Major Neurocognitive Disorders Due to Frontotemporal Disease

Frontotemporal dementia (FTD) is one of the most common types of dementia among individuals under the age of 65, also known as early-onset dementia. It primarily encompasses three distinct clinical subtypes: behavioral variant of FTD (bvFTD), semantic variant primary progressive aphasia (PPA), and nonfluent agrammatic variant PPA (nfPPA). FTD is a strongly familial disorder with genetic heterogeneity, despite a large number of cases being sporadic. Over 80% of the hereditable FTD is attributed to mutations in three genes and predominantly related to the abnormal accumulation of three types of protein. A comprehensive history from the patient and family, followed by a detailed neurological examination, is vital to the evaluation of FTD. Neuropsychological evaluation and imaging modalities like positron emission tomography (PET) or magnetic resonance imaging (MRI) scans can help validate the clinical diagnosis. Despite substantial development in identifying biomarkers for FTD, no specific test is available. Unfortunately, there are no FDA-approved pharmacological treatments for frontotemporal dementia currently, although there are several ongoing clinical trials. Psychoeducation and behavioral interventions can be very effective in the management of bvFTD. Speech therapy and neurostimulation can be useful in PPA. Cholinesterase inhibitors, antidepressants, antipsychotics, mood stabilizers, sedatives, and stimulants have been used for symptomatic management of FTD with limited efficacy.