Morphology and function of cerebral arteries in adults with pompe disease.

ABSTRACT:

Objective

Cerebrovascular abnormalities have been reported in adult patients with Pompe disease. The objective was to study these abnormalities by (1) determining the diameter and mean flow velocity (MFV) of large cerebral arteries and (2) estimating cerebral blood flow (CBF), resistance index (RI) and cerebrovascular reactivity (CVR) as functions of resistance vessels.

Methods

In ten adults with Pompe disease and twenty controls, the diameter, peak systolic (PSV) and end-diastolic velocities (EDV) of arteries supplying the brain were quantified by MR angiography and sonography. MFV, RI and CBF were calculated. CVR in the middle cerebral artery (MCA) was determined by hyperventilation and acetazolamide injection.

Results

MR angiography revealed dilation of cerebral ar

SUBMITTER: Hensel O 

PROVIDER: S-EPMC4375123 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature