MRI findings | Leukodystrophy |
---|---|
Pattern of white matter involvement | |
Predominantly frontal periventricular | Metachromatic leukodystrophy, hereditary diffuse leukoencephalopathy with neuroaxonal spherods |
Predominantly parietal | Adult onset autosomal dominant leukodystrophy |
Predominantly periventricular | Adult polyglucosan body disease |
Predominantly posterior | Krabbe disease |
Predominantly occipital | X linked adrenoleukodystrophy |
Posterior white matter changes progressing anteriorly | X linked adrenoleukodystrophy |
Anterior temporal lobe changes | CADASIL, adult polyglucosan body disease (involvement of temporal lobe) |
Contrast enhancement | X linked adrenoleukodystrophy, Alexander disease |
Sparing of U fibres | Metachromatic leukodystrophy, cerebrotendinous xanthomatosis, adult polyglucosan body disease, Krabbe disease |
Involvement of U fibres | L-2-hydroxyglutaric aciduria |
Corpus callosum | |
Thinning | Adult onset autosomal dominant leukodystrophy, vanishing white matter disease, hereditary diffuse leukoencephalopathy with neuroaxonal spherods |
Hyperintensities | Hereditary diffuse leukoencephalopathy with neuroaxonal spherods, vanishing white matter disease |
Sparing | Cerebrotendinous xanthomatosis, adult polyglucosan body disease (atrophy of corpus callosum can develop late) |
Other findings | |
Enhancement of corticospinal tracts | Krabbe disease |
Cerebellar/brainstem white matter change | Adrenomyeloneuropathy, cerebrotendinous xanthomatosis, Alexander disease |
T2 hypointensity of dentate nucleus | Cerebrotendinous xanthomatosis, L-2-hydroxyglutaric aciduria |
Atrophy medulla oblongata and upper cervical cord | Alexander disease |
Spinal cord atrophy | Adult polyglucosan body disease, adrenomyeloneuropathy |
Cystic changes | Vanishing white matter disease, mitochondrial disease |
CADASIL, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.