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Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia
Stroke 32:877-882, Maher,C.O.,et al, 2001
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Article Abstract
Twelve patients (3.7%) had a history of cerebral vascular malformations. Ten patients had arteriovenous malformations. 1 had a dural arteriovenous fistula, and 1 had a cavernous malformation. Seven patients (2.1%) presented with intracran ial hemorrhage, 2 presented with seizures alone and 3 were discovered incidentally. The average age at the time of symptomatic intracranial hemorrhage was 25.4 years. All patients with a history of intracranial hemorrhage were classified as Rankin grade I or II at a mean follow-up interval of 6.0 years. A history of cerebral infarction or transient ischemic attack was found in 29.6% of patients with HHT and a pulmonary arteriovenous fistula. The risk of intracranial hemorrhage is low among people with HHT. Furthermore, a majority of these patients have a good functional outcome after hemorrhage. The data do not suggest a compelling indication for routine screening of patients with HHT for asymptomatic cerebral vascular malformations. By comparison, pulmonary arteriovenous fistulae are a much more frequent cause of neurological symptoms in this population.
 
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angiography,cerebral
arteriovenous malformation
arteriovenous malformation,cerebral
cerebral infarction
cerebrovascular accident
cerebrovascular accident,familial occurrence
familial
fistula,arterio-venous,pulmonary
hereditary hemorrhagic telangiectasia(HHT)
intracerebral hemorrhage
intracranial hemorrhage
malformation,vascular
malformation,vascular,cerebral
malformation,vascular,screening for
prognosis
review article
risk factors
telangiectases
telangiectases,retinal
transient ischemic attack

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