The ALS Association 2014 Clinical Conference Phoenix, AZ

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Withdrawn due to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)

16. Concentration
Scoring (Discontinue after failure on 2 consecutive trials.)
Score for the maximum span correct (2 – 5)
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) Score 4
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) Score 4

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.