purpura

Background: Reversible cerebrovascular constriction syndrome (RCVS) presents with thunderclap headache and ischemic neurological deficits due to focal vasoconstrictions in major intracranial arteries. Reversibility of focal vasoconstrictions is the hallmark of RCVS. The commonest conditions associated with RCVS are a post-partum state and administration of vasoactive agents.

Methods: We describe a series of 3 cases of thrombotic thrombocytopenic purpura (TTP) who presented with thunderclap headache and focal neurological deficits.

Results: Vascular imaging revealed focal areas of constrictions in multiple intracranial arteries. All cases were treated with plasmapheresis and serial transcranial Doppler (TCD) and magnetic resonance angiography revealed resolution of cerebral vasoconstriction, accompanied by clinical recovery.

Conclusion: RCVS should be considered in patients with TTP who presented with thunderclap headache. TCD is an important tool for monitoring the course of cerebral vasoconstriction in TTP.