Reduced Cerebrovascular CO 2 Reactivity in CADASIL

• DOI: 10.1161/01.str.32.1.17
• Publication Date: 2011-06-17T20:08:03Z
• Authors: Thomas Pfefferkorn; Sebastian von Stu...; Jürgen Herzog; Thomas Gasser; Gerhard F. Hamann; Martin Dichgans
• Keywords: CADASIL; Angiopathy; Transcranial Doppler

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukencephalopathy (CADASIL) is a hereditary angiopathy caused by mutations in Notch3. microvessels show an accumulation of granular osmiophilic material the vicinity degenerating vascular smooth muscle cells. To study cerebrovascular function CADASIL, we performed measurements on cerebral hemodynamics using transcranial Doppler sonography.Middle artery (MCA) mean blood flow velocity (MFV), CO(2) reactivity, resistance index were measured bilateral sonography 29 CADASIL individuals (mean age, 49.0+/-2.4 years) equal number age- sex-matched control subjects.Compared subjects, reactivity was reduced (33.4+/-2.7% versus 45.3+/-3.0%; P:<0.01). This difference remained significant when only nondisabled (Rankin=0, n=21) included analysis (P:<0.05). significantly lower disabled than (24.5+/-2.7% 36.8+/-3.4%; P:<0.05). MCA MFV (45.6+/-2.2 cm/s 54.2+/-2.4 cm/s; P:<0.05) correlated negatively age both affected (r=-0.314; subjects (r=-0.339; Resistance not altered (59.0+/-1.0% 57.7+/-1.2%; P:=0.42).In there reduction basal MFV. The suggests functional impairment vasoreactivity probably related to cell dysfunction. early role impaired evolution disease.