title: Basilar Artery Changes in Fabry Disease.
abstract: Dolichoectasia of the basilar artery is a characteristic finding of Fabry disease. However, its prevalence, severity, and course have been poorly studied. This study quantitatively evaluated, by MRA, a panel of basilar artery parameters in a large cohort of patients with Fabry disease.
  
  
  
  Basilar artery mean diameter, curved length, "origin-to-end" linear distance (linear length), and tortuosity index ([curved length ÷ linear length] - 1) were retrospectively measured on 1.5T MRA studies of 110 patients with Fabry disease (mean age, 39.4 ± 18.6 years; 40 males) and 108 control patients (mean age, 42.0 ± 18.2 years; 40 males).
  
  
  
  Patients with Fabry disease had increased basilar artery mean diameter (P< .001) and basilar artery linear length (P= .02) compared with control patients. Basilar artery curved length and tortuosity index correlated with age in both groups (P< .001), whereas basilar artery linear length correlated with age only in patients with Fabry disease (P= .002). Patients with Fabry disease showed a basilar artery curved length mean increase of 4.2% (9.7% in male patients with Fabry disease versus male control patients), whereas the basilar artery mean diameter had a mean increase of 12.4% (14.3% in male patients with Fabry disease versus male control patients). Male patients with Fabry disease had increased basilar artery mean diameter, curved length, and tortuosity index compared with female patients with Fabry disease (P= .04,P= .02, andP< .001, respectively) and male control patients (P< .001,P= .01, andP= .006, respectively). Female patients with Fabry disease demonstrated an age-dependent increase of basilar artery mean diameter that became significant (P< .001) compared with female control patients above the age of 45 years.
  
  
  
  The basilar artery of patients with FD is subjected to major remodeling that differs according to age and sex, thus providing interesting clues about the pathophysiology of cerebral vessels in Fabry disease.
authors.affiliation: From the Sezione di Neuroscienze (R.M.), University of Salerno, Salerno, Italy.
authors.forename: R
authors.identifier: 0000-0002-5130-3971
authors.initials: R
authors.lastname: Manara
authors.affiliation: Radiology Division (R.Y.C., F.C.).
authors.forename: R Y
authors.identifier: 0000-0002-7530-301X
authors.initials: RY
authors.lastname: Carlier
authors.affiliation: Department of Neurosciences (S.R., V.C., M.E.), University of Padova, Padova, Italy.
authors.forename: S
authors.identifier: 0000-0003-1013-6158
authors.initials: S
authors.lastname: Righetto
authors.affiliation: Department of Neurosciences (S.R., V.C., M.E.), University of Padova, Padova, Italy.
authors.forename: V
authors.identifier: 0000-0003-2033-5927
authors.initials: V
authors.lastname: Citton
authors.affiliation: S. Giovanni e Ruggi d'Aragona Hospital (G.L.), Salerno, Italy.
authors.forename: G
authors.identifier: 0000-0001-6612-7002
authors.initials: G
authors.lastname: Locatelli
authors.affiliation: Radiology Division (R.Y.C., F.C.).|Division of Medical Genetics (F.C., D.P.G.), University of Versailles, Versailles, France.
authors.forename: F
authors.identifier: 0000-0003-3708-4917
authors.initials: F
authors.lastname: Colas
authors.affiliation: Department of Neurosciences (S.R., V.C., M.E.), University of Padova, Padova, Italy.
authors.forename: M
authors.identifier: 0000-0001-6788-6190
authors.initials: M
authors.lastname: Ermani
authors.affiliation: Division of Medical Genetics (F.C., D.P.G.), University of Versailles, Versailles, France.
authors.forename: D P
authors.identifier: 0000-0002-8355-007X
authors.initials: DP
authors.lastname: Germain
authors.affiliation: Neurological Unit (A.B.), St. Bassiano Hospital, Bassano del Grappa, Italy. alessandro.burlina@aslbassano.it.
authors.forename: A
authors.identifier: 0000-0002-2224-4706
authors.initials: A
authors.lastname: Burlina
mesh_terms: D000328:Adult; D000368:Aged; D001488:Basilar Artery / Q000473:pathology*; D001921:Brain / Q000473:pathology*; D000795:Fabry Disease / Q000473:pathology*; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult