Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia

Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia.

BACKGROUND AND PURPOSE Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. METHODS Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. A...

Cerebrovascular Manifestations of Hereditary Hemorrhagic Telangiectasia.

eNS Manifestations of Hereditary Hemorrhagic Telangiectasia 569

Hereditary hemorrhagic telangiectasia (HHT) is a familial angiodysplastic disorder. Dermal, mucosal, and visceral vascular lesions of this disorder are well known. However, central nervous system (CNS) manifestations, occurring in as many as one-third of patients, have not been well appreciated until recently. The etiology of neurologic symptomatology includes hypoxemia or ischemia secondary to...

Clinical manifestations of hereditary hemorrhagic telangiectasia.

Sixty-four patients with symptomatic hereditary hemorrhagic telangiectasia were retrospectively studied in order to determine the true incidence of clinical manifestations in this disease. This select group had a significantly higher incidence of gastrointestinal hemorrhage and pulmonary arteriovenous fistula formation than has been previously reported. Data are presented regarding the course a...

Bleeding risk of cerebrovascular malformations in hereditary hemorrhagic telangiectasia.

OBJECT Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant vascular dysplasia with a high prevalence of cerebrovascular malformations (CVMs), mostly manifested as arteriovenous malformations (AVMs). The natural history and bleeding risk of these CVMs is unknown. The authors investigated the risk of bleeding in conjunction with clinical and radiological features in patients ...