- Email: [email protected]
DISEASE STAGING IN FRONTOTEMPORAL DEMENTIA AND ALZHEIMER'S DISEASE: THE CONTRIBUTION OF THE FRONTOTEMPORAL DEMENTIA RATING SCALE (FTD-FRS) IN A 12-MONTH FOLLOW-UP STUDY
P732
Poster Presentations: Monday, July 17, 2017
81¼9.407, p¼0.0002, R2¼.188, b¼7.897) but not the lPRC, ERC, or PHC (all p-values <.05). Conclusions: Verbal semantic memory tests are commonly used tools in AD diagnosis. We found that the fluency performance for living, but not nonliving, things differentiated NCs from very early AD patients. Further, we showed that only mean cortical thickness of the mPRC predicted living thing fluency performance. These results are in line with the findings of Kivisaari et al. (2012) showing that only mPRC thickness significantly predicted picture naming performance for living relative to nonliving things. Fluency tasks with living thing categories may provide an early marker of incipient AD.
P2-301
Table 2 Percentage of patients in each severeity level generated by the FTD-FRS and the CDR-FTD at baseline and 12-month follow up assessment. Severity level
bvFTD % AD % Assessment (n¼31) (n¼28)
FTD-FRS Very Mild
Baseline Follow up Mild Baseline Follow up Moderate Baseline Follow up Severe Baseline Follow up Very Severe Baseline Follow up Profound Baseline Follow up p value CDR-FTD 0,0 Mild Baseline Follow up 0,5 Moderate Baseline Follow up 1,0 Severe Baseline Follow up 2,0 Very Severe Baseline Follow up p value
DISEASE STAGING IN FRONTOTEMPORAL DEMENTIA AND ALZHEIMER’S DISEASE: THE CONTRIBUTION OF THE FRONTOTEMPORAL DEMENTIA RATING SCALE (FTD-FRS) IN A 12-MONTH FOLLOW-UP STUDY
Thais Bento Lima-Silva1, Valeria S. Bahia1, Mario Amore Cecchini, Sr,1, Luciana Cassimiro1, Henrique Cerqueira Guimaraes2, Leandro Boson Gambogi3, Paulo Caramelli2, Marcio Luiz Figueredo Balthazar4, Benito Pereira Damasceno5, Sonia Maria Dozzi Brucki6, Leonardo Cruz de Souza3,7, Eneida Mioshi8, Ricardo Nitrini6, Monica Sanches Yassuda6,9, 1University of S~ao Paulo, S~ao Paulo, Brazil; 2 Universidade Federal de Minas Gerais, Belo Horizonte, Brazil; 3Federal University of Minas Gerais, Belo Horizonte, Brazil; 4Unicamp, Campinas, Brazil; 5University of Campinas, Campinas, Brazil; 6Medical School of University of S~ ao Paulo, S~ ao Paulo, Brazil; 7Neurology Department, Belo 8 Horizonte, Brazil; University of East Anglia, Norwich, United Kingdom; 9 State University of Campinas UNICAMP, Campinas, Brazil. Contact e-mail: [email protected]
3,23 0,00 3,23 0,00 16,13 19,35 48,39 45,16 16,13 12,90 12,90 22,58 p¼0,018 0,00 0,00 29,03 6,45 51,61 70,97 19,35 22,58 p¼0,012
PPA% (n¼12)
3,57 8,33 3,57 0,00 14,29 0,00 3,57 0,00 32,14 41,67 38,29 41,67 46,43 33,33 50,00 33,33 3,57 8,33 3,57 16,67 0,00 8,33 0,00 8,33 p¼0,067 p¼0,201 0,00 0,00 0,00 0,00 28,57 8,33 14,29 8,33 57,14 66,67 71,43 66,67 14,29 25,00 14,29 25,00 p¼0,068 p¼1,000
Note. p value refers to Wilcoxon matched pairs test. FTD-FRS¼ Frontotemporal Dementia Rating Scale; CDR- FTD¼ Clinical Dementia Rating scale including Language and Behavior domains.
gressive Aphasia (PPA) (8 semantic variant and 4 non-fluent variant) and 28 of AD. Family members or caregivers who had frequent contact with the patients completed the FTD-FRS. The CDR-FTD was completed by physicians. Patients completed the Addenbrooke’s Cognitive Examination-Revised. To determine annual progression, the 12-month follow up assessments were used. Results: There were significant changes in FTD-FRS and CDR-FTD scores from baseline to follow-up for all groups (Table 1). Analyses based on disease severity levels revealed that the FTDFRS and the CDR-FTD scores captured transitions to more severe
Background: Instruments to stage dementia progression are usually based on Alzheimer’s disease (AD) and do not include the specific symptoms of frontotemporal dementia (FTD). The FTD-FRS was elaborated to stage FTD and recently an extended version of the Clinical Dementia Rating scale included Language and Behavior domains (CDR-FTD). Methods: Participants were 71 individuals, aged 40+ years with at least two years of schooling: 31 received the diagnosis of behavioral variant (bv) FTD, 12 of Primary Pro-
Table 1 Means and standard deviations for the FTD-FRS and CDR-FTD sum of boxes scores at baseline and 12-month follow up assessment bvFTD (n¼31)
Age (50-87 anos) Schooling (0-21 anos) ACE-R Total MMSE FTD-FRS Baseline Follow up P* value CDR-FTD Baseline Follow up P* value
AD (n¼28)
PPA (n¼12)
Media
DP
Media
DP
Media
DP
P**
66,94 11,74 71,71 23,61 27,53 21,83 0,002 8,06 9,00 <0,001
9,26 4,57 16,36 4,96 (26,25) (19,26)
74,15 9,43 67,63 23,25 48,45 45,36 0,018 7,07 7,84 0,001
9,22 4,49 11,51 3,49 (25,62) (22,56)
61,42 14,33 65,75 23,75 47,78 32,22 0,069 9,96 10,38 0,043
5,87 4,40 12,80 3,81 (32,79) (24,63)
<0,001 a,b <0,001 b 0,271 0,696 0,004 a <0,001 a
(3,59) (3,31)
(3,16) (2,88)
(4,22) (4,02)
0,105 0,082
Note: *p value refers to Wilcoxon matched pairs test. FTD-FRS¼ Frontotemporal Dementia Rating Scale; CDR-FTD¼ Clinical Dementia Rating scale including Language and Behavior domains; MMSE+ Mini Mental State Examination; ACE-R¼ Addenbrooke’s Cognitive Examination-Revised. **p-valor Kruskal-Wallis test, followed by the multiple comparison test: a.bvFTDsAD, b.ADsPPA; c.bvFTDsPPA. Multiple comparisons test without statistical significance.
Poster Presentations: Monday, July 17, 2017
stages from baseline to 12-month follow-up in the bvFTD group only, however, results for the AD group approached statistical significance (Table 2). Conclusions: FTD-FRS and the CDR-FTD can aid in staging and determining progression in FTD.
P2-302
ONE-YEAR INTERVAL MONTREAL COGNITIVE ASSESSMENT AND RISK OF PERITONITIS IN SELF-CARE PERITONEAL DIALYSIS PATIENTS
Yat Fung Shea1, Mi Suen Connie Lee1, Ming Yee Maggie Mok1, Man Fai Lam1, Leung Wing Chu1,2, Hon Wai Felix Chan1, Tak-Mao Chan1, 1 Queen Mary Hospital, Hong Kong, Hong Kong; 2The University of Hong Kong, Hong Kong, Hong Kong. Contact e-mail: [email protected] Background: Cognitive impairment (CI) may have a negative
impact on the outcome of patients on peritoneal dialysis (PD), especially in the risk of peritonitis. Methods: This was a two-year prospective single-centre cohort study. Hong Kong Montreal Cognitive Assessment (HK-MoCA) was performed in patients newly started on PD between July 2011 and August 2014, and repeated one year later. Demographics and clinical data including co-morbidities, medications, peritonitis were collected. CI was determined by locally defined HK-MoCA cut-off. Patients were classified as “cognitively impaired” (Ci) if they remained CI or became CI at one-year; remaining patients were classified as “cognitively preserved” (Cp). Results: 104 patients were included. An age older than 65 years old was an independent risk factor for CI (OR 3.37, 95% CI 1.31-8.65, p¼0.01). Ci had a higher PD peritonitis rates (1 episode per 22 patient months vs. 1 episode per 54 patient months, p¼0.01) and longer median length of admissions (11 days (IQR 6-25) vs. 0 day (IQR 0-6), p<0.001) than Cp after exclusion of helpers. Baseline serum albumin level and classification as Ci were independent factors predicting PD related peritonitis (hazard ratios 0.90 (95% CI 0.82-0.98) p¼0.02, 2.48 (95% CI 1.12-5.49) p¼0.03 respectively) using cox regression model. Classification as Ci was associated with shorter peritonitis free survival according to Kaplan Meier curve (p¼0.01). Conclusions: Age is an independent risk factor for CI in PD patients. CI increases the risk of PD-related peritonitis.
P2-303
ADVANCING RESEARCH AND TREATMENT IN FRONTOTEMPORAL LOBAR DEGENERATION (ARTFL) NORTH AMERICAN RARE DISEASE CLINICAL RESEARCH CONSORTIUM: PROGRESS AND CHARACTERIZATION OF INITIAL PARTICIPANTS
Adam L. Boxer1, Howard J. Rosen2, Brad F. Boeve3, Hilary Heuer4, Murray Grossman5, Giovanni Coppola6, Bradford C. Dickerson7, Yvette M. Bordelon8, Christina Dheel3, Kelley Faber9, Julie A. Fields3, Jamie Fong4, Tatiana M. Foroud10, Nupur Ghoshal11, Neil Graff-Radford12, Ging-Yuek Robin Hsiung13, Edward D. Huey14, David J. Irwin5, Kejal Kantarci3, Daniel Kaufer15, Anna Karydas16, David S. Knopman3, Joel H. Kramer1, Walter A. Kukull17, Irene Litvan18, Codrin Lungu19,
P733
Ian R. Mackenzie13, Mario F. Mendez20, Bruce L. Miller1, Matt R. Miller3, Chiadi U. Onyike21, Alex Pantelyat21, Madeline Potter9, Rosa Rademakers12, Erik D. Roberson22, Margaret Sutherland23, Maria Carmela Tartaglia24, Arthur W. Toga25, Sandra Weintraub26, Zbigniew Wszolek12, 1University of California, San Francisco, San Francisco, CA, USA; 2University of California San Francisco, San Francisco, CA, USA; 3Mayo Clinic, Rochester, MN, USA; 4UCSF, San Francisco, CA, USA; 5Penn FTD Center, University of Pennsylvania, Philadelphia, PA, USA; 6Semel Institute for Neuroscience and Behavior at UCLA, Los Angeles, CA, USA; 7Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA; 8University of California Los Angeles, Los Angeles, CA, USA; 9Indiana University, Indianapolis, IN, USA; 10 Indiana University School of Medicine, Indianapolis, IN, USA; 11 Washington University School of Medicine, St. Louis, MO, USA; 12Mayo Clinic, Jacksonville, FL, USA; 13University of British Columbia, Vancouver, BC, Canada; 14Gertrude H. Sergievsky Center at Columbia University, New York, NY, USA; 15University of North Carolina, Chapel Hill, NC, USA; 16 University of California - San Francisco, San Francisco, CA, USA; 17 National Alzheimer’s Coordinating Center, University of Washington, Seattle, WA, USA; 18University of California San Diego, San Diego, CA, USA; 19National Institutes of Health, Washington, DC, USA; 20University of California at Los Angeles, Los Angeles, CA, USA; 21Johns Hopkins University, Baltimore, MD, USA; 22University of Alabama at Birmingham, Birmingham, AL, USA; 23NIH, Bethesda, MD, USA; 24University Health Network, Toronto, ON, Canada; 25University of Southern California, Los Angeles, CA, USA; 26Northwestern University, Chicago, IL, USA. Contact e-mail: [email protected] Background: The Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) consortium aims to 1) characterize the North American population of FTLD patients in preparation for clinical trials and 2) characterize longitudinal changes in familial FTLD (fFTLD) over one year to develop new clinical trial outcome measures. Methods: Participants are either symptomatic with a sporadic FTLD spectrum disorder, including bvFTD, CBS, FTD-ALS, nfvPPA, PSP, or svPPA (Project 1) or are at risk for or symptomatic with fFTLD with a potentially autosomal dominant syndrome, regardless of whether there is a known underlying mutation (Project 2). ARTFL is closely linked to the LEFFTDS project, sharing a common infrastructure and assessments. Participants receive clinical neurological exams, neuropsychological testing, medical history, and blood draw for biomarker collection as well as surveys on lifestyle factors, autoimmune disease history, and attitudes towards clinical trial participation. Familial participants return for a follow-up visit in one year. Asymptomatic family members also undergo MRI. All ARTFL participants are genotyped for FTLD-associated mutations. Results: We report here the characteristics of 566 (288 female (51%)) participants enrolled over 15 sites through December 2016. 280 individuals with sporadic FTLD have been enrolled in Project 1 (mean age 66 years [range 34-89]) with the most common diagnoses being bvFTD (26.5%), PSP (19.3%), and svPPA6bvFTD (15.1%). CDR-SB scores were higher in bvFTD and PSP than svPPA; MDS-UPDRS was highest in PSP >> bvFTD >> svPPA (normal). Project 2 has enrolled 84 symptomatic (mean age 57.9 years; mean CDR-SB: 5.68) and 201 asymptomatic (mean age 47.7; mean CDR-SB: 0.03) fFTLD. Conclusions: ARTFL is actively enrolling participants across North America to characterize sporadic and familial FTLD and
Poster Presentations: Monday, July 17, 2017
81¼9.407, p¼0.0002, R2¼.188, b¼7.897) but not the lPRC, ERC, or PHC (all p-values <.05). Conclusions: Verbal semantic memory tests are commonly used tools in AD diagnosis. We found that the fluency performance for living, but not nonliving, things differentiated NCs from very early AD patients. Further, we showed that only mean cortical thickness of the mPRC predicted living thing fluency performance. These results are in line with the findings of Kivisaari et al. (2012) showing that only mPRC thickness significantly predicted picture naming performance for living relative to nonliving things. Fluency tasks with living thing categories may provide an early marker of incipient AD.
P2-301
Table 2 Percentage of patients in each severeity level generated by the FTD-FRS and the CDR-FTD at baseline and 12-month follow up assessment. Severity level
bvFTD % AD % Assessment (n¼31) (n¼28)
FTD-FRS Very Mild
Baseline Follow up Mild Baseline Follow up Moderate Baseline Follow up Severe Baseline Follow up Very Severe Baseline Follow up Profound Baseline Follow up p value CDR-FTD 0,0 Mild Baseline Follow up 0,5 Moderate Baseline Follow up 1,0 Severe Baseline Follow up 2,0 Very Severe Baseline Follow up p value
DISEASE STAGING IN FRONTOTEMPORAL DEMENTIA AND ALZHEIMER’S DISEASE: THE CONTRIBUTION OF THE FRONTOTEMPORAL DEMENTIA RATING SCALE (FTD-FRS) IN A 12-MONTH FOLLOW-UP STUDY
Thais Bento Lima-Silva1, Valeria S. Bahia1, Mario Amore Cecchini, Sr,1, Luciana Cassimiro1, Henrique Cerqueira Guimaraes2, Leandro Boson Gambogi3, Paulo Caramelli2, Marcio Luiz Figueredo Balthazar4, Benito Pereira Damasceno5, Sonia Maria Dozzi Brucki6, Leonardo Cruz de Souza3,7, Eneida Mioshi8, Ricardo Nitrini6, Monica Sanches Yassuda6,9, 1University of S~ao Paulo, S~ao Paulo, Brazil; 2 Universidade Federal de Minas Gerais, Belo Horizonte, Brazil; 3Federal University of Minas Gerais, Belo Horizonte, Brazil; 4Unicamp, Campinas, Brazil; 5University of Campinas, Campinas, Brazil; 6Medical School of University of S~ ao Paulo, S~ ao Paulo, Brazil; 7Neurology Department, Belo 8 Horizonte, Brazil; University of East Anglia, Norwich, United Kingdom; 9 State University of Campinas UNICAMP, Campinas, Brazil. Contact e-mail: [email protected]
3,23 0,00 3,23 0,00 16,13 19,35 48,39 45,16 16,13 12,90 12,90 22,58 p¼0,018 0,00 0,00 29,03 6,45 51,61 70,97 19,35 22,58 p¼0,012
PPA% (n¼12)
3,57 8,33 3,57 0,00 14,29 0,00 3,57 0,00 32,14 41,67 38,29 41,67 46,43 33,33 50,00 33,33 3,57 8,33 3,57 16,67 0,00 8,33 0,00 8,33 p¼0,067 p¼0,201 0,00 0,00 0,00 0,00 28,57 8,33 14,29 8,33 57,14 66,67 71,43 66,67 14,29 25,00 14,29 25,00 p¼0,068 p¼1,000
Note. p value refers to Wilcoxon matched pairs test. FTD-FRS¼ Frontotemporal Dementia Rating Scale; CDR- FTD¼ Clinical Dementia Rating scale including Language and Behavior domains.
gressive Aphasia (PPA) (8 semantic variant and 4 non-fluent variant) and 28 of AD. Family members or caregivers who had frequent contact with the patients completed the FTD-FRS. The CDR-FTD was completed by physicians. Patients completed the Addenbrooke’s Cognitive Examination-Revised. To determine annual progression, the 12-month follow up assessments were used. Results: There were significant changes in FTD-FRS and CDR-FTD scores from baseline to follow-up for all groups (Table 1). Analyses based on disease severity levels revealed that the FTDFRS and the CDR-FTD scores captured transitions to more severe
Background: Instruments to stage dementia progression are usually based on Alzheimer’s disease (AD) and do not include the specific symptoms of frontotemporal dementia (FTD). The FTD-FRS was elaborated to stage FTD and recently an extended version of the Clinical Dementia Rating scale included Language and Behavior domains (CDR-FTD). Methods: Participants were 71 individuals, aged 40+ years with at least two years of schooling: 31 received the diagnosis of behavioral variant (bv) FTD, 12 of Primary Pro-
Table 1 Means and standard deviations for the FTD-FRS and CDR-FTD sum of boxes scores at baseline and 12-month follow up assessment bvFTD (n¼31)
Age (50-87 anos) Schooling (0-21 anos) ACE-R Total MMSE FTD-FRS Baseline Follow up P* value CDR-FTD Baseline Follow up P* value
AD (n¼28)
PPA (n¼12)
Media
DP
Media
DP
Media
DP
P**
66,94 11,74 71,71 23,61 27,53 21,83 0,002 8,06 9,00 <0,001
9,26 4,57 16,36 4,96 (26,25) (19,26)
74,15 9,43 67,63 23,25 48,45 45,36 0,018 7,07 7,84 0,001
9,22 4,49 11,51 3,49 (25,62) (22,56)
61,42 14,33 65,75 23,75 47,78 32,22 0,069 9,96 10,38 0,043
5,87 4,40 12,80 3,81 (32,79) (24,63)
<0,001 a,b <0,001 b 0,271 0,696 0,004 a <0,001 a
(3,59) (3,31)
(3,16) (2,88)
(4,22) (4,02)
0,105 0,082
Note: *p value refers to Wilcoxon matched pairs test. FTD-FRS¼ Frontotemporal Dementia Rating Scale; CDR-FTD¼ Clinical Dementia Rating scale including Language and Behavior domains; MMSE+ Mini Mental State Examination; ACE-R¼ Addenbrooke’s Cognitive Examination-Revised. **p-valor Kruskal-Wallis test, followed by the multiple comparison test: a.bvFTDsAD, b.ADsPPA; c.bvFTDsPPA. Multiple comparisons test without statistical significance.
Poster Presentations: Monday, July 17, 2017
stages from baseline to 12-month follow-up in the bvFTD group only, however, results for the AD group approached statistical significance (Table 2). Conclusions: FTD-FRS and the CDR-FTD can aid in staging and determining progression in FTD.
P2-302
ONE-YEAR INTERVAL MONTREAL COGNITIVE ASSESSMENT AND RISK OF PERITONITIS IN SELF-CARE PERITONEAL DIALYSIS PATIENTS
Yat Fung Shea1, Mi Suen Connie Lee1, Ming Yee Maggie Mok1, Man Fai Lam1, Leung Wing Chu1,2, Hon Wai Felix Chan1, Tak-Mao Chan1, 1 Queen Mary Hospital, Hong Kong, Hong Kong; 2The University of Hong Kong, Hong Kong, Hong Kong. Contact e-mail: [email protected] Background: Cognitive impairment (CI) may have a negative
impact on the outcome of patients on peritoneal dialysis (PD), especially in the risk of peritonitis. Methods: This was a two-year prospective single-centre cohort study. Hong Kong Montreal Cognitive Assessment (HK-MoCA) was performed in patients newly started on PD between July 2011 and August 2014, and repeated one year later. Demographics and clinical data including co-morbidities, medications, peritonitis were collected. CI was determined by locally defined HK-MoCA cut-off. Patients were classified as “cognitively impaired” (Ci) if they remained CI or became CI at one-year; remaining patients were classified as “cognitively preserved” (Cp). Results: 104 patients were included. An age older than 65 years old was an independent risk factor for CI (OR 3.37, 95% CI 1.31-8.65, p¼0.01). Ci had a higher PD peritonitis rates (1 episode per 22 patient months vs. 1 episode per 54 patient months, p¼0.01) and longer median length of admissions (11 days (IQR 6-25) vs. 0 day (IQR 0-6), p<0.001) than Cp after exclusion of helpers. Baseline serum albumin level and classification as Ci were independent factors predicting PD related peritonitis (hazard ratios 0.90 (95% CI 0.82-0.98) p¼0.02, 2.48 (95% CI 1.12-5.49) p¼0.03 respectively) using cox regression model. Classification as Ci was associated with shorter peritonitis free survival according to Kaplan Meier curve (p¼0.01). Conclusions: Age is an independent risk factor for CI in PD patients. CI increases the risk of PD-related peritonitis.
P2-303
ADVANCING RESEARCH AND TREATMENT IN FRONTOTEMPORAL LOBAR DEGENERATION (ARTFL) NORTH AMERICAN RARE DISEASE CLINICAL RESEARCH CONSORTIUM: PROGRESS AND CHARACTERIZATION OF INITIAL PARTICIPANTS
Adam L. Boxer1, Howard J. Rosen2, Brad F. Boeve3, Hilary Heuer4, Murray Grossman5, Giovanni Coppola6, Bradford C. Dickerson7, Yvette M. Bordelon8, Christina Dheel3, Kelley Faber9, Julie A. Fields3, Jamie Fong4, Tatiana M. Foroud10, Nupur Ghoshal11, Neil Graff-Radford12, Ging-Yuek Robin Hsiung13, Edward D. Huey14, David J. Irwin5, Kejal Kantarci3, Daniel Kaufer15, Anna Karydas16, David S. Knopman3, Joel H. Kramer1, Walter A. Kukull17, Irene Litvan18, Codrin Lungu19,
P733
Ian R. Mackenzie13, Mario F. Mendez20, Bruce L. Miller1, Matt R. Miller3, Chiadi U. Onyike21, Alex Pantelyat21, Madeline Potter9, Rosa Rademakers12, Erik D. Roberson22, Margaret Sutherland23, Maria Carmela Tartaglia24, Arthur W. Toga25, Sandra Weintraub26, Zbigniew Wszolek12, 1University of California, San Francisco, San Francisco, CA, USA; 2University of California San Francisco, San Francisco, CA, USA; 3Mayo Clinic, Rochester, MN, USA; 4UCSF, San Francisco, CA, USA; 5Penn FTD Center, University of Pennsylvania, Philadelphia, PA, USA; 6Semel Institute for Neuroscience and Behavior at UCLA, Los Angeles, CA, USA; 7Massachusetts General Hospital/Harvard Medical School, Boston, MA, USA; 8University of California Los Angeles, Los Angeles, CA, USA; 9Indiana University, Indianapolis, IN, USA; 10 Indiana University School of Medicine, Indianapolis, IN, USA; 11 Washington University School of Medicine, St. Louis, MO, USA; 12Mayo Clinic, Jacksonville, FL, USA; 13University of British Columbia, Vancouver, BC, Canada; 14Gertrude H. Sergievsky Center at Columbia University, New York, NY, USA; 15University of North Carolina, Chapel Hill, NC, USA; 16 University of California - San Francisco, San Francisco, CA, USA; 17 National Alzheimer’s Coordinating Center, University of Washington, Seattle, WA, USA; 18University of California San Diego, San Diego, CA, USA; 19National Institutes of Health, Washington, DC, USA; 20University of California at Los Angeles, Los Angeles, CA, USA; 21Johns Hopkins University, Baltimore, MD, USA; 22University of Alabama at Birmingham, Birmingham, AL, USA; 23NIH, Bethesda, MD, USA; 24University Health Network, Toronto, ON, Canada; 25University of Southern California, Los Angeles, CA, USA; 26Northwestern University, Chicago, IL, USA. Contact e-mail: [email protected] Background: The Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) consortium aims to 1) characterize the North American population of FTLD patients in preparation for clinical trials and 2) characterize longitudinal changes in familial FTLD (fFTLD) over one year to develop new clinical trial outcome measures. Methods: Participants are either symptomatic with a sporadic FTLD spectrum disorder, including bvFTD, CBS, FTD-ALS, nfvPPA, PSP, or svPPA (Project 1) or are at risk for or symptomatic with fFTLD with a potentially autosomal dominant syndrome, regardless of whether there is a known underlying mutation (Project 2). ARTFL is closely linked to the LEFFTDS project, sharing a common infrastructure and assessments. Participants receive clinical neurological exams, neuropsychological testing, medical history, and blood draw for biomarker collection as well as surveys on lifestyle factors, autoimmune disease history, and attitudes towards clinical trial participation. Familial participants return for a follow-up visit in one year. Asymptomatic family members also undergo MRI. All ARTFL participants are genotyped for FTLD-associated mutations. Results: We report here the characteristics of 566 (288 female (51%)) participants enrolled over 15 sites through December 2016. 280 individuals with sporadic FTLD have been enrolled in Project 1 (mean age 66 years [range 34-89]) with the most common diagnoses being bvFTD (26.5%), PSP (19.3%), and svPPA6bvFTD (15.1%). CDR-SB scores were higher in bvFTD and PSP than svPPA; MDS-UPDRS was highest in PSP >> bvFTD >> svPPA (normal). Project 2 has enrolled 84 symptomatic (mean age 57.9 years; mean CDR-SB: 5.68) and 201 asymptomatic (mean age 47.7; mean CDR-SB: 0.03) fFTLD. Conclusions: ARTFL is actively enrolling participants across North America to characterize sporadic and familial FTLD and