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Friday, January 29, 2016

INTRACRANIAL ANEURYSMS : GENETICS AND ASSOCIATED DISEASES



Of patients who have SAH from ruptured aneurysm, 5% to 10% will have one or more first-order relatives who have also had a ruptured aneurysm. 

The inheritance is probably dominant with variable penetrance. 

Conditions associated with intracranial aneurysms include 〰〰〰〰〰〰〰〰〰〰〰〰〰〰〰〰〰

🏷polycystic kidney disease (5% of aneurysms series, 33% of polycystic kidney series)

🏷coarctation of the aorta (1% of aneurysm patients, 5% of coarctation patients, all of whom are hypertensive)

🏷sickle cell disease

🏷drug abuse (cocaine: generalized vasoconstriction and hypertension; intravenous use: mycotic aneurysms)

🏷hypertension (30% to 40% of patients with SAH). 

Rarer associations 
〰〰〰〰〰〰〰
🏷fibromuscular dysplasia

🏷Marfan's syndrome

🏷tuberous sclerosis

🏷Ehlers-Danlos syndrome

🏷hereditary hemorrhagic telangiectasia

🏷moyamoya disease

🏷pseudoxanthoma elasticum. 

🏷Choriocarcinoma 

🏷cardiac myxomas 


Ref: Handbook of Neuroanesthesia, 4th Edition, p:144

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