Alterated cerebrovascular reactivity in patients with adult-onset Pompe disease: consequences of vascular smooth muscle involvement?
O Hensel, F Hanisch, T Müller, I Katona, J Weis, S Zierz · Klinische Neurophysiologie · 2012 · Heft 03
Bibliografische Angaben
Zusammenfassung
Aims: Involvement of smooth muscle in blood vessels in late-onset glycogenosis type II (GSDII, Pompe disease), a skeletal muscle disease, suggests a multisystemic disorder. Patients/ Methods: Ten patients with GSDII (8/10 on enzyme replacement therapy, ERT) were screened for pathological glycogen accumulation in smooth muscles of blood vessels (skeletal muscle and skin biopsy), by magnetic resonance angiography (MR-A), transcranial duplex ultrasound and for vascular risk factors. Cerebrovascular reactivity as a function of smooth muscles in the arterioles was tested by changing the CO2 partial pressure. Results:…