Abstract
Hereditary dysphasic disinhibition dementia (HDDD) describes a familial disorder characterized by personality changes, language and memory deficits. The neuropathology includes frontotemporal lobar atrophy, neuronal loss and gliosis and, in most cases abundant amyloid beta plaques and neuropibrillary tangles. A Pick/Alzheimer's spectrum was proposed for the original family HDDD1. Here we report the clinicopathologic case of an HDDD1 individual with additional Alzheimer's disease (AD) pathology, as a common comorbidity, which may mislead clinicians in diagnosis.
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