Framing an Appropriate Research Question 6b9b26d93da94caf993c038d9efcdedb.pdf
Presentation 229 a dallas forshew_ the als cognitive behavorial screen_als cbs_fast tool to use in the clinc.2014
1. Dallas A Forshew, RN, BSN
Manager, Clinical Research
Forbes Norris ALS Research Center
California Pacific Medical Center, San Francisco
2. Outline
Statement of the problem
Definition of FTD and variants
Overview of ALS CBS™
How to administer ALS CBS™
How to score ALS CBS™
Resources
3. What is Frontotemporal Dementia?
FTD: severe form of cognitive / behavioral impairment
Frontal and/or temporal areas of brain are damaged and start to
slowly atrophy
May start with
Poor decision making (executive dysfunction)
Personality changes or behavior problems (behavior variant) or
Language difficulty (trouble finding words)
Gets worse as time passes. Does not get better.
Patients with easy crying or laughing (pseudobulbar) do not have
this problem more than other ALS patients
Clear criteria for uncovering FTD (Neary, 1998)
Not at all like Alzheimer’s disease (memory mostly stays normal)
4. FTD is not new in ALS
Old descriptions
Withdrawn due to
depression
Stubborn
Seeking control in some
area of life
Anger outbursts due to
frustration of ALS
Denial
Language problems due
to dysarthria
FTD behaviors
Apathetic
Dis-inhibited
Poor judgement
Easily frustrated
Quick to anger
Lack of insight
Language difficulty
Word finding
Spelling
Aphasia
5. Incidence of FTD in ALS
FTD 22%
AD 4%
Normal
48%
Not normal,
Not FTD 26%
Normal 48%
FTD 22%
AD 4%
Behavior
Variant 17%
Executive
Dysfunction
9%
The 26% that is not normal but also not ALS/FTD is redefined as
ALSci (Executive Dysfunction 9%), ALSbv (Behavior Variant 17%)
Lomen-Hoerth C, Murphy J
6. ALS in FTD
36 patients with sporadic FTD and no known
motor neuron disease
14% definite ALS
36% possible ALS
14% fasciculations (1 pt = definite ALS after 1 yr)
17% swallowing trouble
5% other abnormalities
C. Lomen-Hoerth, T. Anderson, B. Miller, Neurology, 2002
7. Genetic Overlap of ALS and FTD
Familial: 10% of ALS, 40% of FTD
Affected family members may have only ALS,
only FTD, or both in familial cases
TDP-43 was discovered to be the major disease
protein in both ALS and the most common form
of FTD. Mutations in TDP-43 cause ALS and FTD.
C9ORF72 is responsible for 20-40% of all familial
ALS cases and 12% of FTD cases (2011)
4-8% of sporadic ALS cases and 3% of sporadic
FTD cases have mutations in C90RF72
8. Why is this Important?
ALS patients with cognitive problems, behavioral
problems, or FTD
• Are half as likely to accept interventions such as NIV
or PEG
• They die, on average, a year earlier
Portet et al. ALS and Other MND 2001;2:23-29
Hodges et al. Neurology 2001;61:349-354
Compliance NIV PEG
ALS-FTD 5 of 18 (28%) 4 of 16 (25%)
ALS only 14 of 23 (61%) 8 of 12 (67%)
Olney RK, Lomen-Hoerth C, Forshew DA, et al. ALS/MND 11/2003
9. Why Screen? Prognosis
Executive dysfunction is a negative prognostic
indicator in patients without dementia
Elanin, et al, Neurology. 2001, 76:1269
Decreased survival of ALS patients with either
ALS-FTD or cognitive impairment (executive
dysfunction)(ALSci)
ALS-FTD: 23 vs. 34 months (p=0.026)
ALSci: 24 vs. 38 months (p<0.0001)
10. ALS CBS™
ALS Cognitive Behavioral Screen
Validated for sensitivity, accuracy, specificity
Fast 5 minute SCREEN
Cognitive component
Performed by MD, RN, SLP, research coordinator
4 sections each worth 5 points
Attention, concentration, tracking/monitoring, initiation and
retrieval
Behavioral component
Self-administered by family member
Compares current behavior with past – changes
18 questions
11. Attention: Commands
I am going to say some commands. Please listen
carefully and do what I say.
If patient is unable to indicate with finger, movement can be
substituted with eyes, arm or other means.
Point/indicate (with your finger) to the ceiling and then
to your left.
Touch your shoulder, point to the floor, then make a
fist.
Scoring
1 point for getting both correct.
12. Attention: Mental Addition/Language
I am going to say some phrases. I want you to tell
me the number of syllables in each.
For example ‘the table’ has 3 syllables.
The weather is nice. (correct response: 5)
Tomorrow will be sunny. (correct response: 7)
Scoring
1 point for getting both correct.
Score 0 if > 20 seconds on either
13. Attention:
Eye Movements - Saccades
Hold up two fingers in front of patient, about 36 inches
apart, at patient’s eye level.
Wiggle one finger. Ask the patient to look at the finger that moves
without moving their head, and then look back at you. Randomly
move left and right finger, pausing 2 seconds between each trial,
for a total of 8 trails.
Patient Instructions: I am going to hold my fingers up. I want you
to keep your head straight and look at me. When I wiggle a finger, I
want you to look at that finger and then look back at me. Try not to
move your head, only your eyes. Each time I wiggle a finger, look at
it and then back to me. (Do 1-2 trials with the pt as an example) We
will do that a few times. Ready? (Do 8 random trials).
14. Attention:
Eye Movements – Anti-saccades
Patient Instructions: Good, next I am going to wiggle a finger
again, but this time, I want you to look AWAY from the finger that
moves. For example, if I move this finger (wiggle one) then I want you
to look at the other finger, not the one that moves, ok? Let’s try it (do 1-
3 trials). Just like before, try to keep you head still and just move your
eyes. After each one, look back at me. Ready? (Do 8 random trials).
Scoring
Saccades: 1 pt for 8 correct; Anti-saccades 2 pts for 8 correct, 1 for 7
Scoring errors: This includes any incorrect gaze, even if the patient
immediately self-corrects. An error is also counted if a patient
attempts to anticipate your move and looks before you wiggle a
finger.
15. Concentration
I am going to say some numbers. After I say them, I want
you to say them to me backwards or in reverse order.
For example, if I say 3-6, you would say 6-3.
If written, do not allow allow pt to write forward span.
Discontinue after failure on 2 consecutive trials.
2-9 (9-2) 7-8-6-4 (4-6-8-7)
9-4 (4-9) 8-4-1-7 (7-1-8-4)
8-7-2 (2-7-8) 8-2-5-9-4 (4-9-5-8-2)
5-8-1 (1-8-5) 5-8-6-3-9 (9-3-6-8-5)
17. Tracking / Monitoring: Months
Please say the months of the year backward,
starting with December
Scoring
Errors include omissions, repetitions, and intrusions
Score 2 if no errors, 1 if 1 error, 0 if 2 or more errors
Score 0 if patient took > 60 seconds
18. Tracking / Monitoring: Alphabet
Please say (or write) the alphabet for me.
Scoring
Mark uncorrected errors, omissions, repetitions, or
intrusions
Score 1 for no errors, 0 if any errors
19. Tracking / Monitoring:
Alternation Task
I want you to alternate between numbers and letters
starting with 1-A, and then 2-B, 3-C, and so on. Please
continue from there, alternating between number-letter,
number-letter, in order, without skipping any until I tell
you to stop.
4-D 5-E 6-F 7-G 8-H 9-I 10-J 11-K 12-L 13-M
Scoring
Errors: Any mistake in sequencing, i.e. 7-H or 8-9
A consistent error counts as only 1 error i.e. 7-H, 8-I, 9-K
Score 2 for all correct, 1 for only 1 error, 0 for > 1 error
20. Initiation and Retrieval
Say (write) as many words as you can starting with the
letter F, as quickly as you can, in 1 minute. (Show patient
Fluency Rules) You cannot say or write the names of
people, places, or numbers. Please do not say or write the
same word with just a different ending like truck, trucks.
(S words can be substituted for F words.)
Scoring
Score 3 for > 12 words, 2 for 12-8 words, 1 for 7-5 words, 0 for < 4)
Errors include repeats, people, places, or numbers
0 errors score 2 more, 1 error gets 1 more point, 2 or more errors-0
If 4 or less words, the total score is o, no matter how few errors
21. Guidelines for Total Score
This is a SCREENING tool
Diagnosis of FTD requires extensive testing with a
qualified neuropsychologist
Manual and Instructions:
Susan Woolley, PhD WoolleS@sutterhealth.org
Score interpretation
17-20: Do not support presence of clear cog impairment
< 16: Suspicion of cognitive impairment
< 10: Considerable suspicion, further testing needed
22. Resources
Family Caregiver Alliance
800-445-8106 www.caregiver.org
ALS Association
800-782-4747 www.alsa.org
FYI informational fact Sheets
ALS and Cognitive Changes: A Guide for Patients and
Families
ALS, Cognitive Impairment & Frontotemporal Lobar
Dementia (for professionals)
23. Many Thanks
Forbes Norris ALS Research Center
Susan Woolley, PhD
WooleS@sutterhealth.org
ALS Center at UCSF
Catherine Lomen-Hoerth, MD, PhD
Jennifer Murphy, PhD
ForsheD@cpmcri.org
24. References
Woolley SC, York MK, Moore DH et al. Detecting frontotemporal
dysfunction in ALS: Utility of the ALS Cognitive Behavioral Screen
(ALS-CBS™). Amyotroph Lateral Scler 2010; 11(3): 303-311.
Lomen-Hoerth C, Murphy J, Langmore S, Kramer JH, Olney RK, Miller
B. Are amyotrophic lateral sclerosis patients cognitively normal?
Neurology 2003; 60(7):1094-1097.
Olney R, Murphy J, Forshew D, Garwood E, Miller B, Langmore S,
Kohn M, Lomen-Hoerth C. The effects of executive and behavioral
dysfunction on the course of ALS. Neurology 2005; 65: 1774-1777.