title: Retinal nerve fiber layer evaluation by optical coherence tomography in Leber's hereditary optic neuropathy.
abstract: OBJECTIVE
  To study retinal nerve fiber layer (RNFL) thickness by optical coherence tomography (StratusOCT) in patients with Leber's hereditary optic neuropathy (LHON).
  
  
  METHODS
  Cross-sectional study.
  
  
  METHODS
  Thirty-eight patients with LHON were analyzed and compared with an age-matched control group of 75 patients. Patients with LHON were classified as having early LHON (E-LHON, n = 8) when the duration of the disease was shorter than 6 months and atrophic LHON (A-LHON, n = 30) when the duration was longer than 6 months.
  
  
  METHODS
  The fast RNFL thickness (3.4) scan acquisition protocol was used.
  
  
  METHODS
  Retinal nerve fiber layer thickness as measured by StratusOCT.
  
  
  RESULTS
  Compared with the control group, eyes with E-LHON showed a thicker RNFL in the 360 degrees average measurement (P<0.01) and in the superior (P<0.01), nasal (P<0.05), and inferior quadrants (P<0.05); no significant changes were detected in the temporal quadrant. Eyes with A-LHON revealed a thinner RNFL in all measurements (P<0.001); the fibers of the nasal quadrant showed the lowest amount of reduction (38% vs. 42%-49.8% in the other quadrants). In cases with A-LHON and visual recovery, RNFL was significantly thicker in all measurements (P<0.001), except the temporal quadrant, with respect to A-LHON without visual recovery.
  
  
  CONCLUSIONS
  On the basis of OCT data, the RNFL is thickened in E-LHON and severely thinned in A-LHON. RNFL is likely to be partially preserved in A-LHON with visual recovery. The temporal fibers (papillomacular bundle) are the first and most severely affected; the nasal fibers seem to be partially spared in the late stage of the disease.
authors.affiliation: Centro di Oftalmologia Salus, Bologna, Italy. p.barboni@eyeproject.com <p.barboni@eyeproject.com>
authors.forename: Piero
authors.initials: P
authors.lastname: Barboni
authors.forename: Giacomo
authors.initials: G
authors.lastname: Savini
authors.forename: Maria Lucia
authors.initials: ML
authors.lastname: Valentino
authors.forename: Pasquale
authors.initials: P
authors.lastname: Montagna
authors.forename: Pietro
authors.initials: P
authors.lastname: Cortelli
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: De Negri
authors.forename: Federico
authors.initials: F
authors.lastname: Sadun
authors.forename: Stefania
authors.initials: S
authors.lastname: Bianchi
authors.forename: Lora
authors.initials: L
authors.lastname: Longanesi
authors.forename: Maurizio
authors.initials: M
authors.lastname: Zanini
authors.forename: Antonello
authors.initials: A
authors.lastname: de Vivo
authors.forename: Valerio
authors.initials: V
authors.lastname: Carelli
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003430:Cross-Sectional Studies; D003941:Diagnostic Techniques, Ophthalmological; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009412:Nerve Fibers / Q000473:pathology*; D029242:Optic Atrophy, Hereditary, Leber / Q000175:diagnosis* / Q000235:genetics; D012165:Retinal Ganglion Cells / Q000473:pathology*; D041623:Tomography, Optical Coherence