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Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Series of 126 Patients
Medicine 86:1-7, Cottin,V.,et al, 2007
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Article Abstract
Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia, and visceral vascular manifestations. Infectious and ischemic central nervous system (CNS) manifestations due to embolism through pulmonary arteriovenous malformations (PAVMs) represent the main cases of morbidity. PAVMs were diagnosed as a result of systematic screening procedures (29%), incidental imaging findings (15%), dyspnea (22%), or CNS symptoms (13%). Fifty-three CNS events directly related to HHT (excluding migraine) were observed in 35% of patients: cerebral abscess (19.0%), ischemic cerebral stroke (9.5%), transient cerebral ischemic attack (6.3%), and cerebral hemorrhage (2.4%). The median age of onset was 33 years for cerebral abscesses (range, 11-66 years), and 53.5 years for ischemic cerebral events (range, 2-72 yr). Fifty-three CNS events directly related to HHT (excluding migraine) were observed in 35% of patients: cerebral abscess (19.0%), ischemic cerebral stroke (9.5%), transient cerebral ischemic attack (6.3%), and cerebral hemorrhage (2.4%). The median age of onset was 33 years for cerebral abscesses (range, 11-66 yr), and 53.5 years for ischemic cerebral events (range, 2-72 yr). Migraine was reported in 16% of patients. The diagnoses of PAVM and HHT were made at the time of the cerebral abscess in 13 cases (54%).
 
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abscess,intracerebral
CAT scan,chest
central nervous system,infection of
cerebral embolism
cerebrovascular accident
chest x-ray,abnormal
dyspnea
echocardiogram
echocardiogram,contrast
epistaxis
fistula,arterio-venous,pulmonary
gene
gene mutation
genetic neurologic disorders
heralding manifestation
hereditary hemorrhagic telangiectasia(HHT)
hypoxia
intracerebral hemorrhage
neurologic complications of,chronic pulmonary disease
pulmonary disease
review article
telangiectases
transient ischemic attack

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