MRI of the Optic Nerves and Chiasm in Patients With Leber Hereditary Optic Neuropathy

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Title MRI of the Optic Nerves and Chiasm in Patients With Leber Hereditary Optic Neuropathy
Creator Christelle Blanc, MD; Françoise Heran, MD; Christophe Habas, MD; Yannick Bejot, MD, PHD; José Sahel, MD, PHD; Catherine Vignal-Clermont, MD
Affiliation Department of Neurology (CB, YB), CHU de DIJON, Dijon, France; Department of Neuro-radiology (FH), Fondation Ophtalmologique Adolphe de Rothschild, Paris, France; Department of Neuro-radiology (CH), Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, France; Clinical Center of Investigations (JS, CV-C), Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, France; and Department of Neuro-Ophthalmology (JS, CV-C), Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
Abstract The aim of this study was to characterize brain and orbital MRI features of patients with Leber hereditary optic neuropathy (LHON), with particular attention to the optic nerves and chiasm. We studied a patient cohort with genetically confirmed LHON followed at 2 ophthalmologic hospitals in France between 2013 and 2015. High-resolution brain and orbital MRI studies were analyzed for each patient during the first 12 months after the onset of visual loss was analyzed. Our study included 20 men and 8 women with a mean age of 38.3 years at diagnosis, and all had genetic mutations for LHON. Nineteen patients (67.9%) had T2 hyperintensity in the posterior portion of both optic nerves and in the optic chiasm, and enlargement of the chiasm was found in 16 patients (59.3%). No enhancement of the optic nerves or chiasm was detected. The T2 hyperintensity lesions were not associated with the time between symptom onset and obtaining MRI, the mutation type, or sex of the patient. Nonspecific T2 white matter lesions were found in MRI of 6 patients, but without the characteristics of those found in patients with multiple sclerosis. Involvement of the posterior portions of the optic nerves has been described previously in case reports of patients with LHON. Our results support this observation with neuroimaging performed within 1 year of onset of visual loss. Enlargement of the optic chiasm also may occur in patients with LHON. The pathophysiology of the MRI changes is not yet understood.
Subject Adult; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging / methods; Male; Middle Aged; Optic Atrophy, Hereditary, Leber / diagnosis; Optic Chiasm / pathology; Optic Nerve / pathology; Reproducibility of Results; Retrospective Studies
OCR Text Show
Date 2018-12
Language eng
Format application/pdf
Type Text
Publication Type Journal Article
Source Journal of Neuro-Ophthalmology, December 2018, Volume 38, Issue 4
Collection Neuro-Ophthalmology Virtual Education Library: Journal of Neuro-Ophthalmology Archives: https://novel.utah.edu/jno/
Publisher Lippincott, Williams & Wilkins
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management © North American Neuro-Ophthalmology Society
ARK ark:/87278/s64z08rk
Setname ehsl_novel_jno
ID 1500770
Reference URL https://collections.lib.utah.edu/ark:/87278/s64z08rk
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