We report a 44-year-old woman, with a family history of early-onset dementia, presenting with primary progressive aphasia. This clinically variable syndrome has multiple underlying pathologies, and correlations between clinical manifestations and postmortem neuropathologic findings are controversial. Our patient suffered worsening language impairment with major word-finding difficulties but preserved comprehension. She also developed episodic memory impairment. Her condition progressed to dementia with behavioral changes. Magnetic resonance imaging showed early left perisylvian and bitemporal atrophy. The patient died shortly afterward from colon cancer. Neuropathologic examination revealed advanced early-onset Alzheimer and Lewy body disease, plus a clinically nonrelevant metastasis of her colon cancer in her left parietal lobe. Genetic examination revealed a p.Glu184Asp mutation in the presenilin1 gene. Our findings confirm the importance of a thorough appreciation for the clinical and neuropathologic correlations in patients with atypical neurodegenerative dementias.

*Department of Neurology and Centre of Clinical Neuroscience and ‡Department of Pathology 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic Departments of †Pathology and Molecular Medicine and ††Neurology Thomayer Hospital Prague Czech Republic §Department of Radiology Na Homolce Hospital Prague Czech Republic ∥Department of Neurology 3rd Faculty of Medicine Charles University Prague Czech Republic ¶Neurodegenerative Brain Diseases group Center for Molecular Neurology VIB Antwerp Belgium Laboratory of Neurogenetics Institute Born Bunge University of Antwerp Antwerp Belgium **Department of Communication Disorders Faculty of Education Comenius University Bratislava Slovakia

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