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HOW TO TREAT 41
Figure 4 . Sagittal MRI demonstrating volume loss in the frontal lobes with preservation of mesial temporal lobes .
Case courtesy of Associate Professor Frank Gaillard .
Case courtesy of Associate Professor Frank Gaillard .
Figure 5 . Axial MRI demonstrating volume loss in the frontal lobes with preservation of mesial temporal lobes .
How to Treat Quiz .
1 . Which THREE statements regarding young-onset dementia are correct ? a Young-onset dementia refers to a dementia where symptom onset occurs before age 65 . b Genetic causes of dementia are relatively common . c People with young-onset dementia face major challenges . d People with young-onset dementia can be misdiagnosed with psychiatric disorders .
2 . Which THREE are more common causes of dementia in younger people ? a Alzheimer ’ s disease . b Alcohol-related dementias . c Multiple sclerosis . d Traumatic brain injury .
3 . Which THREE are red flags for young-onset dementia ? a A new-onset psychiatric presentation in someone middle-aged with no previous psychiatric history . b Behaviour changes inconsistent with an individual ’ s previous personality . c Cognitive / behavioural / psychiatric changes in someone where there is a family history of young-onset dementia . d New neurological symptoms .
4 . Which THREE may indicate the condition is neurodegenerative rather than psychiatric ? a Onset in late adolescence or early adulthood . b Primitive reflexes . c Upper or lower motor neuron signs . d Family history of dementia at a younger age .
5 . Which THREE statements regarding the diagnosis of young-onset dementia are correct ? a The Mini-Mental State Examination is the cognitive assessment of choice . b Refer early to specialist service if young-onset dementia is suspected . c The neuroimaging of choice is an MRI . d It may take 4-5 years to make a diagnosis of young-onset dementia .
6 . Which TWO statements regarding young-onset dementia are correct ? a Children of those with young-onset dementia worry about the future and require practical and psychological reveals executive impairments — slowed processing speed , impaired social cognition and abstract reasoning . His NUCOG score is 59 / 100 .
Functional neuroimaging ( positron emission tomography , see figure 6 ) shows moderate bilateral hypometabolism of the frontal lobes .
On examination at the specialist unit , Gary has weakness in the upper limbs and intrinsic muscles of the hands . He has bilateral palmomental reflexes . Of note are fasciculations in
YOUNG-ONSET DEMENTIA
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Case courtesy of Associate Professor Frank Gaillard . support at times . b Most dementia care services are suitable for young people with dementia and their families . c The spouse often transitions into a caregiver , assuming a different role and more responsibility . d Younger people with dementia usually prefer to live in a residential facility .
7 . Which THREE statements regarding the management of young-onset dementia are correct ? a Cholinesterase inhibitors will modify disease progression . b Consider management from a biopsychosocial perspective for the individual and the family . c Executive deficits may be improved by structure and routine . d Patients can benefit from the ongoing care of a collaborative multidisciplinary team .
8 . Which THREE medications may be used in declining cognition ?
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Figure 6 . Nuclear med scan demonstrating severe hypoperfusion in the left posterior frontal and anterior temporal lobes along the Sylvian fissure .
his tongue , bilateral triceps , deltoid and pectoralis muscles . Nerve conduction studies reveal fasciculations in most muscles with chronic partial denervation and confirm the presence of lower motor neuron / anterior horn cell involvement .
Respiratory function tests suggest a moderately restrictive ventilation defect with a forced vital capacity of 60 % ( normal 80 % to 120 %) and maximal expiratory pressure ( MEP ) of 19 %.
a Donepezil . b Mirtazapine . c Rivastigmine . d Memantine .
9 . Which THREE statements regarding the behaviour changes associated with dementia are correct ? a They may precipitate entry into residential care and presentations to ED . b Pharmacological management of these behaviour changes is first-line . c They may be manifestations of an unmet need the patient cannot communicate . d Treatment includes practical person-centred strategies to meet the individual ’ s needs .
10 . Which THREE statements regarding the prognosis of young-onset dementia are correct ? a Those with young-onset dementia have a significantly increased risk of death compared with the general population . b All types of young-onset dementia have the same median survival time . c Causes of death are similar in older and younger people with dementia . d There is evidence to suggest that young-onset AD declines faster than older-onset AD .
Sleep studies show severe disordered breathing and likely nocturnal hypoventilation syndrome .
Gary is diagnosed with frontotemporal dementia-motor neuron disease . Following family feedback , genetic testing for C9orf72 mutation is arranged . Gary is discharged into supported accommodation with follow-up by the neuropsychiatry and specialist MND service .
CONCLUSION
THE GP is essential in the care of an individual and family affected by young-onset dementia . Having known the patient for some time , the GP is able to provide objective collateral history .
The GP , assisted by good communication with the specialist / s , is also able to regularly monitor physical health , behaviours , medication benefits and adverse effects , assess the wellbeing of the individual and family , and provide a ‘ sounding box ’ for families . This offers reassurance and stability for a disorder that may cause much psychosocial instability .
RESOURCES
• Dementia Australia — Information and support for people living with younger onset dementia bit . ly / 3h0k0nt — Other support services bit . ly / 3Nxl0LZ
• Australian Institute of Health and Welfare – Younger onset dementia : new insights using linked data bit . ly / 3h0ujYp
• Australian Government Department of Health and Aged Care – Younger people in residential aged care : priorities for action bit . ly / 3vY6lRR
• Australian Dementia Network – Find a Memory Clinic or Cognitive Decline Assessment Service bit . ly / 3SUtuOm
• Australian Government Department of Health and Aged care – MRI and PET locations bit . ly / 3U6Dgy0
References Available on request from howtotreat @ adg . com . au