Article Figures & Data
Figures
- 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.
- 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.
- 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.
- Fig 4.
Linear regression graphs illustrating the relationship between ICARS score and cerebellar (A) and brain stem (B) volume for both subtypes.
Tables
- 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) 10:7 11:6 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 length 68.7 ± 3.5 (58–73) 21.6 ± 0.7 (21–22) N.A. ICARS 34.7 ± 10.8 (17–60) 30.6 ± 10.9 (15–46) N.S. I) Posture and gait 11.0 ± 7.8 (2–29) 9.0 ± 5.2 (3–23) P = .043c II) Kinetic functions 13.0 ± 4.8 (2–24) 13.0 ± 6.1 (5–32) N.S. III) Speech 3.0 ± 1.5 (0–5) 3.0 ± 1.6 (0–6) N.S. IV) Oculomotor 4.0 ± 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.
-
- Table 2:
Correlation of disease severity and CNS volumes with disease duration and CAG repeat length
Disease Duration CAG Repeat Length SCA3 SCA6 SCA3 SCA6 ICARS score ρ = 0.598 ρ = 0.632 N.S. N.S. P = .011a P = .006b Cerebellar volume ρ = −0.506 ρ = −0.623 N.S. N.S. P = .038a P = .017a Brain stem ρ = −0.51 N.S. N.S. N.S. volume P = .036a a Significant with P < .05.
b Highly significant with P < .01.
SCA3 SCA6 Control Subjects Cerebellum volume (ml) Mean ± SD 119.0 ± 17.2 98.1 ± 14.4 132.6 ± 11.3 P 004ab 006 (SCA3)bc, <.001 (SCA6)b Brain stem volume (ml) Mean ± SD 21.5 ± 3.8 27.3 ± 2.5 28.8 ± 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.
- Table 4:
Linear regression between ICARS total score and CNS volumes: simple linear regression and linear regression including disease duration as a covariate
SCA Linear Regression Type Coefficient B (95% CI) R2 P Cerebellar volume 3 Simple −0.341 (−0.631 to −0.543) 0.29 .024a Including DD −0.19 (−0.50 to −0.11) 0.46 .20 6 Simple −0.407 (−0.758 to −0.057) 0.30 .026a Including DD −0.33 (−0.76 to −0.14) 0.31 .15 Brain stem volume 3 Simple −2.01 (−3.141 to −0.879) 0.49 .002b Including DD −1.51 (−2.7 to −0.31) 0.60 .02a 6 Simple −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.
- 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 Type SCA Cerebellum Brain Stem Simple Ps.R2P Including DD Ps.R2P Simple Ps.R2P Including DD Ps.R2P I) Posture and gait 3 0.35 0.018a 0.45 0.10 0.23 0.025a 0.41 0.11 6 0.41 0.010a 0.43 0.062 0.23 0.044a 0.36 0.10 II) Kinetic functions 3 0.05 0.32 0.065 0.72 0.14 0.14 0.14 0.19 6 0.21 0.053 0.27 0.29 0.29 0.025a 0.40 0.048a III) Speech functions 3 0.08 0.26 0.49 0.62 0.03 0.52 0.51 0.32 6 0.30 0.036a 0.43 0.11 0.07 0.27 0.41 0.521 IV) Oculomotor functions 3 0.46 0.007b 0.57 0.048a 0.30 0.03a 0.49 0.18 6 0.04 0.37 0.05 0.42 0.17 0.10 0.24 0.046a a Significant with P < .05.
b Significance with P < .01.
- 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
Model R2 of the Model Included Predictor B OR 95% CI of B P Value 1 0.489 Intercept 77.9 Brain stem volume −2.01 −0.7 −3.14 −0.88 0.02 - 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
Model R2 of the Model Included Predictors B OR 95% CI of B P Value 1 0.386 Intercept, 105.03 Brain stem volume −2.73 −0.62 −4.63 −0.84 .008 2 0.553 Intercept, 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
In this issue
Jump to section
Related Articles
Cited By...
- Transcriptomic changes across subregions of the primate cerebellum support the evolution of uniquely human behaviors
- Neuroradiological findings in GAA-FGF14 ataxia (SCA27B): more than cerebellar atrophy
- Credit assignment in movement-dependent reinforcement learning
- Macro- and Microstructural Changes in Patients with Spinocerebellar Ataxia Type 6: Assessment of Phylogenetic Subdivisions of the Cerebellum and the Brain Stem