North Carolina Neurological Foundation (NCNF)

Major Neurocognitive Disorder Due To Frontotemporal Degeneration

Major neurocognitive disorder due to frontotemporal degeneration is a progressive disorder marked by deterioration in behavior, personality, and language, with relative preservation of memory early in the course. Etiology includes genetic mutations (e.g., MAPT, GRN, C9orf72), abnormal tau or TDP-43 protein deposition, and sporadic neurodegeneration. Clinically, patients exhibit disinhibition, apathy, language deficits, and executive dysfunction. Diagnosis relies on clinical criteria, neuroimaging (MRI or PET), and exclusion of other dementias. Management is largely supportive, focusing on behavioral strategies, speech therapy for language deficits, caregiver support, and pharmacologic interventions for symptomatic relief.