Article Figures & Data
Figures
- Fig 1.
A, EJ diagnosed at the age of 4 years 6 months. MRI performed at the age of 10 years, 10 months. B, LI, 2 years, 9 months. Note the tigroid appearance of the WM on T2-weighted sequences.
- Fig 2.
LI, 2 years 7 months. A, Involvement of the posterior limb of the internal capsules (solid arrows). B, Involvement of the corticospinal tracts in the brain stem (open arrows) on axial T2-weighted sequences.
- Fig 3.
MRI manifestations in LI and EJ patients (number of patients with each abnormality in parentheses) as a function of age of onset. Dark gray bars indicate GM abnormalities; light gray bars, WM abnormalities. Arrows indicate the mean age for each category. The mean score is noted at the center of each bar.
- Fig 4.
LI. Axial T2-weighted sequence, genu of CC (arrows). A, Normal (3 years 1 month). B, Central involvement (3 years 4 months). C, Diffuse involvement (4 years). EJ. Axial FLAIR sequence, splenium of the CC (arrows). D, Diffuse hyperintensity (3 years 6 months), then curvilinear hyperintensity (4 years 6 months) (E).
- Fig 5.
LI (3 years 11 months). A, Axial T2-weighted image shows the absence of frank T2 hyperintensity in the posterior limb of the internal capsule (arrow). B, Axial T1-weighted image shows loss of differentiation between the capsule and thalamus (arrow). C, Age-matched control. Image shows good differentiation between the capsule and thalamus (arrow).
- Fig 6.
Axial T2-weighted sequences. A, EJ (5 years of age). Note frank T2 hypointensity and atrophy of the thalami (arrows). B, Age-matched control. Note the normal appearance of the thalami (arrows).
- Fig 7.
A, Axial T2-weighted sequence. LI (3 years 7 months of age). Note atrophy of the caudate nuclei and thalami and lack of significant decrease in the volume of the putamina. B, Age-matched control. Note the normal appearance of the caudate nuclei (arrows) and thalami. C, Correlation between the length of the thalami (T) and the caudate nuclei (CN) in EJ patients (r = 0.8, P = .0002).
Tables
- Table 1:
Mean values of CC thicknesses (EJ) compared with controls at the same age, with corresponding P values
Localization Values (mean ± SE) (mm) P Value EJ Controls Genu of CC 7.6 ± 1 9.9 ± 0.4 .13 Mild body of CC 3.5 ± 0,4 5.8 ± 0.2 .33 Splenium of CC 6 ± 0.7 8.3 ± 0.2 <.05 -
Note:—SE indicates standard error.
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- Table 2:
Mean values of measures of frontal and occipital horns and third ventricle compared with controls at the same age, with corresponding P values (LI)
Measures Values (mean ± SE) (mm) P Value LI Controls R frontal horn 5.4 ± 0.8 3.7 ± 0.2 <.0001 L frontal horn 5.4 ± 0.8 3.7 ± 0.2 <.0001 R occipital horn 8.5 ± 0.8 7 ± 0.3 <.0001 L occipital horn 8.5 ± 0.7 7.4 ± 0.3 <.0001 Third ventricle 6 ± 0.6 4.2 ± 0.3 <.0001 -
Note:—R indicates right; L, left.
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- Table 3:
Mean values of measures of frontal and occipital horns and third ventricle, compared with controls at the same age, with corresponding P values (EJ)
Measures EJ Controls P Values R frontal horn 4.4 ± 0.8 3.2 ± 0.2 .21 L frontal horn 4.5 ± 0.6 2.7 ± 0.1 .15 R occipital horn 9 ± 0.9 7.9 ± 0.4 .15 L occipital horn 8,7 ± 0.8 8.2 ± 0.4 .8 Third ventricle 5.1 ± 1.8 3.4 ± 0.3 .73 -
Note:—R indicates right; L, left.
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- Table 4:
Mean values of measures of thalami and caudate nuclei (length, width, curve, and surface) compared with controls at the same age, with corresponding P values
Measures Values (mean ± SE) (mm) P Values EJ Controls Length of R thalamus 27.3 ± 0.7 32.6 ± 0.4 <.0001 Length of L thalamus 27.7 ± 0.6 32.4 ± 0.4 <.0001 Width of R thalamus 11.3 ± 0.3 15.2 ± 0.3 <.0001 Width of L thalamus 12.3 ± 0.7 15.1 ± 0.2 <.0001 Curve of R thalamus 68.3 ± 1.4 82.2 ± 0.7 <.0001 Curve of L thalamus 68.3 ± 1.4 83.6 ± 0.4 <.0001 Surface of R thalamus 263.6 ± 7.6 444.7 ± 2.8 <.0001 Surface of L thalamus 267.3 ± 5.5 442.5 ± 3 <.0001 Length of R caudate nucleus 15.3 ± 0.3 18.4 ± 0.2 <.0001 Length of L caudate nucleus 15.6 ± 0.3 18.6 ± 0.3 <.0001 Width of R caudate nucleus 7 ± 0.5 8.3 ± 0.2 <.0001 Width of L caudate nucleus 6.6 ± 0.6 8.1 ± 0.2 <.0001 Curve of R caudate nucleus 38 ± 1.3 44.5 ± 0.4 <.0001 Curve of L caudate nucleus 38 ± 1.3 44.9 ± 0.3 <.0001 Surface of R caudate nucleus 88.3 ± 2.9 120.9 ± 2 <.0001 Surface of L caudate nucleus 88.3 ± 2.9 117.9 ± 1.8 <.0001 -
Note:—R indicates right; L, left.
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Patient Sex Score Age (yr/mo) (MRI) Time to Onset of Clinical Symptoms (yr) NAA Decreased Cho Increased Lactate Resonance Myo-Inositol Increased LI 1 F 19 2.12 1.02 2 M 19 3.08 1.66 1 1 1 0 3 M 14 2.66 1.66 1 1 0 0 4 M 24 2.42 1.6 0 0 0 0 5 M 21 2.83 1.33 1 1 1 1 6 M 12 3.33 1.83 0 0 0 0 17 4 3.5 1 1 1 1 7 M 23 2.75 1.09 1 1 1 1 8 M 17 3.9 2.5 1 1 0 0 EJ 9 F 24 4.5 0.67 0 0 1 1 10 F 25 6.16 3 0 0 0 0 11 F 18 5.4 1.25 1 1 0 1 -
↵a 0 = absent, 1 = present.
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