In support of the MRI findings are postmortem data from an SPG7 case, which showed neuronal loss in the dentate nucleus.21 While the above imaging finding is not specific for SPG7 mutations, yet it is an important characteristic and merits further consideration.22 We propose that dentate nucleus hyperintensity on MRI T2 sequences, without severe overlying cerebellar atrophy and in the context of a typical phenotype, aid the diagnosis of mutant SPG7. This evidence concerns the gene SPG7 and Cerebellar atrophy.