Snapshot A 58-year-old male is brought to the physician by his daughter for "strange" behavior. He has been innapropriately making sexual remarks, invading people's personal space, and urinating in public. She has noticed that he has been binge eating sweets as of late. His manners and social abilities have drastically worsened. On physical exam, there is a deficit in executive functioning and working memory. Visuospatial function is intact. Introduction Neurodegenerative disorder affecting the frontal and/or temporal lobes dementia behavioral decline and changes in personality aphasia There are different variants behavioral variant primary progressive aphasia Pick bodies hyperphosphorylated tau inclusion bodies ballooned neurons Presentation Behavioral variant disinhibition presents early hyperorality apathy compulsiveness Primary progressive aphasia language deficit (aphasia) early in disease Memory deficits present later Visuospatial function is usually intact Primitive reflexes relatively early than other dementias Evaluation Clinical diagnosis Histologic findings Pick bodies round intracellular aggregates of hyperphosphorylated tau proteins same protein seen in Alzheimer's disease but results in tangles NOT round aggregates MRI atrophy of the frontal and/or anterior temporal lobes Differential Alzheimer's disease Parkinson's disease Huntington's disease Treatment No medications have been shown to be disease modifying treatment is based on symptoms e.g., SSRIs for behavioral symptoms Prognosis, Prevention, Complications Prognosis progressive symptom deterioration worsening disinhibition and personality changes impairment in activities in daily living