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Research ArticleBrain

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

L. Eichler, B. Bellenberg, H.K. Hahn, O. Köster, L. Schöls and C. Lukas
American Journal of Neuroradiology May 2011, 32 (5) 890-897; DOI: https://doi.org/10.3174/ajnr.A2387
L. Eichler
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B. Bellenberg
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H.K. Hahn
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O. Köster
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L. Schöls
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C. Lukas
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    Fig 1.

    Example of quantitative brain stem volumetry. Upper left: 3D segmentation of the brain stem. Lower left and middle: brain stem segmentation in orthogonal views. The borders of the brain stem are defined according to the method proposed by Luft et al.18 Right: midsagittal view showing anatomic landmarks.

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    Fig 2.

    Effect of the correction for natural aging on the cerebellum volume. Lines represent the linear regression within the control group and SCA subgroups of age. Cerebellar volumes before (A) and after (B) age correction; age dependence is fully compensated in the control group and reduced in the patient groups.

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    Fig 3.

    Sagittal T1-weighted images of the brain stem in patients with SCA and in a healthy control subject. Brain stem atrophy is clearly visible in SCA3 and to a lesser extent in SCA6. Note the marked atrophy of the cerebellum in SCA6.

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    Fig 4.

    Linear regression graphs illustrating the relationship between ICARS score and cerebellar (A) and brain stem (B) volume for both subtypes.

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    Table 1:

    Demographic and clinical characteristics of patients with SCA3 and SCA6 (mean ± SD, range)

    SCA3 (n = 17)SCA6 (n = 17)Difference of Mean SCA3 vs SCA6a
    Age (yr)49.8 ± 11.5 (27–67)65.8 ± 6.5 (52–75)P = .008b
    Sex (M/F)10:711:6
    Disease duration (yr)6.4 ± 4.8 (0–18)8.9 ± 5.7 (1–23)N.S.
    Age at onset (yr)43.4 ± 12.3 (19–62)55.6 ± 8.7 (41–67)P = .004b
    CAG repeat length68.7 ± 3.5 (58–73)21.6 ± 0.7 (21–22)N.A.
    ICARS34.7 ± 10.8 (17–60)30.6 ± 10.9 (15–46)N.S.
        I) Posture and gait11.0 ± 7.8 (2–29)9.0 ± 5.2 (3–23)P = .043c
        II) Kinetic functions13.0 ± 4.8 (2–24)13.0 ± 6.1 (5–32)N.S.
        III) Speech3.0 ± 1.5 (0–5)3.0 ± 1.6 (0–6)N.S.
        IV) Oculomotor4.0 ± 1.5 (0–6)5.0 ± 1.2 (2–6)N.S.
    • a Statistically significant differences between the SCA3 and SCA6 patient groups under consideration of the disease duration; analysis of variance (GLM model) with disease duration as a covariable.

    • b Significant with P< .01.

    • c Significant with P < .05.

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    Table 2:

    Correlation of disease severity and CNS volumes with disease duration and CAG repeat length

    Disease DurationCAG Repeat Length
    SCA3SCA6SCA3SCA6
    ICARS scoreρ = 0.598ρ = 0.632N.S.N.S.
    P = .011aP = .006b
    Cerebellar volumeρ = −0.506ρ = −0.623N.S.N.S.
    P = .038aP = .017a
    Brain stemρ = −0.51N.S.N.S.N.S.
    volumeP = .036a
    • a Significant with P < .05.

    • b Highly significant with P < .01.

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    Table 3:

    Cerebellum and brain stem volumes of patients and control subjects

    SCA3SCA6Control Subjects
    Cerebellum volume (ml)
    Mean ± SD119.0 ± 17.298.1 ± 14.4132.6 ± 11.3
    P004ab006 (SCA3)bc, <.001 (SCA6)b
    Brain stem volume (ml)
    Mean ± SD21.5 ± 3.827.3 ± 2.528.8 ± 2.0
    P<.001ab<.001 (SCA3),bc .027 (SCA6)d
    • a Group differences between SCA3 and SCA6 assessed by variance analysis with disease duration as a covariate (GLM model).

    • b Highly significant with P < .01.

    • c Group differences between control groups and patient groups assessed by Student ttests.

    • d Significant with P < .05.

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    Table 4:

    Linear regression between ICARS total score and CNS volumes: simple linear regression and linear regression including disease duration as a covariate

    SCALinear Regression TypeCoefficient B (95% CI)R2P
    Cerebellar volume3Simple−0.341 (−0.631 to −0.543)0.29.024a
    Including DD−0.19 (−0.50 to −0.11)0.46.20
    6Simple−0.407 (−0.758 to −0.057)0.30.026a
    Including DD−0.33 (−0.76 to −0.14)0.31.15
    Brain stem volume3Simple−2.01 (−3.141 to −0.879)0.49.002b
    Including DD−1.51 (−2.7 to −0.31)0.60.02a
    6Simple−2.732 (−4.626 to −0.837)0.39.008b
    Including DD−2.37 (−4.3 to −0.44)0.46.02a
    • a Significant with P < .05

    • b Significant with P < .01.

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    Table 5:

    Ordinal regression between ICARS subscore groups and CNS volumes: simple ordinal regression and ordinal regression including disease duration (DD) as a covariate

    Regression TypeSCACerebellumBrain Stem
    Simple Ps.R2PIncluding DD Ps.R2PSimple Ps.R2PIncluding DD Ps.R2P
    I) Posture and gait30.35 0.018a0.45 0.100.23 0.025a0.41 0.11
    60.41 0.010a0.43 0.0620.23 0.044a0.36 0.10
    II) Kinetic functions30.05 0.320.065 0.720.14 0.140.14 0.19
    60.21 0.0530.27 0.290.29 0.025a0.40 0.048a
    III) Speech functions30.08 0.260.49 0.620.03 0.520.51 0.32
    60.30 0.036a0.43 0.110.07 0.270.41 0.521
    IV) Oculomotor functions30.46 0.007b0.57 0.048a0.30 0.03a0.49 0.18
    60.04 0.370.05 0.420.17 0.100.24 0.046a
    • a Significant with P < .05.

    • b Significance with P < .01.

    • View popup
    Table 6:

    Results of stepwise multivariate regression for SCA3 with ICARS score as the dependent variable and brain stem volume and cerebellum volume as independent variables

    ModelR2 of the ModelIncluded PredictorBOR95% CI of BP Value
    10.489Intercept77.9
    Brain stem volume−2.01−0.7−3.14 −0.880.02
    • View popup
    Table 7:

    Results of stepwise multivariate regression for SCA6 with ICARS score as the dependent variable and brain stem and cerebellum volumes as independent variables

    ModelR2 of the ModelIncluded PredictorsBOR95% CI of BP Value
    10.386Intercept,105.03
    Brain stem volume−2.73−0.62−4.63 −0.84.008
    20.553Intercept,124.67
    Brain stem volume,−2.32−0.53−4.04 −0.58.012
    Cerebellar volume−0.32−0.42−0.61 −0.02.039
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American Journal of Neuroradiology: 32 (5)
American Journal of Neuroradiology
Vol. 32, Issue 5
1 May 2011
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Cite this article
L. Eichler, B. Bellenberg, H.K. Hahn, O. Köster, L. Schöls, C. Lukas
Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status
American Journal of Neuroradiology May 2011, 32 (5) 890-897; DOI: 10.3174/ajnr.A2387

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Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status
L. Eichler, B. Bellenberg, H.K. Hahn, O. Köster, L. Schöls, C. Lukas
American Journal of Neuroradiology May 2011, 32 (5) 890-897; DOI: 10.3174/ajnr.A2387
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