Dementia in Younger People

Question 1

What is young onset dementia?

Answer 1

Onset under 65 years old

Question 2

Under 65s vs older 65s dementia presentation, prognosis, impact and support

Answer 2

Question 3

Types of dementia

Answer 3

APOE4 - increases risk
APOE2 - reduces risk
Presenelin 1/2 - 50% chance if have copy

Huntingtons disease, alzheimers disease, genetic less likely in older adults

Question 4

Unique challenges of younger dementia places

Answer 4

• Driving
• Working age
• Stigma
• Role of carer to others
• Multiple losses
• Present to wide range of settings

Question 5

History for ?dementia

Answer 5

• Timeline
• Enquire about memory - recent/remote, knowledge of recent events in news, soaps/sports
• Associated symptoms - language, vision, movement, continence
• Personality/impulsivity/social functioning
• MH - depression/psychosis
• Sleep - REM sleep disorder?
• Appetite - frontotemporal - sweet tooth
• Systemic - falls, motor symptoms, continence

Question 6

MEMORY LANES

Question 7

Rest of history

Answer 7

• PMH
• Psychiatric history
• Current medication/allergies - including compliance
• SH - home situation, ADLs (eating, bathing, continence, finances), driving, occupation, support network
• Substance use - alcohol, cannabis, other substances
• FH - young onset dementia?

Question 8

Assessment of risk

Answer 8

• Self harm/suicide
• Aggression to others
• Wandering
• Self neglect
• Medication compliance
• Meals?
• Falls
• Driving - judgement, spatial orientation and planning
• Carer strain
• Disinhibition - don’t to pay at shops etc

Question 9

Examination of patient with ?dementia

Answer 9

• MSE
• Cognitive assessment - MMSE, GPCOG (primary care), 6CIT (primary care), MOCA, ACE-III, FLB - allow to quantify
• Physical exam with focus on neurological symptoms

GPCOG/6CIT not valid for under 65s - interpret with caution

Question 10

GPCOG

Answer 10

2 part test
Test for patient
Part for informant - collateral, move onto this if don’t do well on first one

Question 11

What to take into consideration with cognitive assessment?

Answer 11

• Take into account baseline - may score well but how did they get there?
• Was it difficult for them?

Question 12

Inv for ?dementia

Answer 12

• Baseline bloods - B12/folate, TFTs, FBC, CRP, U&E, LFT
• Neuroradiology - MRI brain (standard is CT in older patients)
• Can use FDG-PET if need functional imaging - what part of brain is using glucose and what isn’t
• Also used - neuropsychology (more detailed cognitive assessment), OT assessment (dementia is functional diagnosis), CSF (Alzheimers disease - amyloid proteins, Tau, more sensitive in younger people), EEG, genetic testing, ECG

Question 13

Management of dementia in young people

Answer 13

• Explain diagnosis and prognosis
• Consider medication (AD and LBD - cholinesterase inhibitors +/- Memantine in Alz)
• Rivastigmine in PD
• Antipsychotic/antidepressant for BPSD - careful
• MDT - CMHT, OT, SALT, psychology, social
• Voluntary for post diagnostic support - Age UK, Alz Society, Young onset dementia group
• Drivability - assessment
• Legal - wills, POA - Age Uk can help

Question 14

Dementia signs on MRI

Answer 14

Staghorn/knife blade atrophy
Prominent sulci

Question 15

How can posterior cortical atrophy Alzheimers present in young people?

Answer 15

• Cortical blindness - unaware of extent of visual disturbance (do not realise is blind)
• Problems with visual recognition, reading
• Light sensitivity
• Judging distances difficult
• Hallucinations
• Anxious and insight something is not right
• Normal ocular exam

Question 16

Behavioural variant FTD

Answer 16

• 35-65 (or45-65)
• Language/behavioural symptoms
• Later affects movement and memory
• Main symptoms are apathy, loss empathy, disinhibition, overeating, poor judgement, deteriorate in executive function
• Often misdiagnosed as depressive illness

Question 17

FTD umbrella

Answer 17

• MND
• Behavioural varient FTD
• Semantic variant - primary progressive aphasia - loss meaning of words
• non-fluent variant - primary progressive apahsia - loss flow of speech
• Logopenic - primary progressive aphasia - can’t find words, usually Alzheimers variant
• Corticobasal syndrome
• Progressive supranuclear palsy

Question 18

PPA language variants

Answer 18

Question 19

Management of FTD

Answer 19

• Explain diagnosis
• No medical management currently
• MDT - SALT, social, community mental health team, young onset group in Ulverscroft

Question 20

Semantic variant PPA imaging

Answer 20

• Present with imaging changes at time of diagnosis
• Assymmetric temporal lobe atrophy - left more than right
• Assymetric hippocampal atrophy

Question 21

Imitators of dementia

Answer 21

• Delirium
• Psychiatric illness
• Substance misuse
• Menopause –> brain fog
• Fibromyalgia
• Normal pressure hydrocephalus - gait wide based, incontinence, confusion
• Sensory deprivation
• Poorly controlled endocrine disorders - hypothyroid?

Question 22

Rapidly progressing dementias

Answer 22

• Associated neurological symptoms - seizures, spiking temps, focal neuro signs
• Develop over weeks-months-years
• Could be infective cause eg encephalitis

Question 23

Causes of RPD

Answer 23

VITAMINS
* Vascular
* Infection
* Toxic-Metabolic
* Autoimmune
* Mets/neoplasm
* Iatrogenic
* Neurodegenerative
* Systemic

Question 24

Red flags for dementia presentation

Answer 24

• Under 50
• Recent head trauma
• Associated weight loss, headaches, motor sx, seizures, incont, raised ICP
• Positive FH