I would like to remark that the clinical data linking presenilin mutations with non-amyloid dementias have not yet been corroborated with further work demonstrating a causal relationship. For instance, the dementia in the family with the Psen1 insR352 is likely caused by a mutation in progranulin, as reported recently by Boeve et al. (2006).

References:
Boeve BF, Baker M, Dickson DW, Parisi JE, Giannini C, Josephs KA, Hutton M, Pickering-Brown SM, Rademakers R, Tang-Wai D, Jack CR Jr, Kantarci K, Shiung MM, Golde T, Smith GE, Geda YE, Knopman DS, Petersen RC. Frontotemporal dementia and parkinsonism associated with the IVS1+1G->A mutation in progranulin: a clinicopathologic study. Brain. 2006 Nov;129(Pt 11):3103-14. Epub 2006 Oct Abstract

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