Cerebrovascular Disease, Nonatherosclerotic

• • Primary disease of extracranial arteries other than atherosclerosis is rare

Takayasu Arteritis

• • Obliterative arteriopathy involving aortic arch vessels

  • Abdominal aorta and pulmonary arteries can be affected

Internal Carotid Dissection

• • Originates in internal carotid artery

  • Acute event that narrows or obliterates lumen

  • Primary lesion is intimal tear at distal end of carotid bulb

  • May develop spontaneously

Fibromuscular Dysplasia

• • Nonatherosclerotic angiopathy, unknown cause affects specific arteries

  • Usually bilateral disease, involves middle third of internal carotid

  • Irregular zones of overgrowing media, causing concentric rings

  • 20% already had stroke at presentation

Epidemiology

Takayasu Arteritis

• • More common in women

Internal Carotid Dissection

• • Due to trauma or hypertension

  • Most frequent in young adults

Fibromuscular Dysplasia

• • Primarily young women affected

Symptoms and Signs

Internal Carotid Dissection

• • Ipsilateral cerebral ischemic symptoms

  • Acute neck pain, cervical tenderness at the angle of mandible

Fibromuscular Dysplasia

• • 20% already had stroke

Imaging Findings

Internal Carotid Dissection

• • Duplex US: Indicates narrowing

  • Arteriography: Characteristic tapered narrowing; if lumen persists, it resumes normal caliber beyond bony foramen

Fibromuscular Dysplasia

• • Arteriography: Characteristic "string of beads" appearance

Rule Out

• • Atherosclerotic cerebral disease

  • Takayasu arteritis

  • Dissecting aortic aneurysm

  • Internal carotid dissection

  • Fibromuscular dysplasia

• • Duplex US

  • Arteriography

Takayasu Arteritis

• • Corticosteroids and cyclophosphamide are effective

  • Operative treatment avoided unless active arteritis

Internal Carotid Dissection

• • Anticoagulation treatment of choice

  • -In most cases, intramural clot is resorbed restoring normal lumen

  • Operation only for recurrent transient ischemic attacks (TIAs)

  • -If dissection only proximal, replace segment with graft,

    -If stump pressure > 65 mm Hg, consider proximal ligation

    -Extracranial-intracranial bypass possible

Fibromuscular Dysplasia

• • High incidence of neurologic disability

  • -Correct surgically—intraoperative graduated balloon dilation

Surgery

Indications

• • Recurrent TIAs

References