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Macular nerve fibre and ganglion cell layer changes in acute Leber's hereditary optic neuropathy
  1. Nicole Balducci1,
  2. Giacomo Savini2,
  3. Maria Lucia Cascavilla3,
  4. Chiara La Morgia4,5,
  5. Giacinto Triolo3,
  6. Rosa Giglio3,
  7. Michele Carbonelli4,
  8. Vincenzo Parisi2,
  9. Alfredo A Sadun6,7,
  10. Francesco Bandello3,
  11. Valerio Carelli4,5,
  12. Piero Barboni1,3
  1. 1Studio Oculistico d'Azeglio, Bologna, Italy
  2. 2GB Bietti Foundation, Rome, Italy
  3. 3Scientific Institute San Raffaele, Milan, Italy
  4. 4IRCCS Institute of Neurological Sciences of Bologna, Bellaria Hospital, Bologna, Italy
  5. 5Unit of Neurology, Department of Biomedical and Neuromotor Sciences (DIBINEM), University of Bologna, Bologna, Italy
  6. 6Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
  7. 7Doheny Eye Institute, Los Angeles, CA, USA
  1. Correspondence to Dr Nicole Balducci, Studio Oculistico d'Azeglio, Via d'Azeglio, 5 Bologna 40123, Italy; balduccinicole{at}


Aims To evaluate longitudinal retinal ganglion cell inner plexiform layer (GC-IPL) and macular retinal nerve fibre layer (mRNFL) thickness changes in acute Leber's hereditary optic neuropathy (LHON).

Methods Six eyes of four patients with LHON underwent SD-OCT (optical coherence tomography) at month 1, 3, 6 and 12 after visual loss. In two eyes, the examination was carried out in the presymptomatic stage. The relationship and curves for area under the receiver operator characteristic (AUROC) were generated to assess the ability of each parameter to detect ganglion cell loss.

Results Significant longitudinal thinning of GC-IPL and mRNFL was detected in LHON. GC-IPL thinning was detectable in the deviation map during the presymptomatic stage in the inner ring of the nasal sector and then it progressively extended following a centrifugal and spiral pattern. Similarly, mRNFL thinning began in the inferonasal sector and it progressively extended. No further statistically significant changes were detected after month 3. The highest level of AUROC values at 1 month were detected in the nasal sectors and inferonasal mRNFL thickness reached AUROC value=1. All the parameters were equally able to detect ganglion cell loss from month 2 to 12.

Conclusions The natural history of GC-IPL thinning follows a specific pattern of reduction, reflecting the anatomical course of papillomacular fibres. Month 6 represents the end of GC-IPL loss. GC-IPL and mRNFL thinning is detectable before onset of visual loss. These observations can help future therapeutic approaches for both LHON carriers at high risk of conversion and patients with acute early LHON.

  • Genetics
  • Optic Nerve

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