Roll over Pick and tell Alzheimer the news!

In this issue of Neurology, Kertesz et al. integrate neurobehavioral, genetic, and pathologic data to analyze a dominantly inherited disease phenotype that the authors refer to as Pick complex or frontotemporal dementia (FTD).1 This study was promoted by the discovery that mutations in the tau gene cause FTD and parkinsonism linked to chromosome 17 (FTDP-17).2-7 The FTDP-17 phenotype encompasses seemingly heterogeneous, inherited clinicopathologic entities such as pallido-ponto-nigral degeneration, familial multiple system tauopathy, hereditary-dysphasic-disinhibition-dementia, familial progressive subcortical gliosis, and the disinhibition-dementia-parkinsonism-amyotrophy complex.8 These phenotypes are all genetically linked to the tau locus. Not surprisingly, variations in the pathology of FTDP-17 have been detected by ultrastructural and immunocytochemical analysis of aberrant neuronal and glial proteins (e.g., tau, α-synuclein, neurofilament, beta-amyloid, human glial fibrillary acid protein, the prion protein, vimentin, and ubiquitin). Some, but not all, of these variations are due to the location of tau gene mutations and their effect on …