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

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Takayasu Arteritis

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  • • Obliterative arteriopathy involving aortic arch vessels

    • Abdominal aorta and pulmonary arteries can be affected

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Internal Carotid Dissection

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  • • 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

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Fibromuscular Dysplasia

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  • • 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

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Epidemiology

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Takayasu Arteritis

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  • • More common in women

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Internal Carotid Dissection

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  • • Due to trauma or hypertension

    • Most frequent in young adults

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Fibromuscular Dysplasia

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  • • Primarily young women affected

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Symptoms and Signs

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Internal Carotid Dissection

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  • • Ipsilateral cerebral ischemic symptoms

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

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Fibromuscular Dysplasia

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  • • 20% already had stroke

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Imaging Findings

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Internal Carotid Dissection

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  • Duplex US: Indicates narrowing

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

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Fibromuscular Dysplasia

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  • Arteriography: Characteristic "string of beads" appearance

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Rule Out

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  • • Atherosclerotic cerebral disease

    • Takayasu arteritis

    • Dissecting aortic aneurysm

    • Internal carotid dissection

    • Fibromuscular dysplasia

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  • • Duplex US

    • Arteriography

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Takayasu Arteritis

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  • • Corticosteroids and cyclophosphamide are effective

    • Operative treatment avoided unless active arteritis

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Internal Carotid Dissection

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  • • 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

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Fibromuscular Dysplasia

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  • • High incidence of neurologic disability

    • -Correct surgically—intraoperative graduated balloon dilation

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Surgery

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Indications

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  • • Recurrent TIAs

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References

Schievink WI: Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med 2001;344:898.  [PubMed: 11259724]

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