Brief reviews

Abstract

Authors described demographic and clinical characteristics of 100 consecutive patients seen over a 17-year period at the Memory Clinic in Cambridge. Semantic dementia is one of the clinical variants of frontotemporal lobar degeneration, and is considered a cause of early onset dementia in most cases. The most interesting results were: 60% men; mean age of onset of symptoms at 60.3 (7.1) years; 46% of cases had initial symptoms after the age of 65 years; 15% of cases had a positive familial history of dementia in a first degree relative; and median survival of 12.8 years. MRI predominantly showed a left predominant temporal atrophy. Twenty-four out of 32 patients had pathological confirmation, and presented ubiquitin positive and tau negative inclusions; and 13 were positive for TAR DNA binding protein (TDP-43) after re-staining. This report yields important information about this disease in a large sample and provides clues for diagnosis: well-preserved orientation, visuospatial skills, and verbal episodic memory. With regard to impairments, these included: poor category fluency, naming and word comprehension plus surface dyslexia.