A 15-year-old boy with a maternal family history of “optic neuropathy” presented with acute flaccid paralysis in both upper limbs. Cervical MRI demonstrated longitudinally extensive T2 lesions, with significant enhancement of the anterior gray matter. Typical “snake-eye” appearance was observed in the follow-up MRI 1 month later (Figure). Analysis of blood mt-DNA revealed a homoplasmic m.14495T>C mutation, confirming the diagnosis of Leber hereditary optic neuropathy (LHON). Idebenone 900 mg/d was given to him, and he recovered completely 3 months later.
MRI in the acute phase demonstrated a longitudinally extensive T2 hyperintensity lesion extending from the lower medulla oblongata to the thoracic level (A, B, arrows) and a linear strip corresponding to the anterior gray matter after gadolinium enhancement (C, D, arrows). MRI in the recovery phase demonstrated the pencil-like T2 hyperintensity on sagittal views corresponding with the “snake-eye” appearance on axial planes (E, F, arrows), without any enhancement (G, H).
Spinal cord involvement was rare in LHON.1 The enhancement pattern in this case mimicked spinal cord infarction rather than demyelinating diseases.2
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
Appendix Authors
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Teaching slides links.lww.com/WNL/B757
- © 2022 American Academy of Neurology
Disputes & Debates: Rapid online correspondence
- Author Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction
- Reader Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction
REQUIREMENTS
If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.