CNS-MANIFESTATIONS OF HEREDITARY HEMORRHAGIC TELANGIECTASIA

  • 1 January 1984
    • journal article
    • research article
    • Vol. 5  (5) , 569-573
Abstract
Hereditary hemorrhagic telangiectasia (HHT) is a familial angiodysplastic disorder. Dermal, mucosal, and visceral vascular lesions of this disorder are well known. However, CNS manifestations, occurring in as many as 1.3 of the patients, have not been well appreciated until recently. The etiology of neurologic symptomatology includes hypoxemia or ischemia secondary to pulmonary arteriovenous shunting, vascular lesions of the brain and spinal cord ranging from aneurysms to arteriovenous malformations, brain abscesses secondary to pulmonary arteriovenous fistulas, and portal systemic encephalopathy. Angiographic and computed tomographic findings in 4 patients with CNS involvement in HHT are reported.

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