Noninflammatory Vasculopathies

Thromboangiitis obliterans occurs chiefly in middle-aged men who smoke heavily. It commonly involves the peripheral arteries. The disease may also involve the medium-sized and small meningeal and cerebral arteries, producing intimal proliferation, luminal obliteration, and multiple small infarctions (Fig. 4.30).

Moyamoya disease affects young adults. The basal cerebral arteries undergo severe intimal thickening and luminal occlusion. Subsequently, an abnormal collateral vascular network develops at the base of the brain. This network appears on angiogram as a puff of smoke, hence the name moyamoya (Japanese; puff of smoke).

Fibromuscular dysplasia occurs among the middle-aged, mostly in women. The cervical carotids and the vertebrals show segmental muscular fibrous thickening and loss of the elastic layer, which appear on angiogram as string of beads. The disease presents with ischemic stroke and is an important cause of arterial dissection.

Amyloid angiopathy affects the leptomeningeal and the medium-sized and small cortical arteries. The amyloid deposited in the media and adventitia of arteries stains positively with Congo red and shows green birefringence under polarized light. It immunoreacts for P-amyloid peptide, a cleavage product of amyloid precursor protein. The disease affects the elderly and is associated with Alzheimer's disease. It causes small infarctions and intracerebral lobar hemorrhages (Fig. 4.31).

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