PASS & NACC FTD Study

Daisy Sapolsky, MS, CCC-SLPSpeech Language PathologistMGH Frontotemporal Disorders UnitMGH 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 MGH was one of the first five to receive grants

NACC was then established in 1999 by the National Institute on Aging

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 evaluationTo 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 aloneSome 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 0.5 1

normal questionable/very mild impairment mild impairment

ARTICULATION: ability to say sounds and syllables accurately and effortlessly Normal articulation.

Occasional misarticulation and/or effortful or hesitant speech, or dysarthria; difficulty repeating "pa ta ka" and/or pronouncinng multi-syllabic words; 100% intelligible.

Mild and consistent difficulty with articulation; most utterances are intelligible.

FLUENCY: degree to which speech flows easily or is interupted by hesitations, fillers, pauses; reduced fluency is associated with decreased phrase length and words per minute (WPM) Normal flow of speech.

Speech contains occasional blank pauses or use of fillers (umm); reduced WPM and/or phrase length.

Speech is in short phrases, interrupted with pauses or groping for words but there are occasional runs of fluent speech.

SYNTAX AND GRAMMAR: use of word forms (run, ran), functor words (the, an), and word order when forming phrases and sentences in most used modality (speech or writing)

No difficulty in the use of grammar and syntax.

Occasional agrammatism or paragrammatism (i.e., odd sentence structure such as, "I my car drive in your house."); may complain it is effortful to combine words into phrases or sentences

Frequent agrammatism; sentence structures are simple; frequent misuse/ommission of grammatical words or morphology

WORD RETRIEVAL AND EXPRESSION: ability to express the intended word through most used modality (speech or writing)

Difficulty limited to rare word-finding problem or tip-of-the tongue feeling.

Noticeable word-finding pauses during conversation or testing; may substitute a more common word or provide a description of the word (circumlocution); expresses message with most details; may use stereotyped phrases.

Word finding difficulty (pauses or struggling) occurs several times in a 5-minute conversation; difficulty naming common objects; occasional semantic or phonemic paraphasias; expresses overall message with few details.

http://www.ftd-boston.org/

PASS profile: Patient A

0

1

2

3

Artic

Fluency

Synta

x

Word

Ret

rieva

l

Repet

ition

Aud C

omp

Single

Wor

d Com

p

Readi

ng

Writ

ing

Funct

Glob

al

PA

SS

rat

ing

Baseline

Follow up

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

0

1

2

3

Artic

Fluency

Synta

x

Word

Ret

rieva

l

Repet

ition

Aud C

omp

Single

Wor

d Com

p

Readi

ng

Writ

ing

Funct

Glob

al

PA

SS

rat

ing

Baseline

Follow up

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

weaknessesDetermining PPA subtypeMonitoring disease progressionCapturing response to treatment in clinical

trialsspeech-language therapydrug 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

Central Michigan UniversityNorthwestern University

University of California, San Francisco

International interest:Nantes University Hospital, Nantes, FranceWar Memorial Hospital, Sydney, Australia

Questions

Questions?