Beyond the Scores: Clues to neuropsychological disorders from the things patients say and do

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Beyond the ScoresClues to neuropsychological
disorders from the things patients say and do

• Karalyn Patterson
• MRC Cognition Brain Sciences Unit
• Cambridge UK
• Moss Rehab, September 2009

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Unusual talk Examples of things patients say and
do in real life or in clinic or in testing that
are informative about the impaired abilities

• Made possible by seeing patients and their
  families in clinic

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Structure of each example

• 1. Context What was happening at the time?
• 2. Observation What did patient say or do in
  response?
• 3. Message What can we learn from response?
• 4. Sometimes Formal testing that corroborates
  this message
• Formal/informal messages not discrepant!
• But such observations can encapsulate a point or
  serve to direct further research
• 5. Sometimes Why is this behaviour a puzzle,
  or what questions does it raise?

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PatientsMainly semantic dementia (SD)Some
semantically impaired cases with Alzheimers
disease (AD)
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SD Central semantic/conceptual disorder

• ? Affects all modalities of input/output
• ? Affects virtually all forms of knowledge
• ? Apparent order of severity
• expressive language worst
• receptive language
• object recognition/use
• but probably explicable by task demands and
  nature of peripheral-central mappings

box
fox
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Context Please name this

• Response (PS) Its a horse, aint it?
• Then pointing to stripes But what are these
  funny things for?
• Message
• The anomia in SD is not word-finding difficulty
• Formal Testing High degree of consistency across
  occasions and types of testing

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Context AM and his wife get out of their car in
the pouring rain, she hands him an umbrella

• Observation reported later by wife
• He holds the umbrella above his head horizontally
  and without opening it
• Messages
• 1. SD is not a language disorder!
• 2. Deterioration of semantic memory is graded and
  partial

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Context Bruce and his wife driving through the
countryside to visit friend after gap of years

• Observation reported later by wife
• (a) Bruce to wife You have to turn into that
  small place up ahead Correct and wife
  surprised. Then a few minutes later
• (b) Bruce to wife what are those things?
  pointing to sheep in field
• Messages/questions
• 1. Selectivity of disorder visuospatial skills
  and this aspect of episodic memory unaffected
• 2. Profound agnosia/anomia
• 3. horse vs what is that?

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Context object/concept definitionsWhats a
seahorse?

• Response (AM) I didnt know they had horses in
  the sea
• Message Deterioration of knowledge is strongly
  predicted by familiarity /or age of acquisition
  and by typicality
• Formal Testing everything!

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Context naming pictures of animals

• Responses (DG)
• dog, cat, horse -gt correct
• pig, squirrel, sheep, deer, goat, fox etc -gt
  dog
• mouse, rabbit, monkey -gt cat
• lion, zebra, rhino, bear -gt horse
• seahorse -gt little thing
• fish, frog -gt dont know
• Message
• Typicality not only predicts which responses will
  be correct, but also nature of errors

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Context naming pictures of animals

• Responses (DG)
• mouse, rabbit, monkey -gt cat
• pig, sheep, deer, goat, kangaroo -gt dog
• rhino, lion, zebra, bear -gt horse
• Message Errors partially respect true size
• Puzzle Is the size information in the stimulus
  or in the patient?

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Context Conversation in clinic

• JRH How do you spend your time during the days?
• AT Im very good at these eleven thousand..
  Things which you put all together to make
• Message Selectivity of disorder again
• Question What combination of factors makes this
  activity so well suited to the remaining
  abilities in SD?
• Talking to AN about jigsaw he was currently
  doing
• JRH Whats it a picture of?
• AN I dont know, I didnt look.

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Context After AT has done and been praised for
some correct mental arithmetic

• Response (AT) Yes, that seems to work okay,
  thats the odd thing, not everything is off but
  some things are so awful and yet other things
  will look all right
• Message Selectivity of the disorder again
• Some interesting Qs
• Why are numbers special?
• What aspects of number knowledge are preserved in
  SD?
• Is good digit span attributable to content?

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Contrast semantically impaired AD patients
Context Informal assessment in clinic

• JRH If you ordered a pint of beer in the pub,
  and it cost 2.50, and you gave the barman 10,
  how much change would you get?
• DCJ Oh, I wouldnt give as much as that!
• JRH Okay, suppose the beer costs 2.50 and you
  give him 5
• DCJ I dont drink beer
• JRH How long have you been on your own? JC is
  divorced
• JC Oh, have I got to go back about 3,000
  years?
• JRH When is your birthday?
• JC I forget I wish you people had told me
  before I got here...
• JRH Let me put it another way what year were
  you born?
• JC Well, I wasnt very old at the time, so I
  didnt sort of get it
• Message AD patients worse than SD at number
  skills
• but pretty good at trying to get round the
  questions!

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Context Please name this

• AM Its in that country down in Africa... in
  the upper right-hand corner I can show you on my
  thing over there
• Message
• Some preservation of geographical knowledge?
• Puzzle Why?

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Context Conversation or informal assessment in
Clinic

• JRH Where does your daughter work?
• AT At the University of er starts with an M,
  the big important one in the north of England
• JRH Whos the US president? in 2005
• AN Its such a short name I dont know does
  it begin with B?
• Message Sometimes preservation of initial letter
  or sound knowledge
• Why is this a puzzle? Because SD patients benefit
  so little from phonological cueing

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Context Conversations in clinic

• IF talking about his current difficulties
• Well, some of my friends have given myself away
  from them, because I cant talk to them
• JW talking about the pub where he has his
  meals Its very nice, and the steak-and-kidney
  pie is only 4 JRH Do you always have that?
  JW Not every day, but ever such a lot... I do
  have other ones you know what they put certain
  things up
• JB difficulties I want to say you right now
  that this is whats wrong
• Question Is syntax really independent of
  semantics?

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Context Conversation in Clinic, JRH has just
asked about patients problems

• Response (AC) Im playing golf next week, for
  example, er... in an annual syndicate
  competition we have with people I used to, er...
  work with, and at the moment, I cant remember a
  single name of them
• Message Impact of concept level
• Usually in SD, general gt intermediate gt specific
• What could be more specific than individual
  people and their names?
• Some interesting Qs
• Problem naming or knowing?
• Just specificity effect or are people special?
• LgtR vs. RgtL temporal abnormality

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Context Reports from wives of 3 male SD patients

• One patient started to ask his wife What is this
  funny stuff that keeps growing on my face?
• Two patients continued to shave their faces but
  also began to shave hair on other parts of their
  bodies
• Messages
• (a) Beards are at a very specific level of the
  sem syst!
• (b) One major function of semantic memory is
  generalisation and differentiation. When
  semantics deteriorate, not only knowledge e.g.
  about hair declines, but the ability to
  differentiate between different forms/locations
  of hair

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Context Patient being tested on CatFlu

• DC Please tell me the names of as many animals
  as you can think of
• LF dog, cat Claude died 3 weeks ago...
  their dog
• Message
• Recent autobiographical events not only salient
  for SD patients but invade their semantic memory

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Context Conversation in Clinic

• JRH What do you do in the day?
• IB I take our dog for a walk
• JRH What kind of dog do you have?
• IB A Rhodesian Ridgeback
• JRH Do you have any cats?
• IB Cats cats Im not sure what those are
• Message Facilitation -- even of LoFreq items --
  from personal exposure/familiarity/ usage
• Needs to be recent and regular surgeon poor
  naming/matching of surgical instruments chemist
  could not define element psychiatrist
  regularised Jung, Freud placebo in reading
  aloud
• Question Impact on what?

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Context Conversation followed by Definitions

• JRH What sort of work did you do?
• BH I commanded a team on bulk petroleum
• 5-10 minutes later
• JRH petroleum, can you say that? BH can and
  does
• JRH What is petroleum?
• BH .. then hesitantly Something that uses
  petrol?
• 
  
• DV talking about his big trees with something
  on them to eat But last year we didnt have
  anything on 4 of the trees, because we had a late
  frost
• 5-10 minutes later
• JRH What does frost mean? DV Frost frost
  whats that?
• Message
• (a) Lack of generalisation between contexts
• (b) Words they use frequently can mislead!

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Best example yet in domain of language

• IB talking about a big football match involving
  the team he supports
• IB Its wonderful, my friend, to have a 60
  minute entertainment, and if your team wins or
  loses, youll be euphoric or suicidal, but by the
  next day then 5-10 minutes later
• KP Mr B, what does euphoric mean?
• IB Phoric phoric I like to phoric?
• euphoric you phoric!

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Facilitation by recent, regular, specific
experience not confined to words

• Sudoku
• Example courtesy of Raquel Sanchez, Barcelona

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Context Conversation before testing

• As PP comes into the testing room and her husband
  disappears off down the corridor
• PP When is she coming back?
• Message Disintegration of sex/gender knowledge
• Question Is it sex or gender?
• Puzzle either way, since both seem so very
  fundamental

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Context 2pm at home, JL is impatient to go to
dinner at Chinese restaurant

• Observation reported later by Mrs L
• JL Cant we go out to eat now?
• Mrs L Darling, not yet! Then pointing to the
  kitchen wall-clock We go at 7pm.
• A little later, she returns to find JL standing
  on the kitchen table moving hands of clock to
  700.
• Message Partial knowledge again
• Why a puzzle?
• Such an striking combination of understanding and
  not-understanding

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Summary 1 Phenomena that we understand, or at
least have hypotheses about

• Selectivity of disorder
• Graded nature of deterioration
• Profound anomia ? WFD
• Good number skills
• Typicality effects rampant
• Specificity or levels effect
• Invasion of semantic memory by autobiographical

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Summary 2 Phenomena that we are still groping to
understand

• Deterioration of gender/sex knowledge
• Relationship between semantics and syntax
• Problem with people their names just
  specificity?
• Occasional partial phonological/1st-letter
  knowledge
• (a) just more semantics? (b) proper names?
• Partial geographical and size knowledge
• Lack of generalisation/words in context vs.
  isolation
• Benefit of personal exposure
• (a) on what? (b) expertise
• Mixture of understanding and not clock example

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My thanks.

• To John Hodges and Kate Dawson at the Cambridge
  Specialist Dementia Clinic, and to the patients
  and their carers, for allowing me these
  opportunities to observe
• To you, for listening and I hope discussing