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Glasgow, November 2009. Semantic and frontotemporal dementias. Julie Snowden. Cerebral Function Unit Greater Manchester Neuroscience Centre Salford Royal Foundation Trust. Historical Background. Clinical descriptions 1900s Pathology 1920s

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semantic and frontotemporal dementias

Glasgow, November 2009

Semantic and frontotemporal dementias

Julie Snowden

Cerebral Function Unit

Greater Manchester Neuroscience Centre

Salford Royal Foundation Trust

slide3

Historical Background

Clinical descriptions 1900s

Pathology 1920s

Focal cerebral atrophy 1980s

Localisationist

Holistic

Gestalt psychology

Specialisation of function

Neuropsychology

slide4

Spatial orientation (mental map)

Praxis

Alzheimer’s disease (AD)

Executive functions

Social cognition

Parietal

Frontal

Visual perception

Occipital

Temporal

Language

Frontotemporal lobar degeneration (FTLD)

Episodic memory (events)

slide5

Frontotemporal Lobar Degeneration

Progressive aphasia

Fronto-temporal dementia

Semantic dementia

overview of frontotemporal lobar degeneration
Overview of Frontotemporal Lobar Degeneration
  • Onset in middle years (45-65)
  • Insidiously progressive
  • Affects both men and women
  • High familial incidence
  • Median duration 8 years (3-20)
clinical features
Clinical features
  • Few neurological signs  striatal signs

but

  • May be associated with MND
  • Routine EEG normal
  • Focal change on structural / functional imaging
frontotemporal dementia
Frontotemporal dementia
  • Breakdown in social, interpersonal conduct
  • Neglect of self-care and responsibilities
  • Emotional blunting, loss of insight
  • Repetitive, stereotyped behaviours
  • Hyperorality, dietary changes
slide11

Plans/Goals

Checking

Organisation

Attention

Abstraction

Flexibility

New plan

slide12
Planning

Organisation

Attention

Abstraction

Checking

Flexibility

Inertia/purposeless overactivity

Disorganised behaviour

Inattentive, distractible

Concrete

Poor self-monitoring, impaired insight

Inflexible

Loss of sympathy and empathy

weigls blocks
Weigls blocks

Perseverative Dismantling behaviour

shape, colour, motif

slide15

Brixton spatial anticipation test

2

3

4

5

1

6

7

9

10

8

Burgess & Shallice, 1997

slide16

Picture sequencing

Verbal fluency

  • Animals
  • “Billy the budgie
  • and Spot the dog”
  • F words
  • “Frank – my brother”

Concrete, rule violations

slide17

Design fluency in FTD

novel, abstract, 4-lines

Perseverations, rule violations, concrete

slide19

Dorsolateral

Apathy, inertia

Blunting

Executive impairment severe

Phenotypic variation in FTD

Orbital

Disinhibited, fatuous

Executive impairment mild

slide23

AD copy

FTD copy

slide24

MRI coronals

SPECT

coronal

sagittal

FTD

slide25

MRI coronals

SPECT

coronal

sagittal

AD

qualitative performance characteristics in ftd
Qualitative performance characteristics in FTD
  • Impulsivity, inattention
  • Economy of effort, lack of persistence
  • Concreteness of thought
  • Perseverative response
  • Unconstrained by goal – rule violations
frontotemporal lobar degeneration

Non-tau

Ubiquitinated neurones

microvacuolation

Tau

Pick bodies in neurones

Pick cells

Neurofibrillary tangle-like

in neurones and glia

Frontotemporal lobar degeneration

Ubiquitin protein:

TDP-43 protein

(Arai et al. 2006)

semantic dementia overview

Parietal

Frontal

Occipital

Temporal

Frontotemporal lobar degeneration (FTLD)

Semantic dementia: overview
  • Circumscribed, multimodal disorder of meaning
  • Bilateral, often asymmetric degeneration of the anterior temporal lobes -especially inferior temporal gyri
  • Part of spectrum of FTLD

SD

slide32

Semantic dementia

Impaired

Word meaning

naming

comprehension

phonology

syntax

speech fluency

Preserved

slide33

Language

Naming

“A nice little dog”

Superordinate, coordinate, descriptive, omission errors

No TOT effect/effortful word search

No benefit from phonemic cueing

Comprehension

“rabbit ..what’s rabbit?”

Partial/degraded knowledge

Which is the rabbit?

X

slide34
Impaired

Recognition of:

faces, objects

nonverbal sounds

smells, tastes

tactile stimuli

Perception of

sensory stimuli

Semantic loss - multimodal

Preserved

slide35

Perceptual matching 

Category matching X

nature of memory impairment
Nature of memory impairment

word and object meaning

facts, world knowledge

memory for

autobiographical events

Impaired

Preserved

slide38

SEMANTIC DEMENTIA

Bilateral temporal lobe atrophy

behaviour in semantic dementia
Behaviour in semantic dementia
  • Narrowed behavioural repertoire
  • Preoccupations
    • Personal hobby e.g. painting, dress making
    • Word and number games, jigsaws, Sudoku
  • Preference for routine
    • Clockwatching, time bound

More compulsive than frontotemporal dementia

narrowed world view
Narrowed world view
  • Self-centredness
  • Reduced sympathy and empathy
questions
Questions
  • What determines what is lost and retained?
  • What is the relationship between knowledge in different modalities? – amodal vs. multi-modal models
  • What is the relationship between knowledge and experience (semantic-episodic memory)?
slide42

rabbit

?

Animal

famous face and name recognition
Famous face and name recognition

id = provides identifying information

fam = reports familiarity

slide48

MR and SPECT imaging

Left > right

Right > left

name and face identity
Name and Face Identity

% correct

Temporal lobe atrophy

name and face familiarity
Name and Face Familiarity

%

Temporal lobe atrophy

slide51

Margaret Thatcher

Elvis Presley

Face and name identity and familiarity influenced by distribution of temporal lobe atrophy in left and right hemisphere

Snowden et al. Brain 2004; 127: 860-72

factors influencing what is known not known
Factors influencing what is known/not known
  • Frequency, familiarity, typicality
  • Animals: cat, dog, horse

“A nice little dog”

  • Personal familiarity?
slide54
SD patient performance

Graded Naming test 0 / 30

Conversational speech:

“I help Reverend Jones prepare the chalice for the Eucharist. Afterwards, I put the chalice back in the vestry”

slide55

What’s this?

“It’s for telling the time. It’s twenty-four minutes past two”

?

who is
Whois?

current

past

current

past

Personal

Impersonal

Snowden et al. Cog Neuropsychol 1994;11:265-88.

place name recognition
Place name recognition

What does this word mean?

horse

boat

london

skirt

france

scissors

hat

cambridge

slide61

Auvergne(part of France patient had visited)

“It’s in France, by the Dordogne. We used to go there in the caravanette”

Portugal (country not visited)

“What’s Portugal? I’ve never heard of Portugal?”

slide62

Object recognitionEvidence of experiential effects

  • Superior recognition of own objects
  • Superior object recognition in usual context
  • Recognition influenced by personal usage
    • - clothes peg: for closing cereal packet
slide63
Word comprehension

Evidence of experiential effects

Partial knowledge limited to own experience

slide64

Factors influencing what is known/not known

  • Personal familiarity
  • Relevant to current life

Name identification

reversed temporal gradient in retrograde memory
Reversed temporal gradient in retrograde memory

AMI

personal semantics

autobiographical incidents

slide66
Semantic memory

Knowledge of words,concepts facts about the world

- not tied to temporo-spatial context

- acquired early in life

- culturally shared

Episodic memory

Memory for events

- tied to temporo-spatial context

slide67

temporal neocortical memory system

Semantics

words, concepts, facts

episodes, events

medial temporal memory system

slide68

Concept

Dog

small black tame

medium brown wild

large white

small black tame

Experience Dog

Linked to time and space

slide69

temporal neocortex

decontextualised knowledge

contextualised knowledge

autobiographical

facts

events

medial temporal lobes

is learning re learning possible
Is learning/re-learning possible?
  • Factors affecting learning
  • Object recognition
  • Spatial context
  • Temporal context
  • Link to daily experience

Rehearsal necessary to maintain information

slide71

duck

“You see this on the lake when you go for a walk in the park”.

memory for object names
Memory for object names

Snowden et al. Neuropsychologia 2002;40:1715-1728.

practical implications
Practical implications
  • Link objects/words to daily life experience
  • Importance of routine – time and place
slide74

Focal Cerebral Degeneration

Progressive aphasia

Fronto-temporal dementia

Semantic dementia

slide75

Phonology

Syntax

Semantics

progressive non fluent aphasia
Progressive non-fluent aphasia
  • Impaired language expression
  • Comprehension relatively preserved
  • problem in lexical access
  • problem in phonology
  • problem in generation/organisation of sentences
slide77

PA CT

CT scan of patient with non-fluent PA

R

L

frequency of clinical syndromes of frontotemporal lobar degeneration
Frequency of clinical syndromes of Frontotemporal Lobar Degeneration

PA 11%

SD 16%

Based on 245 patients

FTD

59%

FTD/MND 14%

slide83

Subtypes of Ubiquitin Pathology

Type

1

2

3

cytoplasmic inclusions

in cerebral cortex

neurites in cerebral cortex

Type 1

Type 2

Type 3

cytoplasmic inclusions

and neurites in cortex

cytoplasmic inclusions

In hippocampus

Cat’s eye or lentiform

intranuclear inclusions

molecular basis of ftld
Molecular basis of FTLD

Mutations in tau gene on chromosome 17

Mutations in progranulin gene on chromosome 17

Mutations on chromosome 3 in Danish family

Linkage to chromosome 9 in FTD/MND

conclusions
Conclusions
  • Distinct clinical syndromes reflecting distribution of atrophy
  • Confused with Alzheimer’s disease because of:
    • history of memory complaints – semantic dementia
    • neuropsychological test scores – FTD
  • Need to take account of qualitative features
conclusions cont
Conclusions cont.
  • Neuropsychology important
    • in characterising disorder
    • in understanding neurobiology
  • Degenerative disease valuable model for understanding brain function
    • High anatomical and functional specificity