The Alzheimer's Australia NSW blog *Younger Onset Dementia and ME*

*The Alzheimer's Australia NSW blog *Younger Onset Dementia and ME* is a place for people to connect with others, to get information and helpful suggestions.

A diagnosis of younger onset dementia can be very isolating. You may have received a diagnosis, be someone who is caring for a family member, or you may have a mum or dad with younger onset dementia. You are not alone. The National Younger Onset Dementia Key Worker Program provides individualised information and support to improve the quality of life for people with younger onset dementia and also see NSW - Younger Onset Dementia . For more information contact the National Dementia Helpline on 1800 100 500.

Sunday, 15 May 2011

Behavioural-variant frontotemporal dementia: diagnosis,

by Olivier Piguet, Michael Hornberger, Eneida Mioshi, John R Hodges

Patients with behavioural-variant frontotemporal dementia (bvFTD) present with insidious changes in personality and interpersonal conduct that indicate progressive disintegration of the neural circuits involved in social cognition,emotion regulation, motivation, and decision making. The underlying pathological changes are heterogeneous and are characterised by various intraneuronal inclusions. Biomarkers to detect these histopathological changes in life are becoming increasingly important with the development of disease-modifying drugs. Gene mutations have been found that collectively account for around 10–20% of cases. Recently, criteria proposed for bvFTD define three levels of diagnostic certainty: 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 diagnostic certainty. A recently developed staging instrument shows much promise for monitoring patients and evaluating therapies, which at present are aimed at symptom amelioration. Carer education and support remain of paramount importance.
Introduction
Frontotemporal dementia (FTD) is the clinical diagnostic term now preferred to describe patients with a range of progressive dementia syndromes associated with focal atrophy of the orbitomesial frontal and anterior temporal lobes.1 Epidemiological studies suggest that FTD is the second most common cause of young-onset dementia after Alzheimer’s disease (AD).2,3