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Focal degenerative disorders: a clinical approach

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The late stages of many dementias are very similar ... Progressive fluent anomia 2y. Associated surface dyslexia. Features are of semantic dementia ... – PowerPoint PPT presentation

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Title: Focal degenerative disorders: a clinical approach


1
Focal degenerative disorders a clinical approach
  • Aberdeen 2007
  • Tim Griffiths
  • Cognitive Neurology Clinic, Newcastle General
    Hospital
  • http//www.staff.ncl.ac.uk/t.d.griffiths/tdg.html

Supported by the Wellcome Trust (UK
2
Key points
  • An approach to focal dementias
  • The late stages of many dementias are very
    similar
  • At the early stages dementias can have different
    psychological profiles, that can be recognised on
    basis of the history and screening psychological
    measures
  • The key to diagnosis is the recognition of those
    profiles
  • IMAGING IS NOT THE KEY TO THE DIAGNOSIS OF
    DEMENTIA
  • Early correct diagnosis has treatment
    implications and helps patients and their
    families
  • Further information http//www.staff.ncl.ac.uk/t.d
    .griffiths/tdg/html
  • cognitive neurology teaching cases

3
Structure
  • Four examples of patients who were referred to
    neurology
  • Illustration of the approach history,
    psychological features, imaging

4
Patient 1
  • He is losing his memory, doc

5
Patient 1
  • 57R
  • FH ve
  • Birth development n
  • School -gt 15
  • Electrician
  • No neurological event prior to cognitive Sx

6
Patient 1
  • 9 months of memory disturbance according to
    brother
  • Forgetting to do jobs in plant (started writing)
  • New tendency to lose keys and tools
  • Language, praxis, visiospatial skills intact
  • Increasing difficulty with complex multistage
    tasks (wired up a machine incorrectly)
  • Frustrated, mood normal, no biological features
    depression or psychotic features

7
Patient 1
  • NEUROLOGICAL EXAMINATION NORMAL
  • STRUCTURAL IMAGING WITH MRI NORMAL

8
Patient 1 Cognitive assessment
  • Screening assessment
  • MMSE 27/30 ACE 72/100
  • new learning deficit (learning of an address)
  • anterograde verbal memory deficit (3 item 1
    minute recall and 5 minute address recall)

9
Patient 1 Cognitive assessment
  • Detailed neuropsychometry intellectual function
  • WAIS III VIQ 96 (107) PIQ 117(108)
  • Detailed neuropsychometry memory
  • Striking deficits

10
Patient 1 Memory (AMIPB)
11
Patient 1
  • Striking problem with new learning, anterograde
    verbal and visual memory
  • Relative preservation of intellect
  • Diagnosis of early onset AD later developed
    more striking features in non-memory domains
  • NB1 MMSE not much help
  • NB2 diagnosis from history and
    screening/detailed neuropsychological measures
  • NB3 imaging no help (other than exclusion
    lesion)

12
Distribution pathology in typical AD (Braak and
Braak 1991)
13
Patient 2
  • He cannot work out relationships in space, doc

14
Patient 2
  • 57R
  • FH ve
  • Birth development n
  • Physical science degree
  • University Lectuerer
  • No neurological event prior to cognitive Sx

15
Patient 2
  • 1year of difficulties with spatial tasks
  • Problems with reading maps
  • Some difficulties putting clothes on correct way
    around
  • Could not work out where all the wheel nuts were
    when changing wheel
  • No Sx of forgetting spoken information or losing
    things (cf patient 1)
  • Stress in job

16
Patient 2
  • NEUROLOGICAL EXAMINATION NORMAL
  • STRUCTURAL IMAGING WITH MRI NORMAL

17
Patient 2
  • Screening cognitive assessment
  • MMSE 29/30 ACE 97/100
  • Isolated difficulty drawing intersecting
    pentagons
  • Further testing in clinic showed difficulty with
    fragmented letters and rotated shapes

18
Patient 2
  • Detailed neuropsychometry intellect
  • WAIS III VIQ 127(124) PIQ 70(125)
  • Striking verbal performance discrepancy
  • Detailed neuropsychometry perceptual testing
  • VOSP (Visual object and spatial perception
    battery)
  • Striking and significant deficit in all tests (eg
    failed on half trials of incomplete letters
    progressive silhouettes)
  • Detailed neuropsychometry memory
  • WMS III
  • Normal verbal learning and anterograde verbal
    memory

19
Patient 2
  • Striking problem with visiospatial processing
    with preservation new learning and anterograde
    verbal memory (cf patient 1)
  • Diagnosis of atypical AD later developed Sx in
    memory and other domains
  • Form of AD called posterior cortical atrophy
  • NB1 MMSE not much help
  • NB2 diagnosis from history and
    screening/detailed neuropsychological measures
  • NB3 imaging no help (other than exclusion
    lesion)

20
Posterior Cortical Atrophy
  • The pathological process in AD sometimes breaks
    the rules and does not involve medial temporal
    lobe/memory first
  • Atypical form of AD where pathology involves
    parietal lobes or ventral visual association
    areas
  • Relative sparing of medial temporal lobe in early
    stages, unlike typical course pathology in Braak
    and Braak studies
  • Syndrome 1 (parietal syndrome) Balints syndrome
    (optic ataxia, optic apraxia, simultaneous
    agnosia)
  • Syndrome 2 (ventral occipital syndrome) visual
    agnosia

21
Patient 3
  • She cannot talk properly, doc

22
Patient 3
  • 70 R
  • FH ve
  • Birth development n
  • School -gt 15
  • Shop manageress -gt 10 y before assessment
  • No neurological event prior to cognitive Sx

23
Patient 3
  • 4 year of speech disturbance

24
Patient 3
  • NEUROLOGICAL EXAMINATION NORMAL
  • STRUCTURAL IMAGING WITH MRI NORMAL

25
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26
Patient 3
  • Screening Assessment
  • MMSE 24/30 ACE score 69/100
  • Comprehension good
  • Repetition impaired least for nouns
  • Non-fluent agramatical output with phonemic
    paraphrasia
  • 10/10 for naming on Cambridge
  • Reading relatively preserved
  • Verbal fluency 2P/1minute category fluency 8
  • Detailed neuropsychometry
  • WAIS III
  • VIQ 76 PIQ 99
  • AMIPB
  • Anterograde visual memory normal
  • Anterograde verbal memory poor 

27
Patient 3
  • Progressive non-fluent aphasia gt 2y
  • No deterioration in language independent
    cognitive function NB verbal memory
  • She meets criteria for Primary Progressive
    Aphasia, a focal dementia of the left hemisphere
  • In terms of underlying, pathology, this can be an
    atypical form of AD, but is more commonly a form
    of frontotemporal dementia

28
Patient 4
  • He is losing his ability to name anything, doc

29
Patient 4
  • 57 R clerical worker
  • Two year difficulty with naming
  • Neurological examination normal

30
Patient 4
  • Screening assessment
  • MMSE 23/30 ACE score 51/100
  • Comprehension good
  • Repetition normal
  • Fluent output
  • 0/10 for naming
  • Surface dyslexia
  • Detailed neuropsychometry
  • WAIS III VIQ 80 PIQ 107
  • Other tests
  • 43/52 Pyramids and palm trees, three picture
    version

31
Patient 4
32
Patient 4
  • Progressive fluent anomia gt 2y
  • Associated surface dyslexia
  • Features are of semantic dementia
  • Not associated with AD pathology
  • Associated with one of the pathological subtypes
    of frontotemporal dementia
  • A focal dementia affecting the lateral temporal
    lobe (cf typical AD affecting medial temporal
    lobe)

33
(No Transcript)
34
Dementia presenting with focal language
disturbance
  • NON-FLUENT
  • (Patient 3)
  • PPA (Mesulam 1982)
  • Output language gt 2y
  • No other domains
  • NOSOLOGICAL CATEGORY
  • Diagnosis
  • 60 non specific gliosis
  • 20 Pick histology
  • 20 AD (atypical sparing HC)
  • Imaging
  • VBM gt L perisylvian atrophy
  • 1. Posterior superior temporal lobe
  • 2. Inferior parietal lobe
  • Sonty et al
  • Ann Neurol 20035335-49
  • FLUENT
  • (Patient 4)
  • Semantic dementia PPA(fluent)
  • Progressive fluent aphasia
  • Surface dyslexia (prosopagnosia)
  • Diagnosis
  • Always one of histological variants associated
    FTD
  • Imaging
  • VBM gt
  • 1. AP gradient of atrophy
  • 2. frontal atrophy
  • Mummery et al
  • Ann Neurol 20004736-45

35
Focal temporal lobe dementia
  • Recent imaging research

36
Primary Progressive Aphasia (patients like
patient 3) Categorical 22 patient vs control
(RFX) Sonty et al (NorthWestern group) Ann Neurol
20035335-49
37
Semantic dementia (patients like patient 4) 6
patient (individual FFX) Mummery et al Ann Neurol
20004736-45
38
Focal dementia
  • The practical message

39
Focal dementia YOU DO NOT NEED IMAGING TO
DISTINGUISH THESE CONDITIONS
R
L
PATIENT 3 ALZHEIMERS OR FTD PATHOLOGY LEFT
POSTERIOR SUPERIOR TEMPORAL LOBE ? non-fluent
aphasia
PATIENT 2 ALZHEIMERS PATHOLOGY PARIETAL
LOBE ? spatial sx
PATIENT 1 ALZHEIMERS PATHOLOGY HIPPOCAMPUS ?
memory sx
PATIENT 4 FTD PATHOLOGY LEFT LATERAL TEMPORAL
LOBE ? fluent aphasia
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