title: Retinal pathology in idiopathic moyamoya angiopathy detected by optical coherence tomography.
abstract: OBJECTIVE
  To investigate whether patients with moyamoya angiopathy without obvious retinal pathologies such as retinal infarctions or the congenital morning glory anomaly may have subtle subclinical retinal changes.
  
  
  METHODS
  In this cross-sectional study, spectral domain optical coherence tomography was used to analyze the retinal morphology of 25 patients with idiopathic moyamoya angiopathy and 25 age- and sex-matched healthy controls. We analyzed the retinal vasculature with blue laser autofluorescence, lipofuscin deposits with MultiColor confocal scanning laser ophthalmoscopy, and the optic nerve head (ONH) volume with a custom postprocessing algorithm. In addition to the total retinal thickness, semiautomated segmentation was used for segmentation of retinal layers in macular cross scans, macular volume scans, and peripapillary ring scans.
  
  
  RESULTS
  The main finding was a pronounced reduction of the ONH volume in moyamoya angiopathy compared with controls (0.76 ± 0.45 mm(3) and 1.47 ± 0.50 mm(3), respectively; p < 0.0001), which was associated with a less pronounced reduction of the retinal nerve fiber layer in macular volume scans (0.97 ± 0.11 mm(3) and 1.10 ± 0.10 mm(3), respectively; p < 0.001). Autofluorescence and MultiColor confocal scanning laser ophthalmoscopy images revealed no pathologies except for one branch retinal artery occlusion.
  
  
  CONCLUSIONS
  Our results indicate that even patients with moyamoya angiopathy who do not have obvious retinal abnormalities have retinal abnormalities. These can be detected by spectral domain optical coherence tomography, and the association of ONH abnormalities with the vascular changes may suggest that idiopathic moyamoya angiography is a systemic disease involving abnormalities of the early mesodermal development.
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany. phil.albrecht@gmail.com.
authors.forename: Philipp
authors.initials: P
authors.lastname: Albrecht
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Christine
authors.initials: C
authors.lastname: Blasberg
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Lukas
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Marius
authors.initials: M
authors.lastname: Ringelstein
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Ann-Kristin
authors.initials: AK
authors.lastname: Müller
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Harmel
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Ella-Maria
authors.initials: EM
authors.lastname: Kadas
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: David
authors.initials: D
authors.lastname: Finis
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Rainer
authors.initials: R
authors.lastname: Guthoff
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Orhan
authors.initials: O
authors.lastname: Aktas
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Hartung
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Friedemann
authors.initials: F
authors.lastname: Paul
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Alexander U
authors.initials: AU
authors.lastname: Brandt
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Berlit
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Axel
authors.initials: A
authors.lastname: Methner
authors.affiliation: From the Departments of Neurology (P.A., C.B., M.R., A.-K.M., J.H., O.A., H.-P.H.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Neurology Clinic (S.L.), Department of Clinical Neuroimmunology and Multiple Sclerosis Research, University Medical Center Zürich, Switzerland; Charité-Universitätsmedizin Berlin (E.-M.K., F.P., A.U.B.), NeuroCure Clinical Research Center and Department of Neurology, Berlin; Department of Neurology (A.M.), University Medical Center, Johannes Gutenberg University Mainz; and Department of Neurology (P.B., M.K.), Alfried Krupp Hospital, Essen, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Kraemer
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000150:complications* / Q000473:pathology; D009898:Optic Disk / Q000473:pathology; D012044:Regression Analysis; D012160:Retina / Q000473:pathology*; D012164:Retinal Diseases / Q000175:diagnosis* / Q000209:etiology*; D018709:Statistics, Nonparametric; D041623:Tomography, Optical Coherence / Q000379:methods*