Semantic dementia (SD) is a syndrome within the spectrum of frontotemporal lobar degenerations (FTLD) characterized by fluent progressive aphasia (particularly anomia) and loss of word meaning.
To report a unique case of very early semantic dementia with slowly progressive course allowing insights into the early natural history of this disorder.
Tertiary care university hospital and academic center.
A 62-year-old female retired teacher presenting with “memory” complaints.
Main Outcome Measures
Clinical course, neuropsychological data, MRI.
The patient was first evaluated when standard neuropsychological measures were normal, but subtle left anterior temporal lobe atrophy was present. Over the follow-up period of eight years, she developed profound anomia and loss of word meaning associated with progressive left anterior temporal lobe atrophy consistent with semantic dementia. In more recent years, anterograde memory impairment as well as mild prosopagnosia have evolved in association with left hippocampal atrophy and subtle atrophy in the homologous gyri of the right anterior temporal lobe. She remains functionally independent despite her current deficits.
Early identification of patients who will develop semantic dementia is difficult and might be missed with standard clinical, neuropsychological, and structural neuroimaging evaluations. Recognition of this relatively rare syndrome is important for early diagnosis and prognostication, and particularly for therapeutic interventions in the future.