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Metabolic characteristics of cortical malformations causing epilepsy

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Abstract

Purpose

Cortical malformations (CMs) are increasingly recognized as the epileptogenic substrate in patients with medically refractory neocortical epilepsy (NE). The aim of this study was to test the hypotheses that: 1. CMs are metabolically heterogeneous. 2. The structurally normal appearing perilesional zone is characterized by similar metabolic abnormalities as the CM.

Methods

Magnetic resonance spectroscopic imaging (MRSI) in combination with tissue segmentation was performed on eight patients with NE and CMs and 19 agematched controls. In controls, NAA, Cr, Cho,NAA/Cr and NAA/Cho of all voxels of a given lobe were expressed as a function of white matter content and thresholds for pathological values determined by calculating the 95% prediction intervals. These thresholds were used to identify metabolically abnormal voxels within the CM and in the perilesional zone.

Results

30% of all voxels in the CMs were abnormal, most frequently because of decreases of NAA or increases of Cho. Abnormal voxels tended to form metabolically heterogeneous clusters interspersed in metabolically normal regions. Furthermore, 15% of all voxels in the perilesional zone were abnormal, the most frequent being decreases of NAA and Cr.

Conclusion

In CMs metabolically normal regions are interspersed with metabolically heterogeneous abnormal regions. Metabolic abnormalities in the perilesional zone share several characteristics of CMs and might therefore represent areas with microscopic malformations and/or intrinsic epileptogenicity.

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Authors and Affiliations

  1. Dept. of Veterans Affairs (DVA), Medical Center, Magnetic Resonance Spectroscopy Unit, San Francisco (CA), USA

    S. G. Mueller MD, N. Cashdollar BA, D. L. Flenniken BSc.,  P. Vermathen, G. B. Matson PhD & M. W. Weiner MD

  2. Dept. of Radiology, University of California, San Francisco (CA), USA

    S. G. Mueller MD & M. W. Weiner MD

  3. Pacific Epilepsy Program, California Pacific Medical Center, 2100 Webster Street, Suite 115, San Francisco (CA) 94115, USA

    K. D. Laxer MD & J. A. Barakos MD

  4. Dept. of Pharmaceutical Chemistry, University of California, San Francisco (CA), USA

    G. B. Matson PhD

Authors
  1. S. G. Mueller MD
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  2. K. D. Laxer MD
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  3. J. A. Barakos MD
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  4. N. Cashdollar BA
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  5. D. L. Flenniken BSc.
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  6. P. Vermathen
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  7. G. B. Matson PhD
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  8. M. W. Weiner MD
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Corresponding author

Correspondence to K. D. Laxer MD.

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Mueller, S.G., Laxer, K.D., Barakos, J.A. et al. Metabolic characteristics of cortical malformations causing epilepsy. J Neurol 252, 1082–1092 (2005). https://doi.org/10.1007/s00415-005-0819-7

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  • DOI: https://doi.org/10.1007/s00415-005-0819-7

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