title: Association between reversible cerebral vasoconstriction syndrome and thrombotic thrombocytopenic purpura.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Neurology, Department of Medicine, National University Health System, Singapore.
authors.forename: Prakash R
authors.initials: PR
authors.lastname: Paliwal
authors.affiliation: Division of Neurology, Department of Medicine, National University Health System, Singapore.
authors.forename: Hock L
authors.initials: HL
authors.lastname: Teoh
authors.affiliation: Division of Neurology, Department of Medicine, National University Health System, Singapore; Yong Loo Lin School of Medicine, National University of Singapore, Singapore. Electronic address: drvijay@singnet.com.sg.
authors.forename: Vijay K
authors.initials: VK
authors.lastname: Sharma
mesh_terms: D000328:Adult; D005260:Female; D051270:Headache Disorders, Primary / Q000209:etiology; D006801:Humans; D018810:Magnetic Resonance Angiography; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000175:diagnosis / Q000209:etiology*; D018616:Ultrasonography, Doppler, Duplex; D020301:Vasospasm, Intracranial / Q000150:complications*
keywords: Cerebral vasculitis; Plasmapheresis; Reversible cerebral vasoconstriction syndrome; Thrombotic thrombocytopenic purpura; Thunderclap headache; Transcranial Doppler