Fig.8.3. Late-infantile GLD in a girl aged 4years.There are extensive, symmetrical white matter abnormalities, predominantly involving the posterior part of the cerebral hemi-spheres.There is a subtle pattern of radiating stripes.The pos terior limb of the internal capsule and corticospinal tracts in the brain stem are affected.The cerebellar white matter is normal
The pattern of symmetrical and extensive involvement of the cerebral white matter, corpus callosum, internal capsule, and brain stem tracts is indistinguishable from the pattern observed in many metachromatic leukodystrophy patients, particularly when radiating stripes of more normal signal intensity are seen within the abnormal cerebral white mat ter. Patients with early-onset GLD usually have more pronounced cerebral atrophy than patients with early-onset metachromatic leukodystrophy. In patients with later onset metachromatic leukodystrophy, the white matter abnormalities often have a predominantly frontal location, whereas in patients with later onset GLD the parieto-occipital white matter is usual
ly predominantly involved. The hyperdense areas seen on CT in GLD are lacking in metachromatic leukodystrophy.
The CT scan findings with high density in the thalamus and basal ganglia are reminiscent of the CT findings in early-onset GM1 and GM2 gangliosidoses. The MRI findings are, however different (see related chapters).
The pattern with parieto-occipital predominance seen in later onset GLD patients resembles the pattern seen in cerebral X-linked adrenoleukodystrophy. However, the pattern consisting of two zones of different signal change within the abnormal white mat ter and a rim of contrast enhancement in between, which is so characteristic of X-linked adrenoleukody-strophy, has never been described in GLD, although contrast enhancement involving the splenium of the corpus callosum has been described in late-onset GLD patients.
The pattern with selective involvement of the cor-ticospinal tracts may resemble the pattern seen in amyotrophic lateral sclerosis. However, the signal changes are less pronounced in the latter. In adreno-myeloneuropathy a similar pattern of MRI abnormalities can be observed, and this disease should be excluded by means of the appropriate laboratory tests.
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