40 HOW TO TREAT : YOUNG-ONSET DEMENTIA
40 HOW TO TREAT : YOUNG-ONSET DEMENTIA
4 AUGUST 2023 ausdoc . com . au mutation rather than other environmental factors , emphasising the need for accurate diagnosis of type of young-onset dementia and genetic testing . 68
Research does not clearly demonstrate whether the progression of dementia in younger people is faster and associated with more severe impairments compared with older people with dementia . 69 A 2020 review reported evidence to suggest that young-onset AD declines faster than older-onset AD , however the evidence was less consistent with the other young-onset dementias . 70
Compared with the general population , those with young-onset dementia have a significantly increased risk of death , with one Australian study reporting a six-times increased risk compared with the general population . 71
The natural history of dementia is similar in older and younger people , with progressive ongoing decline in cognition , function and behaviour that require support . Causes of death are also similar ( respiratory and cardiovascular ) and generally related to the dementia ( Loi et al , unpublished ). There is little in the literature reporting on palliative care and young-onset dementia , but the authors recommend earlier rather than later referral to these services .
THE FUTURE
CATIONS et al made eight recommendations for timely identification , diagnosis and care for people with young-onset dementia ( see box 2 ). 39
Biomarkers show great promise in distinguishing psychiatric vs young-onset neurodegenerative disorders using CSF and plasma neurofilament light chain ( see figure 1 , unpublished ). 30 , 32 A combination of plasma biomarkers , such as p-tau 181 , glial fibrillary acidic protein , amyloid-beta ( Aβ ) 40 and Aβ42 , may be able to differentiate dementia types . 72 There are studies underway seeking to investigate biomarkers and young-onset dementia and various neurological and psychiatric disorders ( www . themindstudy . org ).
Streamlining pathways to care and improving access using telehealth may improve diagnostic delay . The government pledge to have no-one under 65 entering and living in residential aged care is yet to eventuate ; appropriate housing for individuals with young-onset dementia remains problematic . As well as more timely access to NDIS and consideration of people who have young-onset dementia ( that is , onset at younger than 65 ) but are older than 65 who cannot access the more generous packages offered by NDIS , there is also a need to upskill formal community workers in the NDIS about the needs and issues relevant to those with young-onset dementia .
Clinical pharmaceutical trials have to date been disappointing . However , the age cut-off for inclusion is often 60 or older , which excludes some people with young-onset dementia . There is a paucity of young-onset dementia-specific interventions . 9
CASE STUDIES
Case study one
JULIET , 59 , is referred for specialist opinion after her first admission to a psychiatric hospital . She was paranoid and feared for her life , believing her husband was trying to harm her . Juliet ran away from her home in an
Table 5 . Services for behaviour changes associated with young-onset dementia
State or territory Contact Consideration National
Victoria
NSW
South Australia
Western Australia
Queensland
Northern Territory
Tasmania
ACT
Dementia support Australia 1800 699 799 bit . ly / 3UaQnP3
Victorian State Government Areabased services : State funded clinical mental health service bit . ly / 3hcwUPg
Better Health Channel Victoria bit . ly / 3FPEnOD
General information bit . ly / 3tfgt7C
Older adults bit . ly / 3TmTEtl
General information bit . ly / 3hoANAI
Older adults bit . ly / 3DYS5MC
General information bit . ly / 3EgLKxd
General information bit . ly / 3tjtEUQ
Find a mental health service bit . ly / 2SuEXF0
General information nt . gov . au / wellbeing / mental-health
Hospital mental health services in the Top End and Central Australia bit . ly / 3tfQoVY
General information bit . ly / 3WNReXk
Statewide mental health services bit . ly / 3hvoUJu
General information bit . ly / 2xWZUkw
Canberra mental health programs
agitated , distressed state , was picked up by the police and brought to ED , where she told staff that her husband was torturing and assaulting her . Juliet said he had been gaslighting her by hiding her keys , wallet and phone . She thought she was being poisoned because there was something wrong with her vision as she couldn ’ t see properly or read or write . Collateral history from her sister and husband reveals that Juliet has paranoia about her husband trying to harm her .
Before this episode , she had become increasingly anxious over
Anyone can make a referral for individuals living at home / community or in residential care / supported accommodation
People with youngonset dementia may fall under either adult or aged
People with youngonset dementia may fall under either adult or aged
People with youngonset dementia may fall under either adult or aged
People with youngonset dementia may fall under either adult or aged
People with youngonset dementia may fall under either adult or aged
People with youngonset dementia may fall under either adult or aged
People with youngonset dementia may fall under either adult or aged
Table 6 . Potential triggers for behaviour changes associated with dementia
Trigger Thirst and hunger Temperature changes Inadequate lighting Pain Excessive noise Confusing surroundings Loneliness and boredom
Change in carers / families / routine
Strategy Rehydration and appropriate food Appropriate heating , cooling , blankets Natural daylight Regular analgesia , gentle mobilisation Can try headphones or music ; auditory review Ease of way , signage
Activities appropriate for the individual Structure and routine
Try to minimise
the past four years at her new job as a book-keeper because she felt bullied ; previously she had been high functioning and excelled at work .
Juliet is transferred to a specialist young-onset inpatient unit where she undergoes neuropsychiatric assessment , neuropsychology , functional assessment , speech pathology , lumbar puncture and structural neuroimaging . Her ACE-III is 84 / 100 and NUCOG 69 / 100 .
She has significant visuospatial impairment and apraxias with difficulty with perception . Episodic
Figure 2 . Sagittal T1 MRI brain of PCA , demonstrating posterior atrophy relative to the rest of the brain .
Figure 3 . FLAIR axial slice demonstrating posterior atrophy .
Box 2 . Recommendations for timely identification , diagnosis and care for people with young-onset dementia
• Promote pathways to care for assessment and diagnosis .
• Improve diagnostic accuracy and pathways to research .
• Conceptualise young-onset dementia as a disability .
• Improve integration of disability , health and aged care sectors .
• Invest in telehealth for equitable access to services .
• Improve treatment and management for First Nations People .
• Integrate age-appropriate services for family members .
• Enable access to appropriate housing .
memory is relatively preserved . MRI demonstrates atrophy in the posterior / occipital lobes ( see figures 2 and 3 ). CSF analysis of Aβ42 , p-tau and t-tau are consistent with Alzheimer ’ s disease . Julie is diagnosed with posterior cortical atrophy and started on risperidone 0.5mg nocte and donepezil 5mg , which is eventually increased to 10mg .
Case study two
Gary , 59 , separated and unemployed ,
Case courtesy of Associate Professor Frank Gaillard .
Case courtesy of Royal Melbourne Hospital Neuropsychiatry Unit . is referred to the specialist young-onset dementia inpatient unit after a three-month admission to a local psychiatric hospital . He was admitted there as an involuntary patient after being found by the police living in an abandoned home , in squalor , unkempt and neglecting his personal needs .
On history , Gary reported that in the past two years he had become convinced that a woman in South Africa was in love with him and he wanted to be with her . He had wired several tens of thousands of dollars to South Africa , emptying his bank accounts and superannuation ; he is now in significant debt , having sold his belongings and taken large loans . He became known to the Federal Police as a victim of an international financial scam .
Collateral history from his sister suggests deterioration in his function and behaviour over the past six years , with job loss , apathy and inappropriate sexual behaviour towards women . His wife and three children had ceased contact with him in the past few years .
Gary ’ s MMSE score is 28 / 30 and MRI demonstrates mild frontotemporal volume loss ( see figures 4 and 5 ). Formal neuropsychology testing