Abstract:
:We report a case of frontotemporal dementia caused by a novel MAPT mutation (Q351R) with a remarkably long amnestic presentation mimicking familial Alzheimer's disease. Longitudinal clinical, neuropsychological and imaging data provide convergent evidence for predominantly bilateral anterior medial temporal lobe involvement consistent with previously established neuroanatomical signatures of MAPT mutations. This case supports the notion that the neural network affected in MAPT mutations is determined to a large extent by the underlying molecular pathology. We discuss the diagnostic significance of anomia in the context of atypical amnesia and the impact of impaired episodic and semantic memory systems on autobiographical memory.
journal_name
Neurocasejournal_title
Neurocaseauthors
Liang Y,Gordon E,Rohrer J,Downey L,de Silva R,Jäger HR,Nicholas J,Modat M,Cardoso MJ,Mahoney C,Warren J,Rossor M,Fox N,Caine Ddoi
10.1080/13554794.2013.826697subject
Has Abstractpub_date
2014-01-01 00:00:00pages
684-94issue
6eissn
1355-4794issn
1465-3656journal_volume
20pub_type
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