Dilated basilar arteries in patients with congenital central hypoventilation syndrome

Abstract

Congenital central hypoventilation syndrome (CCHS) patients show hypoventilation during sleep and severe autonomic impairments, including aberrant cardiovascular regulation. Abnormal sympathetic patterns, together with increased and variable CO(2) levels, lead to the potential for sustained cerebral vasculature changes. We performed high-resolution T1-weighted imaging in 13 CCHS and 31 control subjects using a 3.0-T magnetic resonance imaging scanner, and evaluated resting basilar and bilateral middle cerebral artery cross-sections. Two T1-weighted image series were acquired; images were averaged and reoriented to common space, and regions containing basilar and both middle cerebral arteries were oversampled. Cross-sections of the basilar and middle cerebral arteries were manually outlined to calculate cross-sectional areas, and differences between and within groups were evaluated. Basilar arteries in CCHS were significantly dilated over control subjects, but both middle cerebral artery cross-sections were similar between groups. No significant differences appeared between left and right middle cerebral arteries within either group. Basilar artery dilation may result from differential sensitivity to high CO(2) over other vascular beds, damage to serotonergic or other chemosensitive cells accompanying the artery, or enhanced microvascular resistance, and that dilation may impair tissue perfusion, leading to further neural injury in CCHS.

Figures

Fig. 1

High-resolution T1-weighted images show the basilar artery in a CCHS and control subject. The upper panel shows longitudinal (A, sagittal section; B, coronal section) and cross-sectional views (C, axial section, white square; D, magnified area of the square in image C, white circle) of the basilar artery in a CCHS subject (age 14.3 years, male). The lower panel shows longitudinal (E, sagittal section; F, coronal section) and cross-sectional views (G, axial section, white square; H, magnified area of the square in image G, white circle) of the basilar artery in a control subject (14.4 years, male). The basilar artery cross-section in CCHS is larger than the control subject (D vs. H).

Fig. 2

High-resolution T1-weighted images show both left (L) and right (R) middle cerebral arteries in a CCHS and control subject. The upper panel is a longitudinal view (A, coronal section) and cross-sectional views (B, sagittal section; C, sagittal section) of middle cerebral arteries in a CCHS subject (age 13.5 years, female). The lower panel shows longitudinal (D, coronal section) and cross-sectional views (E, sagittal section; F, sagittal section) of middle cerebral arteries in a control subject (14.0 years, female). Both left and right middle cerebral arteries in CCHS show comparable cross-sections as found in the control subject.

Fig. 3

Basilar artery cross-sectional areas from individual control (△) and CCHS (○) subjects. Basilar cross-sectional areas were significantly enlarged in CCHS over control subjects.

Fig. 4

Left and right middle cerebral arteries' cross-sectional areas from individual control (△) and CCHS (○) subjects. Neither left nor right cross-sections showed any significant differences between groups.