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February 15, 2022; 98 (7) Resident & Fellow Section

Teaching NeuroImage: Fasting EEG in Glucose Transporter-1 Deficiency Syndrome

Hassan Imtiaz, Afra Can, Daniela Tapos, Amanda Weber
First published December 3, 2021, DOI: https://doi.org/10.1212/WNL.0000000000013143
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A 12-year-old boy presented for the evaluation of chorea induced by fasting and exercise. EEG, brain MRI, spectroscopy, and metabolic and microarray evaluations were unremarkable. Fasting EEG showed diffuse spike-wave discharges with postprandial normalization (Figure); chorea was not captured. Genetic testing confirmed a pathogenic SLC2A1 variation consistent with glucose transporter-1 (GLUT-1) deficiency syndrome, which is uniquely responsive to ketogenic diet. EEG findings in this syndrome may include multifocal or generalized spikes, with postprandial improvement.1,2 This case suggests a supportive role for fasting EEG in the diagnosis of glucose transporter-1 deficiency syndrome, but the sensitivity and specificity remain unclear.

Figure
Figure EEG During Fasting (A and B) Showing Diffuse, Poorly Formed 3–4 Hz Spike-Wave Discharges

EEG after meal (C and D) showing background normalization. This EEG is shown on a 15-second epoch with a sensitivity of 7 μV in a longitudinal bipolar montage, left over right.

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.

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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/B689

References

  1. 1.↵
    1. Koch H,
    2. Weber YG
    . The glucose transporter type 1 (Glut1) syndromes. Epilepsy Behav. 2018;91:90-93.
    OpenUrlCrossRef
  2. 2.↵
    1. von Moers A,
    2. Brockmann K,
    3. Wang D, et al.
    EEG features of Glut-1 deficiency syndrome. Epilepsia. 2002;43(8):41-45.
    OpenUrlCrossRefPubMed

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