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General Reference

Nejm 2001;344:898; Neurol 1995;45:1517

Pathophys and Cause

Cause:Spontaneous, or trauma that is often trivial ("hairdresser's stroke"), or spinal manipulation (Med Let 2000;44:50)

Pathophys:Intimal tear in extracranial portions of the vessels

Epidemiology

1/100000/yr for vertebral and 2.5/100000/yr for carotid. 2% of all ischemic CVAs but 10-25% of those age <50 yr; peak in 40s

Increased in Ehler-Danlos, Marfan's, osteogenesis imperfecta, polycystic kidney disease, and with positive family hx.

Signs and Symptoms

Sx:

Age 35-55 yr;

Carotid: sometimes h/o minor neck twist or trauma; facial pain (50%), unilateral headache (66%) often hemicrania, cerebellar ischemic sx (63%), retinal ischemia w amaurosis fugax, cranial nerve palsies (10%), dysgeusias, tinnitus (35%) often pulsatile

Vertebral: posterior neck pain (50%)/headache (66%); cerebral, cerebellar, brain stem ischemic sx (90%), eg, lateral medullary plate syndrome (Thrombotic Stroke)

Si:Horner's syndrome (46%) (Horner's Syndrome (Oculosympathetic Paresis)); bruit (24%) (Nejm 1994;330:393)

Course

Contralateral dissection in 2%, usually w/i 1 mo; rarely in same artery

Complications

Raeder's neuropathic pain (Horner's Syndrome (Oculosympathetic Paresis)); CVA; blindness

Lab and Xray

Xray:MRI/MRA (Nejm 1996;335:1368) usually diagnostic; or angiography

Treatment

Rx:Heparin followed by warfarin × 3 mo; surgery sometimes if progresses