Share

March 15, 2022; 98 (11) Resident & Fellow Section

Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction

Bing Zhao, Yuan Sun, Yong Qing Zhang, Chuanzhu Yan
First published January 11, 2022, DOI: https://doi.org/10.1212/WNL.0000000000013320
Full PDF
Citation
Permissions

Make Comment

See Comments

Downloads
956

Share

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.

Figure
Figure Spinal Cord MRI

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

Table

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

References

  1. 1.↵
    1. Alves C,
    2. Goldstein A,
    3. Teixeira SR, et al.
    Involvement of the spinal cord in primary mitochondrial disorders: a neuroimaging mimicker of inflammation and ischemia in children. AJNR Am J Neuroradiol 2021;42(2):389-396.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Zalewski NL,
    2. Rabinstein AA,
    3. Krecke KN, et al.
    Characteristics of spontaneous spinal cord infarction and proposed diagnostic criteria. JAMA Neurol 2019;76(1):56-63.
    OpenUrlPubMed

Letters: Rapid online correspondence

  • Author Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction
    • Bing Zhao, neurologist, Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, 266035, C
    • Yuan Sun, neurologist, Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, 266035, C
    • Yongqing Zhang, neurologist, Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, 266035, C
    • Chuanzhu Yan, neurologist, Department of Neurology, Qilu Hospital (Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao, Shandong, 266035, C
    Submitted April 23, 2022
  • Reader Response: Teaching NeuroImage: Leber Hereditary Optic Neuropathy With Longitudinal Spinal Cord Lesion Mimicking Spinal Cord Infarction
    • Neha K Irani, Consultant Neurologist and Neuro-opthalmologist, Royal Perth Hospital, Fiona Stanley Hospital, Joondalup Health Campus in Perth Western Australia
    • Kevin D O'Connor, Consultant Neurologist and Neuroimmunologist, Royal Perth Hospital, Joondalup Health Campus in Perth Western Australia, Australia
    • Jane H Lock, Consultant Neuro-ophthalmologist and Strabismus Surgeon, Royal Perth Hospital, Sir Charles Gairdner Hospital, Perth Children's Hospital, Northern Eye Surgery in Perth, Western Australia
    Submitted March 27, 2022
Comment

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.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
Advertisement

Related Articles

Topics Discussed

Alert Me