1

LaMarre

Appendix e-1

A. FTDC Consensus Criteria for behavioral variant Frontotemporal dementia (bvFTD) ***Adapted from Rascovsky et al., 2011 (1)***

  1. Shows progressive deterioration of behavior and/or cognition by observation or history (as provided by knowledgeable informant)
  2. Possible bvFTD: Three of the following behavioral/cognitive symptoms [A-F] must be present:
  1. Early behavioral disinhibition
  2. Socially inappropriate behavior
  3. Loss of manners or decorum
  4. Impulsive, rash or careless actions
  5. Early apathy or inertia
  6. Apathy: loss of interest, drive or motivation
  7. Inertia: decreased initiation of behavior
  8. Early loss of sympathy or empathy
  9. Diminished response to other people’s needs or feelings: positive rating should be based on specific examples that reflect a lack of understanding or indifference to other people’s feelings
  10. Diminished social interest, interrelatedness or personal warmth: general decrease in social engagement
  11. Early perseverative, stereotyped or compulsive/ritualistic behavior
  12. Simple repetitive movements
  13. Complex, compulsive or ritualistic behaviors
  14. Stereotypy of speech
  15. Hyperorality and dietary changes
  16. Altered food preferences
  17. Binge eating, increased consumption of alcohol or cigarettes
  18. Oral exploration or consumption of inedible objects
  19. Neuropsychological profile: executive/generation deficits with relative sparing of memory and visuospatial functions
  20. Deficits in executive tasks
  21. Relative sparing of episodic memory (compared to degree of executive dysfunction)
  22. Relative sparing of visuospatial skills (compared to degree of executive dysfunction)
  1. Probable bvFTD: All criteria must be met
  1. Meets criteria for possible bvFTD
  2. Exhibits significant functional decline
  3. Imaging results consistent with bvFTD
  4. Frontal and/or anterior temporal atrophy on CT or MRI
  5. Frontal hypoperfusion or hypometabolism on SPECT or PET

B. Brief Neurological Exam

1.Spontaneous speech

  1. “Please tell us why you are here today”
  2. “Have you noticed any changes in your memory? language? movement? behavior or mood?

2.Eye movements

  1. Look at camera (about 15 sec.)
  2. Visual pursuit (following stick, vertical and horizontal, left, right)
  3. Saccades (nose to target, vertical and horizontal, right/left)
  1. Motor Exam
  1. Hands in lap
  2. Hold up arms (stop traffic, and elbows up)
  3. Draw a circle, figure 8, brush teeth, saw wood, flip coin (with each hand)
  4. Fine finger movements (rapid/alternating)

4.Gait

  1. Stand up arms folded
  2. Walk at least 10 feet, then turn
  3. Walk on sides of feet
  4. Retropulsion pull test

5.Motor speech exam

  1. Repeat “puh” (x5), “tuh” (x5), “kuh” (x5)
  2. Repeat “puh-tuh-kuh” (x5)
  3. Repeat “house”, “catastrophe”, and “articulatory” one time each
  4. Repeat “articulatory” five times quickly

C. Brief Language Testing

Note: all language tasks were derived from the Boston Diagnostic Aphasia Examination 3rd Edition (2), save single word comprehension and famous faces.

  1. Spontaneous speech: Cookie-theft picture

“I want you to describe everything you see happening in this picture”

  1. Object Naming Task: “What is this?”

BedPyramid

HelicopterFunnel

CamelAsparagus

  1. Irregular Word Reading: “Please read these words out loud for me”

Quick Come

Mist Scout

BouquetSoul

PintSure

Home Mortgage

  1. Syntax comprehension: “I’m going to show you some pictures of people. There will be 4 pictures on each page and I would like you to listen carefully and pick the one that I describe”
  2. The girl kicks the boy who is wearing a hat
  3. The girl is chasing the boy who is wearing boots
  4. The girl hitting the boy is sitting down
  5. The mother is calling her child who has light hair
  6. The woman who is fat is kissing her husband
  7. Repetition of single words, and sentences: “Repeat after me”
  8. Brown
  9. Chair
  10. 1,776
  11. Down to earth
  12. Pry the tin lid off
  13. Methodist Episcopal
  14. The barn swallow captured a plump worm
  15. Single Word Comprehension (16 items; Peabody Picture Vocabulary Test III (3)):

“There are four pictures on this page. I will say a word, and then I want you to point to the picture that best tells the meaning of the word”

  1. Famous faces (4):

“Who is this?”

  1. JFK (politician/president)
  2. Nancy Reagan (first lady)
  3. Cary Grant (actor)
  4. Madeleine Albright (secretary of state)

D. Caregiver Interview Questions

  1. Opening Questions:
  2. When did you first notice a change in your friend/family member?
  3. What was the first thing you noticed?
  4. What other changes have you noticed?
  5. Were these changes gradual or did they come on suddenly?
  6. Memory
  7. Has there been any change in memory? Give me some examples.
  8. Does he/she have trouble recalling recent events?
  9. Recalling events from the distant past?
  10. Does he/she misplace objects?
  11. Forget conversations?
  12. Forget appointments?
  13. Forget to take his/her medication?
  14. Language / Semantics
  15. Has there been a change in language? Give me some examples
  16. Does he/she have trouble speaking/articulating?
  17. Finding words?
  18. Understanding conversations or words?
  19. Reading?
  20. Writing?
  21. Any problems recognizing people?
  22. Any trouble recognizing objects?
  23. Visuospatial
  24. Does he have trouble orienting himself/herself in space?
  25. Does he/she get lost in familiar places?
  26. Executive:
  27. Has he/she become more rigid or inflexible?
  28. Does he/she have trouble planning or organizing activities?
  29. Solving problems?
  30. Any problems with judgment?
  31. Attention / Arousal
  32. Are there any problems with attention or concentration?
  33. Is he/she easily distractible?
  34. During the day, do you notice marked fluctuations in his/her attention or thinking?
  35. Motor:
  36. Have you noticed any changes in movement?
  37. Falls?
  38. Tremor?
  39. Weakness?
  40. Problems with coordination?
  41. Sleep:
  42. Any trouble with sleep?
  43. Does he/she strike out with arms and legs or vocalize while dreaming?
  1. Behavioral Symptoms:
  2. Have you noticed any changes in behavior or mood? Give me some examples.
  3. Psychiatric
  4. Would you say that your friend/family member is depressed?
  5. Anxious?
  6. Are there noticeable fluctuations in mood?
  7. Is he/she irritable or aggressive?
  8. Does he/she hear voices?
  9. Does he/she see things that are not there, such as small people or animals?
  10. Does he/she have visual misperceptions? For example, see shadows or things out of the corner of his/her eye?
  11. Does he/she have beliefs that you know are not true? (for example - insisting that people are stealing things from him/her or are out to harm him)
  12. FTD Specific
  13. Does he/she behave inappropriately in social settings? Give some examples.
  14. Does he/she act impulsively, without thinking?
  15. Does he/she seem passive, less motivated or apathetic?
  16. Does he/she have problems initiating activities or conversations?
  17. Does he/she seem cold or indifferent to other people’s feelings? Is he/she less affectionate or empathic with those around him/her?
  18. Does he/she repeat activities over and over, such as tapping, scratching, picking, opening and closing drawers, etc?
  19. Does he/she engage in activities such as compulsive cleaning, collecting, counting, ordering objects, etc.?
  20. Has there been any change in appetite, weight or eating habits?
  21. Functional:
  22. Is he/she currently working?
  23. Driving?

e-References

e1. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456-2477.

e2. Goodglass H, Kaplan E, Barresi B. Boston Diagnostic Aphasia Examination (3rd Ed.). Philadelphia: Lippincott Williams & Wilkins, 2001.

e3. Dunn LM, Dunn LM. Examiner's Manual for the Peabody Picture Vocabulary Test Third Edition. Circle Pines, Minnesota: American Guidance Service, 1997.

e4. Gorno-Tempini ML, Rankin KP, Woolley JD, Rosen HJ, Phengrasamy L, Miller BL. Cognitive and behavioral profile in a case of right anterior temporal lobe neurodegeneration. Cortex 2004;40:631-644.