Familial multisystem atrophy with possible thalamic dementia

We studied a family with progressive dementia, optic atrophy, and spastic paraparesis. Autopsy of one family member revealed multisystem atrophy with widespread degeneration of the thalamus and marked attenuation of hemispheric white matter to account for the dementia. This family may suffer from a unique disorder, but the findings overlap with other reported cases of thalamic dementia or familial spastic paraparesis.