Severe anterograde amnesia with extensive hippocampal degeneration in a case of rapidly progressive frontotemporal dementia

Frontotemporal dementia (FTD) is usually characterized as a spectrum of rel atively slowly progressive disorders with largely focal frontal or temporal presentations. The development of clinical and research criteria for discr iminating FTD from Alzheimer's disease has relied, in part, on the relative preservation of episodic memory in FTD, We present a patient with FTD who, in addition to the more typical behavioural and language deficits, had a p rofound anterograde amnesia at the time of diagnosis. Neuroimaging confirme d atrophy of frontal and temporal lobes bilaterally, most marked in the ant erior left temporal region. At post-mortem, non-Alzheimer pathology resulti ng in devastating cell loss was revealed in the hippocampi, as well as in t he frontal and temporal cortex, thus providing neuroanatomical corroboratio n of the episodic memory deficit. Progression of the disease was extraordin arily rapid, with just 2 years between reported onset and time of death. Th is case demonstrates that the pattern of FTD may include severe anterograde amnesia as a prominent and early consequence of the disease.