This presentation by Dr Anita Rose, Consultant Neuropsychologist, looks at cognition and MS. It explores assessment, managing cognitive deficits and factors assessing cognition including pain, emotions and fatigue. It was presented at the MS Trust Annual Conference in November 2013.

1. COGNITION AND MS
Dr Anita Rose
Consultant Neuropsychologist

2. Aims/Learning Objectives
• Cognition and MS
• Is it really an issue?
• Assessment
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Is assessment worth it?
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I am not a neuropsychologist so what can I do?
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Assessments
• Cognitive domains
• Neuropsychological/Cognitive profile of MS
• Managing cognitive deficits
• Specific strategies
• Factors affecting cognition including:
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Emotions
Fatigue
Pain

3. Facts and Figures!
• Prevalence
• Not everyone with MS experiences cognitive problems
• As early as the 1800‟s, Jean-Martin Charcot described the
cognitive changes that can occur in MS.
• “There is marked enfeeblement of the memory; conceptions are formed
slowly; the intellectual and emotional faculties are blunted in their totality.
The dominant feeling in the patients appears to be a sort of almost stupid
indifference in reference to all things. It is not rare to see them give way to
foolish laughter for no cause, and sometimes, on the contrary, to melt into
tears for no reason. Nor is it rare, amid this state of mental depression, to
find psychic disorders arise which assume one or other of the classic forms
of mental alienation”
• Yet took clinicians until 2001 to codify a standard test to
measure cognitive function

4. • 1980s research indicated otherwise (Bobholz &Rao 2003)
• 40% - 60% have cognitive deficits
• 80% are mildly affected
• Relate to everyday activities and quality of life
(Higginson et al. 2000)
• Variability (Rao, 1986)
• 50% - 75% unemployed within 10 years of diagnosis
– cognitive issues being reported as leading predictor
(Julian et al., 2008) compared to age, physical disability
& education (15%)

5. Dementia?
• Overt dementia in MS is rare
• Most cases of cognitive impairment in MS are relatively
less severe than those observed in classically dementing
neurological disorders, such as Alzheimer‟s disease
• However, cognitive impairment in MS can be extremely
debilitating, with substantial negative impacts on daily
living

6. Pathology
• Disconnection of large cortical areas and between cortical
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and subcortical structures (Filley, 1989)
“Multiple disconnection syndrome” (Calabrese, 2006)
White matter volume & corpus callosum size correlate
with cognitive dysfunction (Rao 1985, 1989, Hulst et al., 2013)
Lesion burden (Swirsky & Sacchetti, 1992)
Biological in basis (Hurst, 2013)

7. “Hidden” Symptom
• PwMS can present with relatively well-preserved
language and social skills, but sometimes a
marked difficulty with problem solving and insight
• Having preserved language and social ability
means that in ordinary conversation, cognitive
problems may not be apparent
• Might not be apparent on “bedside testing”
• Stigma – within family and wider society
• Fear – PwMS
• Therefore cognition as a symptom is often
ignored

8. Impact
• Frightening
• Think they are going mad, are stupid or are crazy
• Causes problems in relationships, with family life
• Major impact can be seen in employment
• Later stages concerns around safety, independence,
ability to self-care
• Insight

10. ASSESSMENT – IN OTHER
WORDS WHAT ISN‟T
WORKING?

11. Clinical Questions
• Who needs to be assessed
• What cognitive domains need to be covered
• With what frequency should the assessments take place
• Strategies for assessment related to purpose
• Comprehensive: Assessment for rehabilitation, benefits,
vocational planning
• Detection: Identification of individuals showing deficits
• Monitoring: Assessing cognitive changes as a treatment
outcome

12. Assessment & Intervention
• Assessment at Point of diagnosis and Beyond
• Cognitive deficit might be present in early stages (Simioni et al.,
2007)
• Longitudinal studies suggest cognitive deficit could be used
as predictive parameters of MS evolution and severity (Amato
et al., 1995 & 2001; Kujala et al., 2001)
• Impairment predictor of low quality of life, employment issues,
carer stress
• Assessment assists patient, family, healthcare professionals
(Roig & Bagunya, 1997)
• Treatment
• Cognitive rehabilitation
• Aim to improve quality of life
• Environmental adaptation
• Increase autonomy

13. Help I am not a neuropsychologist
• But you are a trained healthcare professional!
• Clinical interview
• Ask questions
• Screening tools:
• Brief
• Inexpensive (or free!)

14. Screening Approaches
• Number of cognitive screens:
• MSNQ (MS Neuropsychological Screening Questionnaire)
• MMSE (Mini Mental Status Examination)
• BRB-N (Brief Repeatable Battery-Neuropsychological
• BNPB (Brief Neuropsychological Battery)
• SEFCI (Screening Examination for Cognitive Impairment)
• RBANS (Repeatable Battery for the Assessment of Neuropsychological Status)
• BSB (Basso Screening Battery)
• MACFIMS (Minimal Assessment of Cognitive Function in MS)
• ANAM (Automated Neuropsychological Assessment Metrics)
• Expensive
• Need to be trained i.e. Neuropsychologist
• American

15. MSNQ
MS Neuropsychological Screening Questionnaire
• Time
• 5 min (patient and informant)
• Results
• Reported symptoms of cognitive and behavioral problems
• Repeatability?
• Utility as change measure not established
• Sensitivity/Specificity (Informant)1
• Sensitivity: .83
• Specificity: .97
• So good measure?
• Subjective report – no objective results but supports clinical interview
• In public domain
[1] Benedict R et al (2003) Mult Sclr. V9 95-101

16. MMSE
Mini Mental Status Examination
• Time
• 5-10 minutes
• Results
• Global summary score
• Repeatability?
• Single form so practice effect
• Sensitivity/Specificity1
• Sensitivity: 21-36% MS
• Generally poor with specific or subcortical lesions
• Specificity: 89-100%
• So good measure?
• Only picks up global damage
• No longer free
• Poor results in MS
[1] Fischer JS (2001) in SD Cook (Ed) Handbook of MS 3 rd ed.

17. Montreal Cognitive Assessment (MoCa)

18. • Time
• 10 mins
• Results
• Global Score but can consider subtests
• Repeatability
• Single form ?practice effects
• MS?
• Number of papers suggest valid for population e.g. Dagenais et al.
2013; Kumar et al. 2012;
• Good measure?
• Visual spatial might be a problem but can be considered when scoring
• Easy to administer
• Free
• Norms available
• No qualifications needed

19. BICAMS
• BICAMS (Brief International Cognitive Assessment for MS) is
an international initiative to recommend and support a cognitive
assessment that is brief, practical and universal
• Consists of:
• The Symbol Digit Modalities Test
• The California Verbal Learning Test –II, first five recall trials
• The Brief Visuospatial Memory Test –Revised, first three recall
trials
• Takes 15 minutes to administer
• All the tests comes from established tests which you have to
purchase
• To purchase you have to be qualified tester – Level 2 e.g.
neuropsychologist
• But worth watching to see what happens
• http://www.bicams.net/

20. “Stupidity is not a handicap – park elsewhere”
Yvette

21. NEUROPSYCHOLOGICAL
PROFILE:
WHAT CAN I EXPECT TO SEE?

22. Cognitive Impairment in MS
• Processing speed
• Attention/concentration
• Sustained
• Complex
• Memory
• Episodic/recent memory
• Working memory
• Executive functioning
(EF)
• Abstract reasoning
• Problem-solving
• Language
• Verbal fluency
• Naming
• Visuospatial skills

23. Information Processing
• Primary deficit (Rao et al., 1991)
• Significant impact (Langdon &
Thompson, 1996)
• Evidence indicates that people
with MS require a longer time to
digest new information
• Takes longer to process
information and formulate the
proper response
• Accuracy rarely affected, just
takes longer (Demaree, 1999)
• Takes longer to complete tasks
• Given additional time = improved
accuracy
• Working memory deficits due to
impaired speed

24. Attention
• Attention = vigilance, capacity for information, switching
attention, selective attention
• Research produced inconsistent findings (Higginson et al.
2000)
• Definition of attention
• Measuring instruments very varied
• However all note Attention difficulties:
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Selective
Divided
Sustained
Alternating
• 20-25% of MS patients
• Deficits in rapid and complex info processing
• Working memory
• Attentional switching
• Rapid visual scanning
• Intact attention span (if not distracted!)

25. Attention
• People with MS report:
• Becoming easily distracted
• Having trouble keeping track of what is being said and done or have
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trouble making sense of things
Having trouble focusing on one person, thing or conversation in
crowded environments
Having trouble keeping track of more than one thing at a time
Having difficulty doing more than one task at a time
Having difficulty learning and remembering information
Becoming easily frustrated with yourself and others
Feeling confused and overwhelmed
Avoiding contact with care givers, friends and family
• All of the above are not helped if distracted
or interrupted

26. Language
• Mild confrontation naming deficits
• Speech abnormalities (dysarthria, hypophonia)
• Poor verbal fluency (retrieval deficit/speed)
• 20-25%

27. Visual Processing
• One study suggests 26% impairment
• Visual processing (Warren, 1993)
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(Vleugels, 2000)
Visual Cognition
Visual Memory
Pattern Recognition
Scanning
Visual Attention
• In people with MS impairments reported as:
• difficulty in recognising objects accurately
• difficulty in reading maps
• difficulty in driving
• problems finding way around
• ability to draw or assemble things is impaired

29. Memory in MS
• 40 – 60% report memory deficits (Brassington & March,
1998)
• On assessment (Beatty et al., 1996):
• Mild – Moderate = 53%
• Severe 22%
• 25% show normal performance
• Core deficit in MS
• Global affects can be seen
• Recent memory
• Retrieval
• Encoding
• Working memory (? Slowed processing)
• Recognition and Implicit relatively unaffected

30. • Most people describe:
• Forgetting appointments
• Forgetting shopping lists or things have to do
• Forgetting what someone has just said
• Reading the newspaper and then forgetting what read

31. Model of Memory

32. Memory Model for the layman!
Attention
Attention must be paid in order
to be able to learn
Recording
The brain needs to 'take in'
and record information
The 3 R’s
of Memory
Retain
The information needs to
be stored in the right spot
Retrieve
Information needs to be
recalled when it is needed again
If problems occur anywhere
in these steps, then
memory difficulties will
occur

33. Executive Functioning
• Cognitive abilities
required to complete
goal-directed
behaviors that are not
automatic,
overlearned, or routine
(Sohlberg & Mateer, 2001).

34. Executive Functioning
• 15-20% of patients with MS exhibit executive
dysfunction
• Impaired goal-directed behavior
• Verbal disinhibition
• Poor self-monitoring (e.g., tangential speech)
• Reduced insight
• Deficits in planning and prioritizing
• Problems with abstraction and conceptualisation

36. Executive Functioning
• Initiation
• Sequential processing
• Inhibition
• Planning
• Set-switching
• Self-Monitoring
• Judgment/Reasoning
• Perseveration
• Goal identification
• Prioritising
• Working memory
• Multi-tasking
• Speed of processing
• Emotional control
• Cognitive
• Insight/Awareness
flexibility/problem-solving

37. MANAGING COGNITIVE
DEFICITS I.E. INTERVENTION

38. Managing Cognitive Deficits
• Neuropsychological rehabilitation
• Interventions aiming to enhance or support cognitive abilities
following brain injury, with an emphasis on achieving functional
changes (Sohlberg & Mateer, 2001)
• Target: reductions in cognitive, emotional,
psychological functioning that encumber
everyday functioning
• Goal: increase independent functioning by means
of enhanced knowledge and skill, behavior
change, or implementation of compensatory
strategies

39. • Foundation of cognitive intervention
• Tx based on current level of function
• Build on strengths to support weaknesses
• Collaborative
• Goal-oriented
• Education

40. Processing Speed
• Complete one activity
at a time
• Schedule more time to
complete tasks
• Limit distractions
• Record information for
later review

41. Attention
• Orienting procedures
• “What am I doing?”
• Minimises gaps in attention
• Pacing
• Realistic expectations
• Elongated performance times
• Minimise frustration – be patient!
• Vary according to time of day
• Schedule adequate rest

42. • Environmental modification
• Work in a quiet environment
• Reduce clutter
• Limit distractions
• Refer to checklists to complete tasks
• Set timers to prevent going overtime
• Work on one task at a time
• Double/triple check work to minimise errors
• Have a significant other check work

43. •
Top Ten tips to help manage attention difficulties:
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Practice
Check in
Modify the environment
Pace
Self care
Monitor mood
Double check
Break tasks down
Do difficult tasks at the best time of day.
Use family and friends for support.

44. Language
• Language
• Communication skills training
• Group interventions
• Modeling and generalization
• Building social networks
• In MS, many language deficits are due to physical changes (i.e.,
dysphagia) and reduced speed of processing.
• Allow more time for communication

45. • Speech and Language referral if required
• Other forms of communication may be available
• Writing, drawing, gesture
• Communication book with pictures
• Communication aids
• Use consistently, have equipment available
• Carry explanatory card

46. • Allow extra time
• Ask questions in a way that requires
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only short answers
Speak more slowly
Keep sentence short and simple
Avoid non-literal speech
One person talk at a time
Encourage people not to provide the word
Encourage self-cueing
• Using the first letter of a word
• Going through different categories and sub-categories
Encourage use of talking around the subject
• Describe it
• Talk around it
• Don‟t get hung up on finding the right word
• Getting the message across it what is important
Give them permission to use gestures
Teach the strategy of asking 20 questions

47. Helping Visual spatial Difficulties
• Raise awareness by providing feedback
• Teach self-monitoring
• Promote compensation and self management so predictions can be
made
• Keep walking into doors
• Put tram lines of tape on the floor to walk between
• Forgetting passages or missing the end of sentences when reading
• Use a piece of paper to cover text and guide reading
• Reading Rulers
• Reduce clutter
• Keep things in consistent places
• Increase contrast
• Scanning
• Dark objects on white background or vice versa
• Coloured tape on sharp corners
• Use written and visual cues

48. Memory:
Learning Strategies
• Repetition
• Repeat the information
over and over and over
again.
• Looking at something one
time is never enough.
• For example, trying to
learn someone‟s name,
repeating it over and over
can aid remembering

49. Multimodal learning
• It helps to learn the same
information in different
ways.
• For example, to learn a
new recipe, it helps to read
over the steps in the
recipe, listen to someone
telling you the steps (or
reading it aloud yourself),
and practice the recipe by
doing it.
• See it, hear it, do it!

50. Errorless Learning
• This is a method of learning which unlike more traditional
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teaching ideas does not encourage guessing.
If attempting to help someone remember a piece of
information it is common practice to suggest that they
„have a guess‟.
This technique suggests that if information cannot be
recalled on the first time of asking, the correct answer
should be given immediately.
Replacing several guesses with the correct answer may
lead to the information being remembered.
This information is less likely to be remembered if several
incorrect guesses are made, as these could later be
recalled by the person with memory problems.

51. • Example of Errorful Learning
• Example of Errorless
Learning
Carer: „Can you remember the
name of your granddaughter?‟
Tom: „Erm...I‟m not sure‟
C: „Why don‟t you have a
guess?‟
T: „Is it Alice?‟
C: „No, but the first letter is
right. Have another guess.‟
T: „Alex?‟
C: „No, try again‟
T: „Amy‟
C: „Yes, that‟s right‟
Carer: „Can you remember the
name of your granddaughter?‟
Tom: „Erm...I‟m not sure‟
C: „Your granddaughter‟s
name is Amy‟
http://www.wales.nhs.uk/sitesplus/861/home

52. Memory:
External Strategies
• Write it down
• When something is
important to remember,
write it down, and keep it in
a safe place
• Check notes regularly.
• Writing information down
also allows for repeated
exposure to the information
• Hear, Write, Read – 3x
exposure
• Calendar
• PDA or cell phone
• Notebook

53. • DIARIES
• Essential addition to anyone‟s memory whether for
forward planning or for remembering past event –
Powell (1994)
• Page a day diary
• Diaries entries can act as cues or triggers
• Check diary regularly
• Cross out things you have done
• Write in future activities/events
• MEMORY AIDS
• Prompts, Post-it notes, Dry-wipe board, Notice
boards, Calendars, Notebooks, Lists, Signs, Labels,
Timers, Alarms, Watch alarms, Pill boxes, Key
finders, Electronic Organisers etc. etc.

54. Memory:
Internal strategies
• Aim is to take in, retain and access information as
effectively as possible
• Concentrate („getting information in‟)
• „Reflective listening‟; recapping what‟s been said in your own
words–perceived as attentive listening
• Incorporates some repetition and deeper processing
• If you miss what is said, ask again
• Chunking/grouping
• Name/face association
• Verbal/non-verbal „recoding‟
• PQRST technique
• Mnemonics

55. Executive Functioning
• Structure and Routine!
• Do things that require the most initiation in the morning or after a
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rest
• Set a small number of goals for each day
Set up (with assistance) organisational practices
Large family calendar
Online bill payment
Use labels
Schedules
Simplify activities
Prioritise
Checklists

56. Executive Functioning
Meta-cognitive strategies
• To regulate behavior and increase goal-
oriented behavior
• Self-talk
• Tracking behaviors
• Self-monitoring
• Tracking errors and attention lapses
• Goal Management Training

57. Goal Management Training
• Maintaining intentions
in goal-directed
behavior is reliant on
intact executive
functioning
• GMT based on theory
of goal neglect
resulting in
disorganised behavior
following frontal lobe
injury

58. AIM
OPTIONS
PROS
CONS
COMMENCE AND MONITOR

59. Awareness/insight
• Education around deficits can help.
• Strategies that raise awareness of performance - videos,
checklists, or giving feedback.
• Feedback:
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non-critical feedback
should be concrete (plain; able to be seen),
Use specific examples and information
Provide opportunities for feedback from others:
• Family
• Friends
• Peers
• Encourage the person to evaluate their own performance in
different areas, particularly focusing on areas that are causing
difficulty.

60. • Build self-esteem by encouraging the person to try a (non-dangerous)
activity that he/she feels confident doing.
• Give the person visual and verbal reminders or “hints” (e.g., a smile or
the words "good job") to improve confidence in carrying out basic
activities more independently.
• Remember lack of insight is part of the neurological damage and not
just the person being obstinate.
• Be aware, however, that denial can also be a coping mechanism to
conceal the fear that he/she cannot do a particular task. Therefore
they may insist that the activity cannot be done or is “stupid.”

61. Influences on Cognition
• Include:
• Physical difficulties
• Fatigue
• Depression/anxiety
• Engagement
• Pain
• Medications

62. “A new study shows that licking the sweat off a frog can cure
depression. The down side is, the minute you stop licking, the
frog gets depressed again!”
Sonet

63. “Work used to interfere with my laziness but thanks to MS I have
all the time in the world to be lazy!”
Wendy Bessenyei