Frontotemporal dementia

Manifests primarily as disruption in personality and social conduct, or as a primary language disorder.

Almost 50% of all affected people display parkinsonism; a smaller subset may have motor neuron disease.

Clinical diagnosis derives primarily from examination and brain imaging.

Diagnostic confirmation is based on pathologic exam or identification of gene mutation.

Treatment is supportive, combining medications with caregiver guidance, community services, and social work interventions.

Frontotemporal dementias (FTDs) are the second most frequent primary neurodegenerative brain diseases (after Alzheimer disease) in adults <65 years of age. The FTD spectrum comprises a heterogeneous group of conditions that appear heritable in some cases. Prominent early symptoms include progressive coarsening of personality, social behavior, self-regulation (of emotions, drives, and behavior), and language. FTDs typically appear in midlife, with peak onset in the sixth decade.[1]Ratnavalli E, Brayne C, Dawson K, et al. The prevalence of frontotemporal dementia. Neurology. 2002 Jun 11;58(11):1615-21. http://www.ncbi.nlm.nih.gov/pubmed/12058088?tool=bestpractice.com [2]Rosso SM, Donker Kaat L, Baks T, et al. Frontotemporal dementia in the Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain. 2003 Sep;126(Pt 9):2016-22. http://brain.oxfordjournals.org/content/126/9/2016.full http://www.ncbi.nlm.nih.gov/pubmed/12876142?tool=bestpractice.com [3]Johnson JK, Diehl J, Mendez MF, et al. Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol. 2005 Jun;62(6):925-30. http://archneur.jamanetwork.com/article.aspx?articleid=788706 http://www.ncbi.nlm.nih.gov/pubmed/15956163?tool=bestpractice.com Gross changes in social behavior and language are often seen, including indifference to self-care and others' needs, loss of speech and comprehension, loss of empathy, distractibility, impulsiveness, disinhibition, stereotyped behaviors and rigid routines, and compulsions.[4]Piguet O, Hornberger M, Mioshi E, et al. Behavioral-variant frontotemporal dementia: diagnosis, clinical staging and management. Lancet Neurol. 2011 Feb;10(2):162-72. http://www.ncbi.nlm.nih.gov/pubmed/21147039?tool=bestpractice.com