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Early-Onset Familial Alzheimer Disease With Coexisting -Amyloid and Prion Pathology

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To the Editor: Familial Alzheimer disease (AD) with early onset has been linked to 3 different genes with an autosomal dominant mode of inheritance: -amyloid, protein precursor, and the presenilins 1 and 2, representing not more than 50% of all cases of early-onset AD cases.¹ Thus, the genetic defect remains unexplained in at least half of the families with histories of early onset of AD. We have recently described such a Swiss family whose members presented with a standard clinical and neuropathologic profile of AD.² In particular, severe neurofibrillary tangle degeneration was present in the hippocampus and in several cortical areas, together with a large amount of -amyloid deposits and senile plaques (SPs). However, known mutations have not been found, either in the -amyloid precursor protein or in the presenilin 1 and 2 genes.² We now report that the brains of 5 deceased members of this family, from 2 generations, . . . [Full Text of this Article]

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