A recent case report described a family with pathologically-confirmed cases ofearly-onset, autosomal-dominant familial AD (EOFAD) linked to a Met233Leu mutationof the presenilin-1 gene (PSEN-1), and one family member developed prominent CBScombined with severe neuropsychiatric and behavioral disturbances resembling thoseoften found in EOFAD. The gene discussed is PSEN1; the disease is early-onset autosomal dominant Alzheimer disease.