Behavioural-variant frontotemporal dementia: An update

Abstract

Behavioural-variant frontotemporal dementia (bvFTD) is characterised by insidious changes in personality and interpersonal conduct that reflect progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation and decision making. The underlying pathology is heterogeneous and classified according to the presence of intraneuronal inclusions of tau, TDP-43 or occasionally FUS. Biomarkers to detect these histopathological changes in life are increasingly important with the development of disease-modifying drugs. Gene mutations have been found which collectively account for around 10-20% of cases including a novel hexanucleotide repeat on chromosome 9 (C9orf72). The recently reviewed International Consensus Criteria for bvFTD propose three levels of diagnostic certainly: possible, probable and definite. Detailed history taking from family members to elicit behavioural features underpins the diagnostic process with support from neuropsychological testing designed to detect impairment in decision-making, emotion processing and social cognition. Brain imaging is important for increasing the level of diagnosis certainty. Carer education and support remain of paramount importance.

Burden of caregivers of patients with frontotemporal lobar degeneration – a scoping review

“…Frontotemporal lobar degeneration (FTLD) is a neurodegenerative disorder resulting from brain atrophy in the frontal and temporal lobes, leading to changes in personality, behavior, language skills and forward planning (Piguet and Hodges, 2013). A systematic review conducted in 2016 estimated that FTLD cases constitute 15% of all cases of dementia in individuals younger than 65 or 70 years (different cut-offs were used in the reviewed studies), making it the second-most common form of presenile dementia after Alzheimer's Disease (AD) (Hogan et al, 2016).…”

“…There is a lack of information as well as appropriate care facilities. Caregiver depression and reduced caregiver wellbeing can occur as a result of neglecting one's own needs (Piguet and Hodges, 2013).…”

What is known about the subjective needs of people with behavioural variant frontotemporal dementia? A scoping review

“…The early stage of the disease is associated less with impairment of memory and more with various strong changes in judgement, behaviour, personality and emotions (Rankin et al . , Piguet & Hodges ). The disease tends to affect individuals at a time of life when they have a job and/or a family to attend to (Mendez ), and the neuropsychological and functional abilities in people with FTD are more severely affected than in individuals with other types of dementia (Piguet et al .…”

Utilidad de la batería ejecutiva y cognición social de INECO en la evaluación de funciones ejecutivas de una serie de pacientes peruanos con demencia fronto-temporal, variante conductual.

“…La apatía es un síntoma temprano muy común de DFTvc; sin embargo, suele ser catalogado erróneamente como depresión. Se manifiesta como inercia, escasa motivación, falta de interés por las actividades de entretenimientos previos y aislamiento social progresivo (8)(9)(10). Puede coexistir desinhibición, la cual suele ser confundida con un episodio maniaco, trastorno obsesivo-compulsivo o un trastorno de la personalidad.…”

“…Esta lleva a acciones impulsivas, manifestadas como falta de tacto o sutileza para hacer o decir lo que realmente se piensa, excesos en sus gastos, actos con contenido sexual inapropiado y un amplio rango de conductas socialmente embarazosas (comportamientos pueriles, excesiva e inapropiada familiaridad con extraños, desobediencia a las normas, entre otros). En ocasiones, es posible que el síntoma inicial se manifieste como juego patológico o en otros casos, como hiper-religiosidad (9,10). Por otro lado, los pacientes presentan conductas estereotipadas, las que pueden ir desde rutinas motoras repetitivas hasta complejas obsesiones.…”