A 32 year-old man presented with progressive cognitive loss over many years.  His examination showed optic atrophy, weakness and spasticity in addition to significant cognitive impairments.

Axial MRI scan (Flair Image): Note the large confluent abnormalities in the white matter.  Although a similar pattern can be seen in late multiple sclerosis, in multiple sclerosis, it is usually a multifocal pattern.  Likewise, in an elderly individual, a similar pattern may be seen in far advanced small vessel disease.  In a younger individual, this is the picture of a leukodystrophy.  Although most leukodystrophies affect infants and children, rarely they may present as adults with cognitive and long tract signs.  Some like metachromatic leukodystrophy (MLD) affect both central and peripheral myelin.

Revised 06/30/03.
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