Spinal Cord Vascular Diseases

Essentials of Diagnosis

• Sudden onset of back or limb pain and neurologic deficit in limbs

• Motor, sensory, or reflex changes in limbs depending on level of lesion

• Imaging studies distinguish between infarct and hematoma

General Considerations

Infarction of the spinal cord

• Rare and typically occurs in the anterior spinal artery territory (which supplies the anterior two-thirds of the cord) because this artery is supplied by only a limited number of feeders

• Usually caused by interrupted flow in one or more of these feeders, eg, with aortic dissection, aortic aneurysm, aortography, polyarteritis, or severe hypotension, or after surgical repair of the thoracic or abdominal aorta

• Usually caudal because the anterior spinal artery receives numerous feeders in the cervical region

• Spinal cord hypoperfusion may lead to a central cord syndrome with distal weakness of lower motor neuron type and loss of pain and temperature appreciation, with preserved posterior column function

Epidural or subdural hemorrhage

• May occur in patients with bleeding disorders or those who are taking anticoagulant drugs, sometimes following trauma or lumbar puncture

• Epidural hemorrhage may also be related to a vascular malformation or tumor deposit

Spinal dural arteriovenous fistulae

• Congenital lesions that present with spinal subarachnoid hemorrhage or myeloradiculopathy

• Most such fistulae are in the thoracolumbar region

• Cervical dural arteriovenous fistulae lead to symptoms and signs in the arms

Symptoms and Signs

Infarction of the spinal cord

• Acute onset of flaccid, areflexic paraplegia that evolves after a few days or weeks into a spastic paraplegia with extensor plantar responses

• Dissociated sensory loss, with impairment of appreciation of pain and temperature but preservation of vibration and position sense

Epidural or subdural hemorrhage

• Sudden severe back pain followed by an acute compressive myelopathy necessitating urgent CT or MRI and surgical evacuation

Spinal dural arteriovenous fistulae

• Motor and sensory disturbances in the legs and sphincter disorders

• Pain in the legs or back often severe

• An upper, lower, or mixed motor deficit in the legs revealed on examination

• Sensory deficits also present and usually extensive, although occasionally confined to radicular distribution

• Spinal subarachnoid hemorrhage sometimes occurs, especially with cervical lesions

Differential Diagnosis

• Primary tumor, eg, ependymoma, meningioma, neurofibroma

• Lymphoma, leukemia, plasma cell myeloma (formerly multiple myeloma)

• Metastases, eg, cancer of the prostate, breast, lung, kidney

• Cervical or lumbar disk disease

• Epidural abscess

• Multiple sclerosis

• Tuberculosis (Pott disease)

Imaging Studies

• Urgent imaging (CT or MRI) is indicated for epidural or subdural hemorrhage

• Spinal dural arteriovenous fistulae

  • Myelography (performed with the patient prone and supine) shows serpiginous filling defects due to enlarged vessels

  • Selective spinal arteriography confirms the diagnosis

  • Spinal MRI may not detect the disorder, although most cases show either T2 hyperintensity in the cord or perimedullary flow voids

Surgery

• Epidural or subdural hemorrhage: urgent surgical evacuation is indicated

• Spinal dural arteriovenous fistulae that are posterior to the cord can be treated by ligation of feeding vessels and excision of the fistulous anomaly or by embolization procedures

Therapeutic Procedures

• Infarction of the spinal cord

  • If signs of infarction are noted after surgery, blood pressure augmentation for 24–48 hours in addition to lumbar drainage has been noted anecdotally to improve outcomes

  • Treatment is otherwise symptomatic

Prognosis

• Delay in treatment of a spinal dural arteriovenous fistula may lead to increased and irreversible disability or to death from recurrent subarachnoid hemorrhage

When to Refer

• All patients

When to Admit

• All patients