Cognitive Dysfunction
Cognitive Dysfunction In MS:
In MS: Addressing
the
Addressing
the Emotional,
Emotional,
Social,Impact
and
Social,
and Vocational
Vocational Impact
Outline
•
•
•
•
•
•
•
•
Historical perspective
Frequency and severity of cognitive changes in MS
Relationship to other disease characteristics
Functions that are affected; functions that are preserved
Impact of cognitive dysfunction
 Emotional
 Social
 Vocational
 Medical
How, when, and why of assessment
Treatment options
Strategies for clinicians
Jean-Martin Charcot
1825-1893
Charcot with Marie Whittman
and Joseph Babinski
André Brouillet - 1887
Jean-Martin Charcot
Second Lecture on Multiple Sclerosis, 1868
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.
Long-Standing Misconceptions
about MS and Cognition
•
•
•
•
•
•
Cognitive impairment (CI) is rare in MS.
CI only occurs in late stage MS or severe MS.
MS is a white-matter disease and does not affect:
1) brain volume, 2) gray matter, 3) the cerebral
cortex.
If an MS patient can pass the mental status exam,
everything is OK.
Memory problems reported by MS patients are
caused by stress, anxiety, and/or depression.
Discussing CI will upset MS patients/families and
ruin the “image” of MS.
Cognition and Other Disease Characteristics
•
•
•
•
•
Cognitive function correlates with number of lesions and
lesion area on MRI, as well as brain atrophy.
Cognitive dysfunction can occur at any time but is more
common later in the disease.
Cognitive dysfunction can occur with any disease
course, but is slightly more likely in progressive MS.
Being in an exacerbation is a risk factor for cognitive
dysfunction.
Depression can worsen cognition, particularly executive
functions (Arnett et al., 1999).
Prevalence of cognitive impairment in MS
Rao et al., 1991
Peyser et al., 1986
43%
54%
Bertrando et al., 1983
55%
Minden et al., 2006
56%
Heaton et al., 1985
56%
Lyon-Caen et al., 1986
60%
Staples & Lincoln, 1979
60%
Rao et al., 1984
64%
Parsons et al., 1957
65%
De Smedt et al., 1984
65%
Cognitive Impairment over a 10-Year Period
Percent Impaired
None
Mild
Moderate
80%
70%
60%
50%
40%
30%
20%
10%
0%
Baseline
Adapted from Amato, MP et al, Archives of
Neurology 2001;58:1602-1606.
4 Years
10 Years
Years of Follow-up
Longitudinal Study of Cognitive
Impairment in MS
•
•
•
Percent Impaired at Baseline: 26%
Percent Impaired after 10 Years: 56%
Predictors of Cognitive Dysfunction after 10 Years:
 Higher EDSS
 Progressive course
 Older age
Amato, MP et al, Archives of Neurology 2001;58:1602-1606.
Cognitive Changes in Multiple Sclerosis
Severity of Cognitive Changes
in Multiple Sclerosis
None
50%
Mild
40%
Moderate to
severe
10%
The Impact of Cognitive Dysfunction
In Daily Functioning
Work status
P<0.01
Social activity
P<0.05
Personal assistance
P<0.01
Community services
Financial status
Cognitively intact (n=52)
Transportation
Cognitively impaired (n=48)
Personal residence
0
1
2
Mean scale score
Rao et al. Neurology. 1991;41:692.
Worsening
3
Impact of Cognitive Impairment on
Employment
45
40
% Employed
35
30
25
20
15
10
5
0
Rao et al. Neurology. 1991;41:692.
Impaired
Not Impaired
Cognitive Functions Affected in MS
•
•
•
•
•
•
Memory - acquisition and retrieval
Attention & concentration - working memory
Speed of information processing
Executive Functioning
Visual/spatial organization
Verbal fluency - word finding
DeLuca, J. What we know about cognitive changes in multiple sclerosis. In LaRocca, N & Kalb, R (eds.)
Multiple sclerosis: understanding the cognitive challenges. New York: Demos Medical Publishing, 2006.
Cognitive Functions Unaffected in MS
•
•
•
•
•
General intellect
Long-term (remote) memory
Recognition memory
Conversational skill
Reading comprehension
DeLuca, J. What we know about cognitive changes in multiple sclerosis. In LaRocca, N & Kalb, R
(eds.) Multiple sclerosis: understanding the cognitive challenges. New York: Demos Medical
Publishing, 2006.
Recognizing Memory Problems
•
•
•
•
•
•
•
Difficulty learning new material or needing to spend
longer to make it stick
Forgetting recent conversations, TV shows, movies
Forgetting appointments
Losing track of medication schedules
Neglecting to do planned tasks
Losing or misplacing things
Forgetting names, phone numbers, etc.
Recognizing Impaired
Attention and Concentration
•
•
•
•
•
•
Difficulty with focus
Cannot stick to one task without getting distracted
Problems screening out distractions
Difficulty with divided attention tasks, e.g., listening to a
family member talk while cooking
Running out of steam when trying to concentrate on
reading material or other intellectual tasks
Poor recall due to lack of attention when information is
being learned
Recognizing Slowed
Information Processing
•
•
•
•
Quality of work is the same but output is much less
Cannot respond quickly when a lot of information is being
presented
Trouble dealing with tasks having a time element, e.g.,
card games, word games, deadlines
Difficulty processing information coming from several
different sources simultaneously
Recognizing Problems in
Executive Functioning
•
•
•
•
•
•
Inability to perform jobs requiring analytic skills
Difficulty following complex arguments or explanations;
missing the point in conversations
Trouble following through with complicated tasks
Being too literal or concrete
Need for increased direction on the job because of
difficulty in setting priorities, organizing time, and
meeting deadlines
Trouble with “multi-tasking”
Recognizing Impaired Visual/Spatial
Organization
•
•
•
•
•
Gets lost when driving; confused about right/left,
north/south
Can’t do puzzles or assemble “some assembly
required” items
Trouble operating machines
Difficulty understanding diagrams
Problems visualizing objects without a picture—
e.g., from a description, incomplete picture, or
disassembled picture
A Word about Cognition and Fatigue
•
•
•
Physical fatigue has less impact on cognitive
performance than people think.
Cognitive fatigue refers to a decline in cognitive
performance following cognitively challenging tasks.
Cognitive fatigue can occur even in the absence of
physical fatigue.
DeLuca, J. What we know about cognitive changes in multiple sclerosis. In LaRocca, N & Kalb, R
(eds.) Multiple sclerosis: understanding the cognitive challenges. New York: Demos Medical
Publishing, 2006.
The Psychosocial Impact of
Cognitive Changes (LaRocca & Kalb, 2006)
•
•
•
The ability to think, remember, and reason is central to a
person’s identity. Changes in cognitive abilities:
 Threaten the sense of self
 Damage self-esteem and self-confidence
Cognitive abilities form the basis of our interactions with
others.
Cognitive impairments:
 Alter communication patterns
 Impact other people’s perceptions
 Interfere with role performance
 Affect the balance in a partnership
I TOLD YOU THAT THIS MORNING!?! I DON’T THINK
YOU’RE PAYING ATTENTION—OR MAYBE YOU JUST
DON’T CARE ABOUT WHAT I HAVE TO SAY
ANYMORE….
I TOLD YOU THAT THIS MORNING…
•
•
•
Possible cognitive deficit(s)?
Possible feelings?
 Wife with MS
 Husband
Recommended strategies?
I TOLD YOU THAT THIS MORNING…
•
•
•
Possible cognitive deficit(s): memory, attention,
info processing
Possible feelings:
Wife with MS: denial, anger, anxiety, guilt,
inadequacy
Husband: frustration, anxiety, abandonment
Recommended strategies: written note, family
calendar, non-distracting environment for
conversations
HOW COULD YOU GET LOST?!?
YOU’VE DRIVEN THAT ROUTE
100 TIMES!!
HOW COULD YOU GET LOST…
• Possible cognitive deficit(s)?
• Possible feelings?
 Wife
 Husband with MS
• Recommended strategies?
HOW COULD YOU GET LOST…
•
•
•
Possible cognitive deficit(s): memory, attention,
visual-spatial, sequencing
Possible feelings:
 Wife: anxiety, loss of respect/confidence, anger
 Husband with MS: anxiety, anger, embarrassment,
loss of confidence
Recommended strategies: pre-planning of route:
maps in memory book; minimal distractions in car
YOU REALLY MESSED UP THE
CHECKBOOK THIS TIME!!
YOU REALLY MESSED UP THE
CHECKBOOK…
•
•
•
Possible cognitive deficit(s): attention/concentration,
organization, planning/sequencing, problem-solving
Possible feelings:
• Wife with MS: embarrassment, guilt, anxiety
• Husband: anger, anxiety, loss of partnership
Recommended strategies: template, distraction-free
environment
“WHERE ARE YOU?!?
OUR MEETING WITH THE CLIENT
STARTED AN HOUR AGO?”
WHERE ARE YOU?!?....
•
•
•
Possible cognitive deficit(s): memory, planning
organization, attention, visual/spatial skills
Possible feelings:
 Boss: anger, frustration, confusion, anxiety
 Employee with MS: embarrassment, frustration,
fear
Recommended strategies: calendar, tickler system,
pre-route planning
MOM—I TOLD YOU YESTERDAY THAT I WAS
GOING TO SARA’S HOUSE AFTER SCHOOL!
MOM—I TOLD YOU YESTERDAY…
•
Possible cognitive deficit(s):
attention/concentration, organization
• Possible feelings:
 Mom with MS: embarrassment, guilt, anxiety
anger, suspiciousness (if not true)
 Child: anxiety, loss of confidence, guilt (if not
being truthful)
• Recommended strategies: family calendar,
distraction-free environment
When Cognitive Evaluation is Appropriate
•
•
•
•
•
•
To establish a baseline
There are reported changes in ability
There is a potentially treatable condition
Person is being started on a new treatment
When considering an application for SSDI or vocational
rehabilitation
When there is a need to know
Note: The standard mental status examination will miss
50% of cognitively impaired patients (Peyser, 1980)
Cognitive Evaluation
•
•
•
•
•
Battery of tests designed to assess areas of reported
difficulties, as well as pre-existing and current strengths
Clinical neuropsychologist, occupational therapist,
speech-language pathologist
Full test battery = 6-8 hours over two days
Expensive/often without insurance coverage
Various screening batteries available, including a 5minute self-report instrument (MS Neuropsychological
Screening Questionnaire [Benedict et al., 2003; 2004])
Treatment of Cognitive Dysfunction
•
•
•
Symptomatic treatments
Disease modifying agents
Cognitive rehabilitation
Symptomatic Treatments – as of 2012,
none shown to be effective in
controlled clinical trials
•Acetylcholinesterase inhibitors
•Anti-fatigue agents
•Stimulants
•Potassium channel
blockers
donepezil
amantadine
modafinil
methylphenidate [attention]
4-aminopyridine
3,4-diaminopyridine
Disease Modifying Agents
•
•
•
•
•
•
•
fingolimod
interferon beta 1a (Cohen et al., 2002)
interferon beta 1b (Flechter et al., 2007)
glatiramer acetate (Schwid et al., 2007)
natalizumab (Iaffaldano et al., 2012; Portaccio et al., 2012;
Mattioli et al., 2011)
mitoxantrone (Zéphir et al., 2008)
teriflunomide
Cognitive Rehabilitation
•
•
Direct retraining of impaired functions
 Memory exercises
 Attention training
Compensatory strategies
 Notebooks, lists, organizers
 Time and energy management
 Substitution strategies
Guidelines for Treatment (for now)
•
•
Symptomatic Treatments – slow progress
 Not much of real value has emerged; all clinical trials have had
negative results
 Disease Modifying Agents – may be most important
 Modest results so far, but if they can slow or halt
accumulation of cerebral lesions . . .
Cognitive Rehabilitation – common-sense help
 Disappointing thus far but common-sense points to
compensatory measures as best strategy
 Address affective and social issues related to MS
Implications for patient care
Even mild cognitive dysfunction can impact treatment:
•
•
Your patients may not:
 Show up on time for appointments
 Follow complex explanations
 Remember what they’ve been told
 Follow through on treatment plans
You may want to:
 Provide informational brochures
 Provide appointment reminders
 Write down specific instructions
 Remind patients to write down their questions
 Invite patients to bring a family member or
friend to appointments
Recommended Strategies for Your Patients
•
•
•
•
•
•
Get someone to work with you.
Make up your mind that it’s OK to do things a little
differently than in the past.
Although abilities may not improve, function can be
enhanced.
Compensation is key—e.g., many memory problems can
be solved with better organization.
Consistency is essential. Stick with your program and
follow through with your new strategies.
Keep the mind active and stimulated.
Summary
•
•
•
•
•
•
•
More that 60% of people with MS experience cognitive
changes.
Cognitive dysfunction is more related to MRI changes
than to other disease characteristics.
While many functions can be affected, some are more
likely to be affected than others.
The impact on individuals and families is significant.
Disease-modifying therapies are the best protection;
symptomatic medications have been shown to be of no
benefit in large-scale clinical trials.
Compensatory strategies are essential.
Adequately treating depression may improve cognitive
functioning.
National MS Society Resources for Your Patients
•
•
•
Booklets
Available from the National Multiple Sclerosis Society (by calling
1-800-344-4867; or online at
www.nationalmssociety.org/Brochures)
 MS and the Mind
 Solving Cognitive Problems
 Fatigue: What You Should Know
 Hold that Thought
Webcast: Hold that Thought: Cognition and MS
(http://www.nationalmssociety.org/multimedia-library/webcasts-podcasts/ms-hold-that-thought/index.aspx)
Website (http://www.nationalmssociety.org/about-multiplesclerosis/symptoms/cognitive-function/index.aspx)
National MS Society Resources for Clinicians
•
MS Clinical Care Network






Website: www.nationalMSsociety.org/MSClinicalCare;
E-mail: healthprof_info@nmss.org
Clinical consultations with MS specialists
Literature search services
Professional publications (Clinical Bulletins; Expert Opinion
Papers; Talking with Your MS Patients about Difficult Topics;
Pamela Cavallo Education Series for nurses, rehab
professionals, mental health professionals, and pharmacists
Professional Education Programs (Nursing, Rehab,
Mental Health)
Consultation on insurance and long-term care issues
Quarterly professional e-newsletter
Recommended Readings
Books
•
•
•
•
Feinstein A. The Clinical Neuropsychiatry of Multiple
Sclerosis (2nd ed.). Cambridge: Cambridge University
Press, 2007.
Gingold J. Facing the Cognitive Challenges of Multiple
Sclerosis. New York: Demos Medical Publishing, 2006.
Kalb R, Holland N, Giesser B. Multiple Sclerosis for
Dummies. Hoboken NJ: Wiley Publishing, 2007.
LaRocca N. Cognitive Challenges: Assessment and
Management. In R. Kalb (ed.) Multiple Sclerosis: The
Questions You Have; The Answers You Need (4th ed.)
New York: Demos Medical Publishing, 2007.
Recommended Readings, cont’d
•
LaRocca N & Kalb R. Multiple Sclerosis:
Understanding the Cognitive Challenges. New York:
Demos Medical Publishing, 2006.
Society Publications
•
•
Expert Opinion Paper: Assessment and Management
of Cognitive Impairment in Multiple Sclerosis, 2008
(www.nationalMSsociety.org/ExpertOpinionPapers).
LaRocca N. Talking with Your MS Patient about
Cognitive Dysfunction, 2009
(www.nationalMSsociety.org/PRCPublications).