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Pediatric Radiology

, Volume 47, Issue 1, pp 126–127 | Cite as

Reply regarding lesional perfusion abnormalities on arterial spin labeling in Leigh disease

  • Matthew T. WhiteheadEmail author
  • Bonmyong Lee
  • Andrea Gropman
Letter to the Editor
  • 92 Downloads

Dear Editor,

We thank Drs. Finsterer and Zarrouk-Mahjoub [1] for their interest in our article [2], and we welcome the opportunity to provide clarification. The authors raised questions and concerns as to diagnostic criteria, associations between genetic defects and perfusion changes, and external factors that may alter brain blood flow such as seizures, strokes, anesthetics and other medications [1]. Herein, we contribute to the comments regarding our recent report and discuss the context of our findings.

It is well known that Leigh disease can be a phenotype of several different genetic defects [3]. While many specific causitive gene mutations have been firmly established, some are of less certain pathogenicity, ranging to include “variants of unknown clinical significance.” We retrospectively identified and analyzed 15 brain MR exams from 8 subjects with clinically, radiographically and genetically confirmed Leigh disease [2]. All patients met clinical dignostic criteria for Leigh...

Keywords

Arterial Spin Label Brain Perfusion Perfusion Change Encephalomalacia Leigh Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Compliance with ethical standards

Conflicts of interest

None

References

  1. 1.
    Finsterer J, Zarrouk-Mahjoub S (2016) Lesional perfusion abnormalities on arterial spin-labeling in Leigh disease. Pediatr Radiol. doi: 10.1007/s00247-016-3718-4 PubMedGoogle Scholar
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    Whitehead MT, Lee B, Gropman A (2016) Lesional perfusion abnormalities in Leigh disease demonstrated by arterial spin labeling correlate with disease activity. Pediatr Radiol 46:1309–1316CrossRefPubMedGoogle Scholar
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    Finsterer J (2008) Leigh and Leigh-like syndrome in children and adults. Pediatr Neurol 39:223–235CrossRefPubMedGoogle Scholar
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    Harreld JH, Helton KJ, Kaddoum RN et al (2013) The effects of propofol on cerebral perfusion MRI in children. Neuroradiology 55:1049–1056CrossRefPubMedPubMedCentralGoogle Scholar
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    Song XX, Yu BW (2015) Anesthetic effects of propofol in the healthy human brain: functional imaging evidence. J Anesth 29:279–288CrossRefPubMedGoogle Scholar
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    Finsterer J (2009) Management of mitochondrial stroke-like-episodes. Eur J Neurol 16:1178–1184CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Matthew T. Whitehead
    • 1
    • 2
    Email author
  • Bonmyong Lee
    • 3
  • Andrea Gropman
    • 2
    • 4
  1. 1.Department of RadiologyChildren’s National Medical CenterWashingtonUSA
  2. 2.The George Washington University School of MedicineWashingtonUSA
  3. 3.Department of RadiologyThe Johns Hopkins Medical InstituteBaltimoreUSA
  4. 4.Department of NeurologyChildren’s National Medical CenterWashingtonUSA

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