Progressive neurological decline after partial spontaneous thrombosis of a Spetzler-Martin Grade 5 arteriovenous malformation in a patient with Leiden factor V mutation: management and outcome

J Neurosurg. 2004 May;100(5):940-5. doi: 10.3171/jns.2004.100.5.0940.


The coexistence of a large intracranial arteriovenous malformation (AVM) and a hypercoagulation disorder is rare. The AVM puts the patient at risk for progressive neurological deficit, seizures, and, most importantly, intracranial hemorrhage The hypercoagulation disorder may result in an increased risk of stroke. The authors describe a 42-year-old man with a Spetzler-Martin Grade 5 AVM who experienced progressive neurological decline. He was subsequently discovered to have partial thrombosis of the AVM, deep cerebral and cortical venous thrombosis, and a hypercoagulation disorder. Hypercoagulation disorders causing neurological deficits are usually treated with anticoagulant medications; however, this approach was not thought to be safe in the presence of a large AVM. Therefore, the AVM nidus was surgically extirpated and a ventriculoperitoneal shunt was placed to treat the increased intracranial pressure caused by the cortical and deep cerebral venous thrombosis. Subsequently, lifelong oral anticoagulation was prescribed. The patient had a progressive neurological recovery and is now living independently at home. The occurrence of partial or complete spontaneous thrombosis of an AVM nidus should raise the possibility of an underlying hypercoagulation disorder.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anticoagulants / administration & dosage
  • Cerebral Angiography
  • Cerebral Cortex / blood supply
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Embolization, Therapeutic
  • Epilepsy, Tonic-Clonic / etiology*
  • Follow-Up Studies
  • Hemoglobins, Abnormal / genetics*
  • Humans
  • Intracranial Arteriovenous Malformations / classification
  • Intracranial Arteriovenous Malformations / diagnosis
  • Intracranial Arteriovenous Malformations / genetics*
  • Intracranial Arteriovenous Malformations / surgery
  • Intracranial Embolism / classification
  • Intracranial Embolism / diagnosis
  • Intracranial Embolism / genetics*
  • Intracranial Embolism / surgery
  • Intracranial Hypertension / diagnosis
  • Intracranial Hypertension / surgery
  • Long-Term Care
  • Magnetic Resonance Imaging
  • Male
  • Neurologic Examination*
  • Postoperative Care
  • Venous Thrombosis / diagnosis
  • Venous Thrombosis / genetics*
  • Venous Thrombosis / surgery
  • Ventriculoperitoneal Shunt


  • Anticoagulants
  • Hemoglobins, Abnormal
  • hemoglobin Leiden