Genotypes of the reported families with cognitive decline and spastin included missense mutations (Ala139Gly)37 as well as large deletion of exon 17.38 One SPAST-affected family has been previously reported in the literature as having a very unusual tau pathology on postmortem brain biopsy.37 As spastin does not limit lifespan, caution is required when interpreting postmortem results as some changes can be age related or due to concurrent diseases. Here, SPAST is linked to Mental deterioration.