title: MR imaging of tuberous sclerosis: pathogenesis of this phakomatosis, use of gadopentetate dimeglumine, and literature review.
abstract: Findings on cranial magnetic resonance (MR) images were correlated with known histopathologic findings in 42 patients with tuberous sclerosis (TS), 17 of whom received gadopentetate dimeglumine, to extend the range of signs on MR images. Four neuroradiologists recorded the number, sites, configurations, and relative signal intensities of intracranial abnormalities. White matter lesions, found in 39 patients (93%), showed four distinct patterns: (a) straight or curvilinear bands extending radially from the ventricle through the cerebral mantle toward the cortex, (b) wedge-shaped lesions, (c) nonspecific conglomerate foci, and (d) cerebellar radial bands. It is concluded that cortical tubers, white matter lesions, subependymal nodules, and sub-ependymal giant cell astrocytomas (SGCAs) may be enhanced after administration of gadopenetetate dimeglumine. Enhancement of a TS lesion does not indicate neoplastic transformation into SGCA. Imaging surveillance every 12 months appears indicated during the peak ages (8-18 years) of occurrence of SGCA. The appearance of white matter lesions supports the theory that the cerebral lesions of TS may be caused by disordered migration of dysgenetic cells.
authors.affiliation: Department of Radiology, Memorial Hospital, Hollywood, FL 33021.
authors.forename: B H
authors.initials: BH
authors.lastname: Braffman
authors.forename: L T
authors.initials: LT
authors.lastname: Bilaniuk
authors.forename: T P
authors.initials: TP
authors.lastname: Naidich
authors.forename: N R
authors.initials: NR
authors.lastname: Altman
authors.forename: M J
authors.initials: MJ
authors.lastname: Post
authors.forename: R M
authors.initials: RM
authors.lastname: Quencer
authors.forename: R A
authors.initials: RA
authors.lastname: Zimmerman
authors.forename: B A
authors.initials: BA
authors.lastname: Brody
mesh_terms: D000293:Adolescent; D000328:Adult; D001254:Astrocytoma / Q000175:diagnosis; D001921:Brain / Q000473:pathology*; D001932:Brain Neoplasms / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D004338:Drug Combinations; D005260:Female; D019786:Gadolinium DTPA; D005910:Glioma / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008536:Meglumine; D009942:Organometallic Compounds; D004369:Pentetic Acid; D014402:Tuberous Sclerosis / Q000175:diagnosis* / Q000473:pathology
chemical_list: D003287:Contrast Media; D004338:Drug Combinations; D009942:Organometallic Compounds; D008536:Meglumine; D004369:Pentetic Acid; D019786:Gadolinium DTPA