sapolsky_ftdupdate_mar2011

Report
PASS & NACC FTD Study
Daisy Sapolsky, MS, CCC-SLP
Speech Language Pathologist
MGH Frontotemporal Disorders Unit
MGH Department of Speech, Language and Swallowing Disorders & Reading Disabilities
National Alzheimer’s Coordinating Center (NACC)
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NACC overview:
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Alzheimer’s Disease Centers (ADCs) were
established in 1984
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NACC was then established in 1999 by the
National Institute on Aging
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MGH was one of the first five to receive grants
To facilitate collaborative research among the 29 ADCs
nationwide
NACC maintains a large database of the data
collected at these visits, which is a valuable
resource for ongoing research
http://www.alz.washington.edu/
National Alzheimer’s Coordinating Center (NACC)
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NACC overview:
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Participation in NACC involves annual visits
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neurological examination and interview
neuropsychological testing
interview with a relative/friend
MGH NACC cohort includes:
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709 people being actively followed
320 people with normal cognition
330 people with some form of MCI
266 people with some form of dementia
NACC FTD Study
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NACC FTD study:
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In pilot phase for the past year (6 centers)
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MGH ready to begin administration of the NACC
FTD module
Goals:
To capture the cognitive changes of FTD through
use of a tailored evaluation
 To collect uniform data on patients with FTD
from centers across the country
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data may be helpful in clinical trial design
Clinical Dementia Rating (CDR)
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Tools for quantifying severity and progression
of dementia:
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Clinical Dementia Rating (CDR)
5-point scale to rate stages of dementia
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no cognitive impairment to severe impairment
six domains of functioning are assessed through interview
and patient performance on testing
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Memory, Orientation, Judgment and Problem solving,
Community Affairs, Home and Hobbies, Personal Care
two supplemental domains added in 2006:
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behavior and language
Progressive Aphasia Severity Scale (PASS)
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PASS (2008)
 A “big picture” clinical tool to rate symptom
severity in a variety of speech/language
domains in people with progressive aphasia
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Modeled after the CDR supplemental language
box, a rating of overall language impairment
Clinicians use their judgment to rate presence and
severity of impairment in each domain, capturing
change from the patient’s baseline
Progressive Aphasia Severity Scale (PASS)
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Captures information about symptoms,
whereas performance-based testing captures
signs of impairment;
 Presumably the two together will provide
more information than either alone
Some patients perform surprisingly better on
testing than in daily life;
 Others are much more capable of
communicating in conversation than they are of
performing on tests
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Progressive Aphasia Severity Scale (PASS)
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All domains rated from normal to severe impairment,
like the CDR:
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0 (normal), 0.5 (questionable), 1 (mild), 2 (moderate), 3 (severe)
 Articulation
 Syntax/grammar
 Fluency
 Word retrieval and expression
 Repetition
 Auditory comprehension
 Single word comprehension
 Reading
 Writing
 Functional communication
Progressive Aphasia Severity Scale (PASS)
Progressive Aphasia Severity Scale (PASS) 5.1 (Sept 16, 2009)
Patient Name:
Primary mode of expression (speech, writing, gesture, etc.):
Visit Date and Type:
Rater name:
0
normal
0.5
1
questionable/very mild impairment
mild impairment
Occasional misarticulation and/or
effortful or hesitant speech, or
dysarthria; difficulty repeating "pa ta Mild and consistent difficulty with
ka" and/or pronouncinng multi-syllabic articulation; most utterances are
words; 100% intelligible.
intelligible.
ARTICULATION: ability to say sounds and
syllables accurately and effortlessly
Normal articulation.
FLUENCY: degree to which speech flows
easily or is interupted by hesitations, fillers,
Speech is in short phrases, interrupted
pauses; reduced fluency is associated with
Speech contains occasional blank
with pauses or groping for words but
decreased phrase length and words per minute
pauses or use of fillers (umm); reduced there are occasional runs of fluent
(WPM)
Normal flow of speech. WPM and/or phrase length.
speech.
Occasional agrammatism or
SYNTAX AND GRAMMAR: use of word
paragrammatism (i.e., odd sentence
forms (run, ran), functor words (the, an), and
structure such as, "I my car drive in
Frequent agrammatism; sentence
word order when forming phrases and
your house."); may complain it is
structures are simple; frequent
sentences in most used modality (speech or
No difficulty in the use effortful to combine words into phrases misuse/ommission of grammatical
writing)
of grammar and syntax. or sentences
words or morphology
Noticeable word-finding pauses during
conversation or testing; may substitute Word finding difficulty (pauses or
a more common word or provide a
struggling) occurs several times in a 5description of the word
minute conversation; difficulty naming
WORD RETRIEVAL AND EXPRESSION: Difficulty limited to rare (circumlocution); expresses message common objects; occasional semantic
ability to express the intended word through word-finding problem or with most details; may use stereotyped or phonemic paraphasias; expresses
most used modality (speech or writing)
tip-of-the tongue feeling. phrases.
overall message with few details.
http://www.ftd-boston.org/
PASS profile: Patient A
PASS rating
3
2
Baseline
Follow up
1
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0
Decline was most significant in the areas that were initially
affected while preserved domains remained areas of
strength.
Relatively fast progression of symptoms.
PASS profile: Patient B
PASS rating
3
2
Baseline
Follow up
1
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Fu
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0
Ratings were stable or changed only slightly (0.5) over 2
years, indicating a relatively slow rate of progression with
many areas of relative strength.
Relatively slow progression of symptoms.
Progressive Aphasia Severity Scale (PASS)
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Potentially useful for:
Generating a profile of strengths and
weaknesses
 Determining PPA subtype
 Monitoring disease progression
 Capturing response to treatment in clinical
trials
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speech-language therapy
 drug treatments
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PASS Paper
Neurology, 2010
NDM Paper
Neurodegenerative Disease Management, 2011
PASS – Next Steps
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Next steps:
Continue longitudinal analysis on the
performance of the PASS and imaging
methods as clinical and imaging markers for
diagnosis and monitoring
 Partner with other centers in the U.S. and
worldwide to use the PASS
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Training materials in development
Global partners in using PASS
Northwestern University
Central Michigan University
University of
California,
San Francisco
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International interest:
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Nantes University Hospital, Nantes, France
War Memorial Hospital, Sydney, Australia
Questions
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Questions?

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