title: Diffuse Brain Hypoperfusion in Advanced Leukoencephalopathy with Calcifications and Cysts.
abstract: Leukoencephalopathy with calcifications and cysts (LCC) is an uncommon condition of unknown etiology occurring in children and adults. Pathological findings include obliterative hyalinosis of the small vessels, myelin loss, intense gliosis, Rosenthal fiber formation, microcalcifications, and hemosiderin deposits. Herein we report a 55-year-old man with LCC documented 10 years ago, in whom we examined brain perfusion by pseudocontinuous arterial spin labeling technique. We demonstrated diffused hypoperfusion of the affected white matter (WM) and of the subcortical gray matter (GM) and cortical GM in the patient in comparison to a group of healthy control subjects, using both qualitative evaluation and region of interest analysis. WM and subcortical GM hypoperfusion reflects the known distribution of LCC microangiopathy. We speculate that cortical hypoperfusion may be related to cerebral atrophy or may reflect deafferentation secondary to severe leukoencephalopathy, and may possibly contribute to severe motor and cognitive impairment. Further studies addressing cerebral blood flow in LCC are necessary.
authors.affiliation: Neurology Unit and Laboratories, A. Meyer Children's Hospital, Florence, Italy; Imago7, IRCCS Stella Maris Foundation, Pisa, Italy. Electronic address: emanuele.bartolini@meyer.it.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Bartolini
authors.affiliation: Structural and Functional Neuroradiology Research Program, A. Meyer Children's and Careggi Hospitals of Florence, Florence, Italy; "Mario Serio" Department of Experimental and Clinical Biomedical Sciences, University of Florence, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Bianchi
authors.affiliation: "Il Bene" Center for Immunological and Rare Neurological Diseases at Bellaria Hospital, IRCCS Neurologia Città di Bologna, Bologna, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Bartolomei
authors.affiliation: Nuclear Medicine, "Le Scotte" University Hospital of Siena, Siena, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Vella
authors.affiliation: "Mario Serio" Department of Experimental and Clinical Biomedical Sciences, University of Florence, Italy.
authors.forename: Lapo
authors.initials: L
authors.lastname: Sali
authors.affiliation: Medical Physics, A.Meyer Children Hospital of Florence, Florence, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Ciccarone
authors.affiliation: "Il Bene" Center for Immunological and Rare Neurological Diseases at Bellaria Hospital, IRCCS Neurologia Città di Bologna, Bologna, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Salvi
authors.affiliation: Structural and Functional Neuroradiology Research Program, A. Meyer Children's and Careggi Hospitals of Florence, Florence, Italy; "Mario Serio" Department of Experimental and Clinical Biomedical Sciences, University of Florence, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Mascalchi
mesh_terms: D002545:Brain Ischemia / Q000000981:diagnostic imaging / Q000209:etiology*; D002114:Calcinosis / Q000150:complications* / Q000000981:diagnostic imaging; D002560:Cerebrovascular Circulation / Q000502:physiology; D003560:Cysts / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D056784:Leukoencephalopathies / Q000150:complications* / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged
keywords: 3-T MRI; White matter; arterial spin labeling; neuroimaging; perfusion; perfusion MRI; regional CBF