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PT  - JOURNAL ARTICLE
AU  - Scaravilli, Alessandra
AU  - Capasso, Serena
AU  - Ugga, Lorenzo
AU  - Capuano, Ivana
AU  - Di Risi, Teodolinda
AU  - Pontillo, Giuseppe
AU  - Riccio, Eleonora
AU  - Tranfa, Mario
AU  - Pisani, Antonio
AU  - Brunetti, Arturo
AU  - Cocozza, Sirio
TI  - Clinical and Pathophysiologic Correlates of Basilar Artery Measurements in Fabry Disease
AID  - 10.3174/ajnr.A8403
DP  - 2024 Nov 01
TA  - American Journal of Neuroradiology
PG  - 1670--1677
VI  - 45
IP  - 11
4099  - http://www.ajnr.org/content/45/11/1670.short
4100  - http://www.ajnr.org/content/45/11/1670.full
SO  - Am. J. Neuroradiol.2024 Nov 01; 45
AB  - BACKGROUND AND PURPOSE: Alterations of the basilar artery (BA) anatomy have been suggested as a possible MRA feature of Fabry disease (FD). Nonetheless, no information about their clinical or pathophysiologic correlates is available, limiting our comprehension of the real impact of vessel remodeling in FD.MATERIALS AND METHODS: Brain MRIs of 53 subjects with FD (mean age, 40.7 [SD, 12.4] years; male/female ratio = 23:30) were collected in this single-center study. Mean BA diameter and its tortuosity index were calculated on MRA. Possible correlations between these metrics and clinical, laboratory, and advanced imaging variables of the posterior circulation were tested. In a subgroup of 20 subjects, a 2-year clinical and imaging follow-up was available, and possible longitudinal changes of these metrics and their ability to predict clinical scores were also probed.RESULTS: No significant association was found between MRA metrics and any clinical, laboratory, or advanced imaging variable (P values ranging from −0.006 to 0.32). At the follow-up examination, no changes were observed with time for the mean BA diameter (P = .84) and the tortuosity index (P = .70). Finally, baseline MRA variables failed to predict the clinical status of patients with FD at follow-up (P = .42 and 0.66, respectively).CONCLUSIONS: Alterations of the BA in FD lack of any meaningful association with clinical, laboratory, or advanced imaging findings collected in this study. Furthermore, this lack of correlation seems constant across time, suggesting stability over time. Taken together, these results suggest that the role of BA dolichoectasia in FD should be reconsidered.BAbasilar arteryFAfractional anisotropyFASTEXFAbry STabilization indEXFDFabry diseaseGb3globotriaosylceramideICCintraclass correlation coefficientLysoGb3globotriaosylsphingosineMSSIMainz Severity Score IndexPCAposterior cerebral arteryTItortuosity index