Abstract
Introduction
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most common subtype of infectious pediatric encephalopathy in Japan. It is sometimes difficult to make an early diagnosis of AESD; excitotoxicity is postulated to be the pathogenesis based on elevated glutamine (Gln) and glutamate (Glu) complex (Glx = Glu + Gln) observed on MR spectroscopy. It is uncertain whether Gln or Glu contributes to the elevated Glx, or whether MR spectroscopy is useful for an early diagnosis.
Methods
Five Japanese patients with AESD (three boys and two girls, 1 year of age) were enrolled in this study. MR spectroscopy was acquired from the frontal white matter (repetition time (TR) of 5000 ms, echo time (TE) of 30 ms) with a 1.5- or 3.0-T scanner. MR spectroscopy was performed four times for two patients, three times for one patient, and two times for two patients. Quantification of Glu and Gln was performed using LCModel.
Results
Glu was elevated in three of four studies on days 1–4 and became normal or low afterward. Gln was normal in three studies on days 1–2, elevated in all seven studies on days 4–12, and became normal or low afterward.
Conclusion
These findings suggest that MR spectroscopy may be useful for an early diagnosis. Acute Glu elevation changes to subacute Gln elevation, suggesting that a disrupted Glu-Gln cycle may play an important role.
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Acknowledgments
This study was supported by the Grants-in-Aid for Scientific Research (B24390258 and C-24591790) from the Japan Society for the Promotion of Science. We thank Daisuke Yoshimaru, RT, at Tokyo Women’s Medical University Yachiyo Medical Center for his technical support to this study.
Ethical standards and patient consent
We declare that all human and animal studies have been approved by Tokyo Women’s Medical University Institutional Review Board and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. We declare that due to the retrospective nature of this study, informed consent was waived.
Conflict of interest
We declare that we have no conflict of interest.
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Takanashi, Ji., Mizuguchi, M., Terai, M. et al. Disrupted glutamate-glutamine cycle in acute encephalopathy with biphasic seizures and late reduced diffusion. Neuroradiology 57, 1163–1168 (2015). https://doi.org/10.1007/s00234-015-1573-x
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DOI: https://doi.org/10.1007/s00234-015-1573-x