title: White matter consequences of retinal receptor and ganglion cell damage.
abstract: OBJECTIVE
  Patients with Leber hereditary optic neuropathy (LHON) and cone-rod dystrophy (CRD) have central vision loss; but CRD damages the retinal photoreceptor layer, and LHON damages the retinal ganglion cell (RGC) layer. Using diffusion MRI, we measured how these two types of retinal damage affect the optic tract (ganglion cell axons) and optic radiation (geniculo-striate axons).
  
  
  METHODS
  Adult onset CRD (n = 5), LHON (n = 6), and healthy controls (n = 14) participated in the study. We used probabilistic fiber tractography to identify the optic tract and the optic radiation. We compared axial and radial diffusivity at many positions along the optic tract and the optic radiation.
  
  
  RESULTS
  In both types of patients, diffusion measures within the optic tract and the optic radiation differ from controls. The optic tract change is principally a decrease in axial diffusivity; the optic radiation change is principally an increase in radial diffusivity.
  
  
  CONCLUSIONS
  Both photoreceptor layer (CRD) and retinal ganglion cell (LHON) retinal disease causes substantial change in the visual white matter. These changes can be measured using diffusion MRI. The diffusion changes measured in the optic tract and the optic radiation differ, suggesting that they are caused by different biological mechanisms.
authors.affiliation: Department of Psychology, Stanford University, Stanford, California, United States Department of Ophthalmology, The Jikei University School of Medicine, Tokyo, Japan.
authors.forename: Shumpei
authors.initials: S
authors.lastname: Ogawa
authors.affiliation: Department of Psychology, Stanford University, Stanford, California, United States Department of Psychology, The University of Tokyo, Tokyo, Japan.
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Takemura
authors.affiliation: Department of Ophthalmology, The Jikei University School of Medicine, Tokyo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Horiguchi
authors.affiliation: Department of Psychology, The University of Tokyo, Tokyo, Japan Tamagawa University Brain Science Institute, Machida, Japan.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Terao
authors.affiliation: Department of Ophthalmology, Atsugi City Hospital, Kanagawa, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Haji
authors.affiliation: Department of Psychology, Stanford University, Stanford, California, United States.
authors.forename: Franco
authors.initials: F
authors.lastname: Pestilli
authors.affiliation: Department of Psychology, Stanford University, Stanford, California, United States.
authors.forename: Jason D
authors.initials: JD
authors.lastname: Yeatman
authors.affiliation: Department of Ophthalmology, The Jikei University School of Medicine, Tokyo, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tsuneoka
authors.affiliation: Department of Psychology, Stanford University, Stanford, California, United States.
authors.forename: Brian A
authors.initials: BA
authors.lastname: Wandell
authors.affiliation: Department of Ophthalmology, The Jikei University School of Medicine, Tokyo, Japan The Japan Society for the Promotion of Science, Tokyo, Japan.
authors.forename: Yoichiro
authors.initials: Y
authors.lastname: Masuda
mesh_terms: D000328:Adult; D000368:Aged; D016923:Cell Death; D038524:Diffusion Magnetic Resonance Imaging; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009412:Nerve Fibers / Q000473:pathology*; D029242:Optic Atrophy, Hereditary, Leber / Q000175:diagnosis*; D012165:Retinal Ganglion Cells / Q000473:pathology*; D041623:Tomography, Optical Coherence; D014795:Visual Pathways / Q000473:pathology*; D066127:White Matter / Q000473:pathology*; D055815:Young Adult
keywords: Leber hereditary optic neuropathy; cone-rod dystrophy; diffusion-MRI; white matter