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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticleSpine Imaging and Spine Image-Guided Interventions
Open Access

Spinal Cord Gray Matter Atrophy in Amyotrophic Lateral Sclerosis

M.-Ê. Paquin, M.M. El Mendili, C. Gros, S.M. Dupont, J. Cohen-Adad and P.-F. Pradat
American Journal of Neuroradiology January 2018, 39 (1) 184-192; DOI: https://doi.org/10.3174/ajnr.A5427
M.-Ê. Paquin
aFrom the Faculté de Médecine (M.-Ê.P.)
cNeuroPoly Lab, Institute of Biomedical Engineering, Polytechnique Montreal (M.-Ê.P., C.G., S.M.D., J.C.-A.), Montreal, Quebec, Canada
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M.M. El Mendili
dSorbonne Universités (M.M.E.M., P.-F.P.) UPMC Univ Paris 06, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, Paris, France
eDepartment of Neurology (M.M.E.M.), Icahn School of Medicine, Mount Sinai, New York, New York
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C. Gros
cNeuroPoly Lab, Institute of Biomedical Engineering, Polytechnique Montreal (M.-Ê.P., C.G., S.M.D., J.C.-A.), Montreal, Quebec, Canada
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S.M. Dupont
cNeuroPoly Lab, Institute of Biomedical Engineering, Polytechnique Montreal (M.-Ê.P., C.G., S.M.D., J.C.-A.), Montreal, Quebec, Canada
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J. Cohen-Adad
bFunctional Neuroimaging Unit, CRIUGM (J.C.-A.), Université de Montréal, Montreal, Quebec, Canada
cNeuroPoly Lab, Institute of Biomedical Engineering, Polytechnique Montreal (M.-Ê.P., C.G., S.M.D., J.C.-A.), Montreal, Quebec, Canada
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P.-F. Pradat
dSorbonne Universités (M.M.E.M., P.-F.P.) UPMC Univ Paris 06, CNRS, INSERM, Laboratoire d'Imagerie Biomédicale, Paris, France
fDépartement des Maladies du Système Nerveux (P.-F.P.), Centre Référent Maladie Rare SLA, Hôpital de la Pitié-Salpêtrière, Paris, France.
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    Fig 1.

    Processing pipeline for the GM segmentation and computation of the GMCSA.

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    Fig 2.

    GM automatic segmentation and manual delineation patients with ALS. Manual delineation of the GM is displayed with the blue line, automatic probabilistic segmentation is shown in red-to-yellow. Dice coefficient comparing the automatic and manual segmentation is shown on the bottom line.

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    Fig 3.

    GMCSA and SCCSA measured on controls and patients with ALS between the C6–C3 vertebral levels. GMCSA (A) and SCCSA (B) averaged within group and plot against the cervical SC axis. Overall, a stronger intergroup difference can be observed for GMCSA. Asterisk (P ≤ .05) and double asterisk (P ≤ .01) at specific vertebral levels indicate significant differences between patients with ALS and controls according to Student t test P values representing control-to-patient differences in GMCSA and SCCSA for each cervical level between C6 and C3 and across levels.

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    Fig 4.

    Boxplot distribution of GMCSA (A) and SCCSA (B) averaged between the C4–C6 vertebral levels. Each dark point represents an individual value. The median is represented as a thick horizontal line and the interquartile range as a light rectangle. The horizontal bar at both extremities of the whiskers represent the 5th and 95th percentiles. The 2 patients presenting the SOD1 gene are identified in the plot.

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    Fig 5.

    Prediction error on the ALSFRS-R at 1 year, from a leave-one-out cross-validation with regression trees. Results are compared between the regression model including clinical predictors (left distribution plot), clinical predictors + SCCSA (middle distribution plot), and clinical predictors + GMCSA + WM/GMCSA (right distribution plot), where each point represents 1 iteration of the leave-one-out cross-validation. The best value is at 0.

Tables

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    Table 1:

    Demographic data for the 25 patients with ALS and the 22 healthy controls

    CharacteristicsControlsPatients with ALS
    At Baseline1-Year Follow-Up Subgroup
    Number222519
    Sex11 F/11 M6 F/19 M3 F/16 M
    Age at baseline, yr ± SD50.9 ± 13.053.3 ± 10.152.8 ± 9.2
    • Note:—F indicates female; M, male.

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    Table 2:

    Clinical data commonly used as prognostic factors for the 25 patients with ALS

    Clinical DataPatients with ALS
    Baseline1-Year Follow-Up Subgroup
    Body mass index at MRI (± SD)23.7 ± 2.724.0 ± 2.7
    Familial transmission2 SOD1 mutated1 SOD1 mutated
    Site of onset1 bulbar; 18 upper; 6 lower1 bulbar; 12 upper; 6 lower
    Delay from first symptom to diagnosis, mo ± SD9.9 ± 5.610.7 ± 6.0
    • Note:—SOD1 indicates SuperOxide Dismutase 1 gene.

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    Table 3:

    Clinical scoresa

    ALSFRS-RMMT
    At MRIProgression Rate at MRI Per MonthAt 1 Year after MRIAt MRI
    Total score, mean ± SD (/maximum)37.96 ± 6.06 (/48)0.59 ± 0.4536.79 ± 4.84 (/48)114.46 ± 24.36 (/140)
    Arm subscore, mean ± SD (/maximum)5.11 ± 2.20 (/8)0.18 ± 0.145.74 ± 2.10 (/8)53.71 ± 12.25 (/70)
    • Note:—MMT indicates manual muscle testing.

    • ↵a Clinical scores are presented both in total (top row) and for the upper limbs only (arm subscore, bottom row). Presented clinical scores are, from left to right: the revised ALSFRS-R at the time of MRI, ALSFRS-R progression rate at MRI, ALSFRS-R at 1 year after MRI, and MMT at MRI. The progression rate represents the decline of ALSFRS-R score per month between the first onset of symptoms and the MRI.

    • View popup
    Table 4:

    Correlation coefficients between CSA (GM and SC) and clinical scores at baseline and at 1 yeara

    PredictorsAt BaselineAt 1 Year
    ALSFRS-R SubscoreMMT Arm SubscoreALSFRS-R Subscore
    GMCSAR = 0.56R = 0.40R = 0.48
    P = .004bP = .049aP = .035c
    SCCSAR = 0.55R = 0.40R = 0.54
    P = .005bP = .054P = .017c
    • Note:—MMT indicates manual muscle testing.

    • ↵a CSA was averaged across the C4–C6 vertebral levels. Clinical scores included: 1) ALSFRS-R at baseline; 2) MMT subscores at baseline; and 3) ALSFRS-R subscore at 1 year.

    • ↵b Significant (P ≤ .01).

    • ↵c Significant (P ≤ .05).

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    Table 5:

    Results of regression tree predictions to make prediction of total ALSFRS-R score at 1 year after MRI with several clinical and MRI predictorsa

    PredictorsCoefficient of Determination R2 (Best Value = 1.0)Mean Squared Error (Best Value = 0.0)
    Clinical predictors0.5441.87
    Clinical predictors + SCCSA0.7225.39
    Clinical predictors + GMCSA + WMCSA/GMCSA0.7423.77
    • Note:—WMCSA indicates white matter cross-sectional area.

    • ↵a GMCSA, WMCSA, and SCCSA are averaged across the C4–C6 vertebral levels. Clinical predictors include age, body mass index, sex, site of onset, delay between first symptoms and diagnosis, total ALSFRS-R score at baseline, and total ALSFRS-R score progression rate at baseline.

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American Journal of Neuroradiology: 39 (1)
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Cite this article
M.-Ê. Paquin, M.M. El Mendili, C. Gros, S.M. Dupont, J. Cohen-Adad, P.-F. Pradat
Spinal Cord Gray Matter Atrophy in Amyotrophic Lateral Sclerosis
American Journal of Neuroradiology Jan 2018, 39 (1) 184-192; DOI: 10.3174/ajnr.A5427

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Spinal Cord Gray Matter Atrophy in Amyotrophic Lateral Sclerosis
M.-Ê. Paquin, M.M. El Mendili, C. Gros, S.M. Dupont, J. Cohen-Adad, P.-F. Pradat
American Journal of Neuroradiology Jan 2018, 39 (1) 184-192; DOI: 10.3174/ajnr.A5427
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