title: Behavioural variant frontotemporal dementia: clinical and therapeutic approaches.
abstract: BACKGROUND
  Behavioural variant frontotemporal dementia (bvFTD) is the most frequent presentation in the clinical spectrum of frontotemporal dementia (FTD) and it is characterised by progressive changes in personality and conduct. Major breakthroughs in molecular biology and genetics made during the last two decades have lent us a better understanding of this syndrome, which may be the first manifestation in many different neurodegenerative diseases.
  
  
  METHODS
  We reviewed the main epidemiological, clinical, diagnostic and therapeutic aspects of bvFTD. Most cases manifest sporadically and the average age of onset is 58 years. Current criteria for bvFTD propose three levels of diagnostic certainty: possible, probable, and definite. Clinical diagnosis is based on a detailed medical history provided by family members and caregivers, in conjunction with neuropsychological testing. Treatments which have been used in bvFDT to date are all symptomatic and their effectiveness is debatable. New drugs designed for specific molecular targets that are implicated in frontotemporal lobar degeneration are being developed.
  
  
  CONCLUSIONS
  BvFDT is a frequent cause of dementia. It is a non-specific syndrome associated with heterogeneous histopathological and biomolecular findings. The definition of clinical subtypes complemented by biomarker identification may help predict the underlying pathology. This knowledge, along with the development of drugs designed for molecular targets, will offer new treatment possibilities.
authors: Servicio de Neurología. Hospital Clínico San Carlos, Madrid, España. Electronic address: martafmatarrubia@gmail.com.
authors: M
authors: M
authors: Fernández-Matarrubia
authors: Servicio de Neurología. Hospital Clínico San Carlos, Madrid, España.
authors: J A
authors: JA
authors: Matías-Guiu
authors: Servicio de Neurología. Hospital Clínico San Carlos, Madrid, España.
authors: T
authors: T
authors: Moreno-Ramos
authors: Servicio de Neurología. Hospital Clínico San Carlos, Madrid, España.
authors: J
authors: J
authors: Matías-Guiu
mesh_terms: D001921:Brain / Q000473:pathology; D057180:Frontotemporal Dementia / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000473:pathology; D009483:Neuropsychological Tests
keywords: Behavioural variant frontotemporal dementia; Clinical aspects; Clínica; Criterios diagnósticos; Degeneración lobar frontotemporal; Demencia frontotemporal variante conductual; Diagnosis; Diagnostic criteria; Diagnóstico; Frontotemporal lobar degeneration; Tratamiento; Treatment