Hereditary Arteriopathies

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) affects young and middle-aged adults. It is associated with mutations of Notch 3 gene on chromosome 19. The disease involves the leptomeningeal and small paren-chymal arteries, and the small arteries of the peripheral nerves, muscles, and skin. The vessel walls are fibrotic and hyalinized, and basophilic and PAS-positive granules are deposited in the media. Skin biopsy and genetic studies confirm the diagnosis.

Familial cerebral amyloid angiopathy, a rare autosomal dominant disorder of the small arteries, predisposes to ischemic and hemorrhagic strokes. The Dutch variety affects elderly subjects, and the vascular deposits contain P-amyloid peptide. The Icelandic variety affects

FIGURE 4.3 1

Amyloid angiopathy. A. Amyloid angiopathy associated with lobar centropa-rietal hemorrhage in a 72-year-old man. B. Amyloid deposits in walls of small blood vessels (Congo red).

FIGURE 4.3 1

Amyloid angiopathy. A. Amyloid angiopathy associated with lobar centropa-rietal hemorrhage in a 72-year-old man. B. Amyloid deposits in walls of small blood vessels (Congo red).

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FIGURE 4.32

Aneurysms. A. Histologic section of a MCA aneurysm. The muscular and elastic layers of the artery end abruptly at the neck of the aneurysm. The aneurysmal wall consists of fibrous tissue (van Gieson-orcein). B. Circle of Willis dissected from the base of the brain shows a cherry-sized unruptured aneurysm at the point of junction of the MCA and posterior communicating artery C. Macrosection of an unruptured ACA aneurysm.

FIGURE 4.32

Aneurysms. A. Histologic section of a MCA aneurysm. The muscular and elastic layers of the artery end abruptly at the neck of the aneurysm. The aneurysmal wall consists of fibrous tissue (van Gieson-orcein). B. Circle of Willis dissected from the base of the brain shows a cherry-sized unruptured aneurysm at the point of junction of the MCA and posterior communicating artery C. Macrosection of an unruptured ACA aneurysm.

younger subjects, and the vascular deposits contain cys-tatin C, an abnormal microprotein (cystatin C amyloid angiopathy).

Ehlers-Danlos syndrome, an autosomal dominant or recessive disorder of collagen synthesis, predisposes to aneurysmal formation, carotid-cavernous fistula, and arterial dissection.

Marfan's syndrome, an autosomal dominant disorder of connective tissue, manifests with cardiovascular (aortic dissection, aneurysmal dilatation), ocular (lens dislocation), and skeletal (elongated habitus) anomalies.

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