sapolsky_ftdupdate_mar2011
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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)
NACC overview:
Alzheimer’s Disease Centers (ADCs) were
established in 1984
NACC was then established in 1999 by the
National Institute on Aging
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)
NACC overview:
Participation in NACC involves annual visits
neurological examination and interview
neuropsychological testing
interview with a relative/friend
MGH NACC cohort includes:
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
NACC FTD study:
In pilot phase for the past year (6 centers)
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
data may be helpful in clinical trial design
Clinical Dementia Rating (CDR)
Tools for quantifying severity and progression
of dementia:
Clinical Dementia Rating (CDR)
5-point scale to rate stages of dementia
no cognitive impairment to severe impairment
six domains of functioning are assessed through interview
and patient performance on testing
Memory, Orientation, Judgment and Problem solving,
Community Affairs, Home and Hobbies, Personal Care
two supplemental domains added in 2006:
behavior and language
Progressive Aphasia Severity Scale (PASS)
PASS (2008)
A “big picture” clinical tool to rate symptom
severity in a variety of speech/language
domains in people with progressive aphasia
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)
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
Progressive Aphasia Severity Scale (PASS)
All domains rated from normal to severe impairment,
like the CDR:
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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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
Fu
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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)
Potentially useful for:
Generating a profile of strengths and
weaknesses
Determining PPA subtype
Monitoring disease progression
Capturing response to treatment in clinical
trials
speech-language therapy
drug treatments
PASS Paper
Neurology, 2010
NDM Paper
Neurodegenerative Disease Management, 2011
PASS – Next Steps
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
Training materials in development
Global partners in using PASS
Northwestern University
Central Michigan University
University of
California,
San Francisco
International interest:
Nantes University Hospital, Nantes, France
War Memorial Hospital, Sydney, Australia
Questions
Questions?