Young Onset Dementia

1
Young Onset Dementia

• Dr Don Brechin
• Consultant Clinical Neuropsychologist
• DoN/SNN PQT Training Day
• Aberdeen, March 2007

2
PREVALENCE
3
Prevalence x Age

Age
4
Prevalence x Age (Harvey et al, 2004)
5
Prevalence x Age (Teesside - 2006)

Age
6
Diagnostic Groups

7
Diagnostic Groups

8
Diagnostic Groups

9
Diagnosis x Age Teesside (2006)
N
Age
10
SOME NEUROPSYCHOLOGY
11
ALZHEIMERS DISEASE
12
Alzheimers Disease
Auguste D (aged 51 years)
13
AD Localisation
14
AD Localisation
36 months
Mildly affected
18 months
15
AD Typical Presentation

• Early symptoms
• Forming new memories
• Rapid forgetting
• Poorly orientated
• Speech becomes sparser in content and more
  repetitive
• Later symptoms
• ADL skills decline and spatial problems emerge
• Failure to recognise family members
• Delusions, aggression and wandering can occur

16
Variants of AD

• Historically, most studies describe
  neuropsychological performance of older adults
  with AD
• These studies suggest a relatively uniform
  presentation of AD

17
Variants of AD

• More recently, three main variants of AD have
  been described
• Typical Amnesic (medial temporal) variant
• Visual (occipitoparietal) variant
• Linguistic (lateral temporal) variant
• All three types are pathologically proven to be
  AD
• It has also been suggested that there is a
  progressive apraxia variant

18
Variants of AD

• Recent data suggests that the visual variant of
  AD is more common in Young Onset Dementia (e.g.
  Kemp et al, 2003)

19
Variants of AD Kemp

• Healthy Control

20
Variants of AD Kemp

• Late Onset AD

Early Onset AD
21
1. Typical (Amnesic) AD
22
(No Transcript)
23
(No Transcript)
24
AD Memory

• Profound anterograde memory deficit
• problems learning new information and retaining
  it after a delay
• Rapid forgetting of material even when calculated
  as a proportion of material originally learned
• Recognition memory formats do not improve
  performance
• Delis et al (1991)

25
Blackwell et al (2004)

• 32 month followup of Blackwell et al (2001)
  sample of people with Questionable Dementia
• Various cognitive tests repeated, including
  CANTAB Paired Associate Learning (PAL)

26
Blackwell et al (2004)

• 43 QD subjects originally
• At follow-up
• 11 converted to probable AD
• 29 did not develop AD
• 2 developed ?DLB (excluded from analysis)
• A combination of CANTAB PAL and a naming task
  (GNT) differentiated these groups at the very
  first assessment with 100 accuracy

27
Fowler et al (2002)

• Normal controls, early AD, and QD patients
• Assessed at 0, 6, 12, 18 and 24m
• Standard neuropsychological battery subtests
  from CANTAB

28
Fowler et al (2002)

• A subgroup of QD patients deteriorated at 6m, and
  by 24m were all diagnosed with AD
• CANTAB PAL performance at 0 months distinguished
  this deteriorating group before any other
  neuropsychological measure (including list
  learning)

29
Fowler et al (2002)
Total PAL Error scores
30
Fowler et al (2002)
PAL errors x group
31
Fowler et al (2002)
Rey AVLT Scores for QD group
32
AD Semantic Memory

• Poor performance on semantic memory tasks as
  evident by
• Reduced ability to recall overlearned facts
  (Norton et al, 1997)
• Reduced confrontation naming (Hodges et al, 1991)
• Reduced category fluency (Monsch et al, 1994)
• Reduced semantic priming (Salmon
  Fennema-Notestine, 1996)

33
Letter and Category Fluency
34
AD Praxis
35
AD Other Deficits

• Executive functioning/attention problems
• Dual-processing tasks
• Shifting attention
• Working memory
• Visuo-spatial functions
• Sublte early symptoms, deteriorating in the
  course of the illness (e.g. Block Design, clock
  drawing, copying a complex figure)

36
AD Summary

• Intellect (pre-morbid and current)
• Memory
• Language
• Perceptual, visuo-spatial and praxis skills
• Executive functions
• Attention

Attention
Retrieval
Input
Storage
37
2. Visual Variant AD

• Posterior occipitoparietal degeneration
• Variety of deficits (not necessarily co-occuring)
• Visual problems
• Dyspraxia
• Dysgraphia
• Simultanagnosia

38
2. Visual Variant AD

• Hodges (2001) Galton et al (2000)
• Progressive visual loss with retriction of fields
  and severe apperceptual agnosia
• Preserved perceptual abilities but severe
  problems with high level visual analysis (e.g.
  simultanagnosia, alexia, colour agnosia) -
  ventral occipito-temporal pathway
• Visual disorientation, navigation problems,
  visual mis-reaching, apraxia - bi-parietal variant

39
3. Linguistic Variant AD

• Fluent aphasia with impaired semantics vs.
• Non-fluent with slow halting speech output
  alternating with disrupted phonology
• Fluent aphasia variant can be confused with
  semantic dementia, although amnesic deficits are
  less evident in the latter

40
Revisiting the Variants

• Lamdon Ralph et al (2003)
• Most variation in performance across time is
  predicted by severity of illness (i.e. Typical
  AD)
• Significant deviations from this profile reveal
  two main variants
• Semantic (i.e. formerly the linguistic group)
• Visuo-spatial

41
Revisiting the Variants

• Lamdon Ralph et al (2003)
• Semantic group
• Semantic deficits (e.g. naming, picture matching)
  and episodic memory deficits
• Preserved receptive language and visuo-spatial
  functons
• Visuo-spatial group
• Visuo-spatial skills impaired and limb apraxia,
  together with episodic memory impairment
• Preserved semantic memory and language

42
Revisiting the Variants

• Lamdon Ralph et al (2003)
• In the semantic and visuo-spatial variants,
  episodic memory is still impaired but the other
  deficits are worse than in typical AD
• Still the possibility of other variants (I.e. the
  progresive apraxia group)

43
4. Progressive Apraxia Variant

• Can include
• Limb apraxia
• Speech apraxia
• Buccofacial apraxia
• Extrapyramidal symptoms (e.g. rigidity,
  myoclonus)
• Symptoms are similar to cortico-basal degeneration

44
CORTICO-BASAL DEGENERATION
45
CBD Presentation

• Stover Watts (2001)
• Insidious with asymmetric onset
• Mean age of onset 63 yr (s.d. 7 yr)
• Mean time to death 7.9 yr
• In the first 3 years, 90 of cases have
• Akinesia
• Rigidity
• Apraxia
• Asymmetric limb dystonia is common
• Alien limb is found in 50 of cases

46
CBD - Case Example

• Initial symptom problems signing name in 1999
• Since then, problems with
• Forming letters when writing
• Typing (formerly proficient as a secretary)
• Orientating objects (purse, bread for sandwich,
  lawn bowl)
• Parking the car straight
• Folding/hanging clothes
• No problems with memory, language, concentration

47
CBD - Case Example
48
DW

• Diagnosis uncertain at first two assessments
• Initial impression - CBD, progressive apraxia or
  focal AD
• Emergence of alien hand and grasp reflexes in in
  Nov 04 pointed towards CBD

49
VASCULAR DEMENTIA
50
Vascular Dementia

• Covers a very diverse range of cerebrovascular
  events and pathologies
• Issues
• Haemorrhagic and ischaemic
• Size of infarct
• Varying foci
• Pathological substrate
• NINCDS-AIREN criteria for diagnosis

51
VaD Types

• Multi-infarct dementia
• Subcortical ischaemic vascular dementia (most
  common type of VaD)
• Strategic infarcts

52
VaD Pathology

• A broad range of pathologies
• Atherosclerotic disease
• Microvascular pathology in hypertension
• Lobar strokes
• Cerebral autosomal dominant ateriopathy with
  subcortical infarcts and leucoencephalopathy
  (CADASIL)
• Mixed pathologies will lead to mixed
  neuropsychological presentations

53
SIVD

• Occlusion of small deep perforating arteries or
  stenosis leading to reduced blood flow
• Results in white matter degeneration, esp
  periventricular
• Can also result in a frontal disconnection
  syndrome (severing fronto-striatal loops)

54
Subcortical Ischaemic Dementia/Disease

• Problems with executive functions
• Reed Mungas (2001)
• Reduced letter fluency vs AD patients
• Kramer et al (2002)
• Reduced Stroop interference performance, category
  formation on card sorting test and poorer
  initiation-perseveration in non-demented SIV
  disease vs controls

55
SIVD

• Reed Mungas (2001)
• Subcortical white matter changes / subcortical
  stroke not as strongly correlated with cognitive
  performance as grey matter changes and
  hippocampal volume
• Subcortical / white matter damage causes real
  (but modest) cognitive changes
• SIVD can present with typical AD-like memory
  changes if hippocampus is involved
• Looi Sachdev (1999)
• SIVD vs. AD
• SIVD better at long term memory tasks
• SIVD worse at executive tasks

56
SIVD Summary

• Intellect (pre-morbid and current)
• Memory
• Language
• Perceptual, visuo-spatial and praxis skills
• Executive functions
• Attention

Attention
Retrieval
Input
Storage
57
References

• Hodges (2001). Early Onset Dementia A
  Multi-Disciplinary Approach. OUP
• Lamdon Ralph et al (2003). Brain, 126, 2350-2362.
• Galton et al (2000). Brain, 123, 484-498.
• Kemp et al (2003). JNNP, 74, 715-719.
• Harvey et al (2004). JNNP, 74, 1206-1209.
• Fowler et al (2002). JINS.
• Blackwell et al (2004). Dementia Geriatric
  Cognitive Disorders, 17, 42-48.
• Swaison et al (2001). Dementia Geriatric
  Cognitive Disorders, 12, 265-280.

58
SERVICE MODELS
59
Teeswide Young Onset Dementia Team

• Memory clinic with Neurology
• Specialised community-based MDT
• Some specialised day care

60
Psychiatry
GP
Neurology
Other
Soc Serv
YODT
Neuropsychology
Medical Diagnosis
YODT Keyworker
Other
Access to services
61
YPWD
Info
Respite
Carer
Medical reviews
Support
YODT Keyworker
Therapies
Benefits
Monitoring
Day Club
Community Activities
Care packages
62
Staffing

• Neuropsychologist/Team Leader
• Team Manager
• CMHN x 2
• Social Workers x 2
• Community Support Workers x 2
• Occupational Therapist
• Day Club staff (Alzheimers Society)
• Admin staff

63
Liaison The Wider Service

• Neurology
• Regional Neurobehavioural Service
• Physicians
• GPs
• District Nursing
• SALT
• Housing
• Voluntary agencies
• Etc

64
DIAGNOSIS
65
Prevalence Estimates for YOD versus Cases
Identified

• Excludes Huntingtons disease
• Includes 12 individuals who are undergoing
  diagnostic assessment.

66
COSTS
67
Long-Term Care

• People with Huntingtons disease account for
• 30 of long-term placements, but
• 50 of the total cost of those placements

68
Long-Term Placement

• Behavioural disturbance is the single biggest
  predictor of placement in long-term care
• The two diagnostic groups characterised by
  behavioural disturbance are
• Huntingtons disease
• Fronto-temporal dementia

69
Long-Term Placement
70
Long-Term Care Costs vs. Community Costs

• Total cost of community care (specialist team
  packages of care) 8,004 p.a.
• Cost of long-term care 31,720 p.a.
• Therefore, keeping one person out of long-term
  placement for a year costs the same as providing
  a specialist community service for four people
  over the same period

71
Long-Term Placement

• These data suggest that the provision of a
  specialist, community-based team on Teesside may
  delay placement in long-term care
• On the basis of these figures, provision of
  community-based teams can be cost-effective