Frontotemporal dementia

Frontotemporal dementia manifests primarily as disruption in personality and social conduct, or as a primary language disorder.

Almost 50% of 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 nonpharmacologic and behavioral interventions, caregiver guidance, community services, and social work involvement.

Frontotemporal dementias (FTDs) are the second most frequent primary neurodegenerative brain diseases (after Alzheimer disease) in adults <65 years of age. FTDs typically appear in midlife, with average age of onset between 45 and 65 years, and peak prevalence in the seventh 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 [4]Coyle-Gilchrist IT, Dick KM, Patterson K, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology. 2016 May 3;86(18):1736-43. https://n.neurology.org/content/86/18/1736.long http://www.ncbi.nlm.nih.gov/pubmed/27037234?tool=bestpractice.com

The FTD spectrum comprises a heterogeneous group of conditions that are heritable in some cases. Prominent early symptoms include progressive coarsening of personality, social behavior, self-regulation (of emotions, drives, and behavior), and language. 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.[5]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