title: Retinal degeneration in primary-progressive multiple sclerosis: A role for cortical lesions?
abstract: Retinal atrophy in multiple sclerosis (MS) is secondary to optic nerve focal inflammation and to injury of the posterior visual pathway.
  
  
  
  To investigate the contribution of cortical lesions (CLs) to retinal pathology in primary-progressive multiple sclerosis (PPMS).
  
  
  
  We performed a cross-sectional evaluation of 25 patients and 20 controls, relating magnetic resonance imaging (MRI) metrics of visual pathway integrity with parameters derived from spectral-domain optical coherence tomography (peripapillary retinal nerve fiber layer (RNFL) thickness, ganglion cell + inner plexiform layer (GCIPL) thickness, and macular volume (MV)).
  
  
  
  Mean RNFL, GCIPL thickness, and MV were significantly reduced in patients compared to controls. MV and GCIPL thickness were significantly correlated with visual acuity. RNFL thinning was associated with thalamus and visual cortex volume (respectively, p = 0.01 and p < 0.05). In addition to thalamic volume, GCIPL thinning was associated with CLs and intracortical lesion number and volume, leucocortical lesion volume (all p ⩽ 0.05) while MV decrease was associated with CLs volume ( p = 0.05) and intracortical lesion number and volume ( p < 0.05).
  
  
  
  Our results suggest that RNFL thinning and GCIPL thinning/MV decrease may be explained by alternative mechanisms including retrograde trans-synaptic degeneration and/or a common pathophysiologic process affecting both the brain with CLs and the retina with neuronal loss.
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA/Department of Neuroscience, Reproductive Sciences and Odontostomatology, University of Naples Federico II, Naples, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Petracca
authors.affiliation: Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics and Maternal and Perinatal Sciences, University of Genoa, Genoa, Italy/Department of Neurology, University of California -San Francisco, San Francisco, CA, USA.
authors.forename: Christian
authors.initials: C
authors.lastname: Cordano
authors.affiliation: Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics and Maternal and Perinatal Sciences, University of Genoa, Genoa, Italy.
authors.forename: Maria
authors.initials: M
authors.lastname: Cellerino
authors.affiliation: Division of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Julia
authors.initials: J
authors.lastname: Button
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Stephen
authors.initials: S
authors.lastname: Krieger
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Roxana
authors.initials: R
authors.lastname: Vancea
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Rezwan
authors.initials: R
authors.lastname: Ghassemi
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Colleen
authors.initials: C
authors.lastname: Farrell
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Aaron
authors.initials: A
authors.lastname: Miller
authors.affiliation: Division of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Peter A
authors.initials: PA
authors.lastname: Calabresi
authors.affiliation: Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Fred
authors.initials: F
authors.lastname: Lublin
authors.affiliation: Departments of Neurology, Radiology and Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY, USA/Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics and Maternal and Perinatal Sciences, University of Genoa, Genoa, Italy.
authors.forename: Matilde
authors.initials: M
authors.lastname: Inglese
mesh_terms: D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020528:Multiple Sclerosis, Chronic Progressive / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D009412:Nerve Fibers / Q000473:pathology*; D009902:Optic Neuritis / Q000473:pathology; D012160:Retina / Q000473:pathology*; D012162:Retinal Degeneration / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012165:Retinal Ganglion Cells / Q000473:pathology*; D014795:Visual Pathways / Q000473:pathology
keywords: Multiple sclerosis; cortical lesions; magnetic resonance imaging; neurodegeneration; retina