PASS & NACC FTD Study

1. 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

2. 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/

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. Progressive Aphasia Severity Scale (PASS) http://www.ftd-boston.org/

10. PASS profile: Patient A Decline was most significant in the areas that were initially affected while preserved domains remained areas of strength. Relatively fast progression of symptoms.

11. PASS profile: Patient B 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.

12. 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

13. PASS Paper Neurology, 2010

14. NDM Paper Neurodegenerative Disease Management, 2011

15. 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

16. 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

17. Questions • Questions?