Cerebral Hemodynamic Changes to Transcranial Doppler in Asymptomatic Patients with Fabry’s Disease

Brain Sciences

MargheritaStefania Rodolico¹  Francesco Fisicaro²  Rita Bella³  Carla Vagli³  Raffaele Ferri⁴  Giuseppe Lanza⁴  Luisa Vinciguerra⁵  Valentina Puglisi⁵  Antonello Giordano⁶ 

[1] C.N.R. Institute for Biomedical Research and Innovation–IRIB, Section of Catania, Via P. Gaifami 18, 95126 Catania, Italy;Department of Biomedical and Biotechnological Science, University of Catania, Via Santa Sofia 89, 95123 Catania, Italy;Department of Medical and Surgical Sciences and Advanced Technologies, University of Catania, Via Santa Sofia 78, 95123 Catania, Italy;Department of Neurology IC, Oasi Research Institute-IRCCS, Via Conte Ruggero 73, 94018 Troina, Italy;Department of Neurology, Azienda Socio-Sanitaria Territoriale (ASST) Cremona, Viale Concordia 1, 26100 Cremona, Italy;Department of Neurology, Guzzardi Hospital, Via Papa Giovanni XXIII, 97019 Vittoria, Italy;

关键词: Fabry’s disease;    cerebrovascular disease;    white matter lesions;    transcranial Doppler;    cerebral blood flow;    cerebral hemodynamics;   

DOI  :  10.3390/brainsci10080546

来源: DOAJ

【 摘 要 】

Background: Patients with Fabry’s disease (FD) may be asymptomatic or show a spectrum of clinical manifestations, including cerebrovascular disease, mainly affecting posterior circulation. Few and conflicting studies on cerebral blood flow (CBF) velocity by transcranial Doppler sonography (TCD) in asymptomatic FD (aFD) subjects have been published. Our study aims to assess TCD in aFD subjects to identify any preclinical CBF change. Methods: A total of 30 aFD subjects were consecutively recruited and compared to 28 healthy controls. Brain magnetic resonance imaging was normal in all participants. TCD was used to study blood flow velocity and indices of resistance of intracranial arteries from the middle cerebral artery (MCA), bilaterally, and from the basilar artery (BA). Cerebral vasomotor reactivity (CVR) was also evaluated from MCA. Results: No difference was found between groups for MCA parameters of CBF velocity and CVR. Compared to controls, a higher mean blood flow velocity and a lower resistance index from BA were observed in FD subjects. No correlation was found between any BA-derived TCD parameter and the level of lyso-globotriaosylceramide. Conclusions: aFD subjects show evidence of altered CBF velocity in posterior circulation. Preclinical detection of neurovascular involvement in FD might allow appropriate management and prevention of future cerebrovascular complications and disability.

【 授权许可】

Unknown