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Research ArticlePediatric Neuroimaging

β-Hydroxybutyrate Detection with Proton MR Spectroscopy in Children with Drug-Resistant Epilepsy on the Ketogenic Diet

J.N. Wright, R.P. Saneto and S.D. Friedman
American Journal of Neuroradiology July 2018, 39 (7) 1336-1340; DOI: https://doi.org/10.3174/ajnr.A5648
J.N. Wright
aFrom the Department of Radiology (J.N.W., S.D.F.)
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R.P. Saneto
bDepartment of Neurology, Division of Pediatric Neurology (R.P.S.), University of Washington and Seattle Children's Hospital, Seattle, Washington.
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S.D. Friedman
aFrom the Department of Radiology (J.N.W., S.D.F.)
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    Fig 1.

    Voxel-of-interest (inner 8 × 8 matrix) and masked region (7 × 7 matrix) that retained spectra based on SNR, NAA, and adequate Cramér-Rao bounds. Across the brain (spectra labeled A–F, corresponding to labeled voxels in image at the upper right), 2 peaks comprising lactate and BHB are shown at 1.33 and 1.2 ppm, respectively, with lactate more prominent in voxels containing CSF in this patient with mitochondrial cytopathy. At the lower right, the composite spectrum from the masked region is shown, with the LCModel fit overlaid.

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    Fig 2.

    Scatterplot of blood serum BHB concentrations (milligrams/deciliter) versus brain BHB/NAA and BHB/Cr ratios demonstrates the strong associations between measures.

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    Table 1:

    Patient clinical data including underlying epilepsy syndrome, seizure type, and antiepileptic medications

    PatientAge (yr)Epilepsy Syndrome21–23Seizure TypeMedications
    11EE (Unclassified)TLEV
    22Metabolic (Mito)TSZNS/LTG/LEV
    27Metabolic (Mito)TS/GTCZNS/LTG/LEV/RFM
    28Metabolic (Mito)TS/MY/GTCZNS/LTG/LEV/RFM
    39MAEMY/AT/GTCVPA/CLB/RFM
    47Metabolic (Mito)MY/RFLTPM
    410Metabolic (Mito)MY/RFLTPM/ZNS
    57EE (MCD)T/AT/TSVGB/LTG
    62Structural (MCD)FocalZNS/TPM
    71Structural (Aicardi)ESVGB
    86Genetic (SCN1A)MY/ATLEV/ETX/CLN
    96Metabolic (Mito)MY/ATLEV/LTG/ETX/CLN
    106MAET/MY/TSLTG
    110.5Genetic (ATP1A3)Focal/SELEV
    111Genetic (ATP1A3)SUB/MYLTG/CLB
    126EE (unclassified)AT/T/GTCCLB
    131EE (unclassified)ESVGB
    142Metabolic (Mito)ESTPM
    154EE (LGS)AT/T/AALTG/LEV/ZNS
    161EE (Unclassified)ESZNS/LEV
    175Genetic (DEAF 1)T/AT/GTCLEV
    181Metabolic (Mito)ESZNS
    • Note:—“Epilepsy Syndrome”: EE indicates epileptic encephalopathy; Mito, primary mitochondrial disease; MAE, myoclonic astatic epilepsy; MCD, malformation of cortical development; LGS, Lennox-Gastaut Syndrome; “Seizure Type”: T, tonic; TS, tonic spasms; GTC, generalized tonic-clonic; MY, myoclonic; AT, atonic; RFL, reflexive seizure; ES, epileptic spasms; SE, status epilepticus; AA, atypical absence; SUB, subclinical seizures; “Medications”: LEV, levetiracetam; ZNS, zonisamide; LTG, lamotrigine; RFM, rufinamide; CLB, clobazam; TPM, topiramate; VGB, vigabatrin; ETX, ethosuximide; CLN, clonazepam; VPA, valproic acid.

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    Table 2:

    Partial correlations of blood BHB concentration (mg/dL) and brain metabolite ratios derived from 1H-MRS, using patient age in months as a covariate

    GroupBHB/CrBHB/ChoBHB/NAABHB/LacCho/CrNAA/CrNAA/ChoLac/NAALac/Cr
    Lag ≤14 d (n = 13)
        R.871.898.886.507−.287−.645−.540.376.253
        Significance.000.000.000.092.367.024.070.228.428
    Lag ≤30 d (n = 23)
        R.803.814.807.538−.165−.553−.443.421.334
        Significance.000.000.000.010.463.008.039.051.128
    Lag ≤120 d (n = 31)
        R.790.802.781.484−.312−.658−.515.523.423
        Significance.000.000.000.008.099.000.004.004.022
    All (N = 49)
        R.579.588.584.393−.170−.387−.312.290.162
        Significance.000.000.000.006.254.007.033.048.278
    • Note:—Lac indicates lactate.

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American Journal of Neuroradiology: 39 (7)
American Journal of Neuroradiology
Vol. 39, Issue 7
1 Jul 2018
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J.N. Wright, R.P. Saneto, S.D. Friedman
β-Hydroxybutyrate Detection with Proton MR Spectroscopy in Children with Drug-Resistant Epilepsy on the Ketogenic Diet
American Journal of Neuroradiology Jul 2018, 39 (7) 1336-1340; DOI: 10.3174/ajnr.A5648

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β-Hydroxybutyrate Detection with Proton MR Spectroscopy in Children with Drug-Resistant Epilepsy on the Ketogenic Diet
J.N. Wright, R.P. Saneto, S.D. Friedman
American Journal of Neuroradiology Jul 2018, 39 (7) 1336-1340; DOI: 10.3174/ajnr.A5648
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