8101: Advancing Research and Treatment for Frontotemporal Lobar Degeneration [ARTFL]: Research Projects 1 & 2

Status: Recruiting


Study Summary

This research study is being done to learn more about normal thinking and behavior, thinking and behavior problems, movement problems, Frontotemporal Dementia and other forms of dementia or movement problems.

For Diseases:

  • behavioral variant frontotemporal dementia (bvFTD)
  • primary progressive aphasia (PPA)
  • semantic variant primary progressive aphasia (svPPA)
  • nonfluent variant primary progressive aphasia (nfvPPA)
  • frontotemporal dementia with amyotrophic lateral sclerosis (FTD/ALS)
  • amyotrophic lateral sclerosis alone
  • corticobasal syndrome (CBS)
  • progressive supranuclear palsy (PSP)
  • oligosymptomatic PSP (oPSP)


Frontotemporal Lobar Degeneration (FTLD) is the neuropathological term for a related group of rare neurodegenerative disorders that cause a variety of impairments in social function and personality, cognitive ability, language, and motor function. In most cases, this disease begins when a person is in their 40s-60s. The prevalence of the behavioral and cognitive syndromes is approximately 15-22 per 100,000; the most common motor syndrome has a prevalence of 6.4 per 100,000 in the US. All FTLD disorders are regularly fatal and generally lead to death within 10 years of diagnosis, sometimes much faster. There are no effective treatments for any FTLD disorder, and it is hard to accurately diagnose or potentially measure the effectiveness of new therapies.

Research on the different FTLD disorders traditionally has been performed either by behavioral neurologists who focus on behavior, cognition and language, or by movement disorder neurologists who focus on movement abnormalities. There is a growing agreement that research needs to incorporate both to successfully develop effective therapies. The ARTFL consortium brings together leading behavioral and movement disorder researchers in North America to focus on studies that enable research for FTLD.

The primary objectives of this study are:

  1. To build a reliable FTLD clinical research network to support treatment and clinical trials that focus on the prevention of FTLD.
  2. To determine the clinical characteristics of sporadic FTLD syndromes in patients who would meet typical clinical trial eligibility criteria, and the barriers to clinical trial participation.
  3. To develop a familial FTLD (f-FTLD) cohort for clinical trials and biomarker studies.

About this Study

This is an observational study of about 1560 individuals with FTLD syndromes. If you participate in this study, you will have an evaluation of your memory and thinking skills and have a physical examination by a neurologist. You and your study partner will complete some questionnaires. You will be asked to have a sample of blood drawn, and you may be asked to participate in magnetic resonance imaging (MRI) of the brain or lumbar punctures for cerebrospinal fluid. The study staff may look at your medical records so that we can collect information about your medical history. Participation in the study will take a total of about 8 hours over the course of a day or may be split into two days. If FTLD runs in your family, you may be asked to return in one year for a follow-up visit where many of the assessments you completed during your first study visit will be repeated.


Targeted Enrollment

To be eligible to participate, you must:

  • Must meet one of the following research diagnostic criteria for a FTLD syndrome:
    • behavioral variant frontotemporal dementia (bvFTD)
    • primary progressive aphasia (PPA)
    • semantic variant primary progressive aphasia (svPPA)
    • nonfluent variant primary progressive aphasia (nfvPPA)
    • frontotemporal dementia with amyotrophic lateral sclerosis (FTD/ALS)
    • amyotrophic lateral sclerosis alone
    • corticobasal syndrome (CBS)
    • progressive supranuclear palsy (PSP)
    • oligosymptomatic PSP (oPSP)
  • OR have a strong family history of FTLD syndromes
  • Between 18 and 85 (inclusive) years of age
  • Able to walk (with assistance) at the time of enrollment
  • Have a reliable study partner who can provide an independent evaluation of functioning
  • Have Mini Mental State Exam (MMSE) scores between 15 – 30 (inclusive)

You are not eligible to participate if:

  • There is a known presence of a structural brain lesion (e.g. tumor) that could reasonably explain symptoms in a symptomatic participant without a known f-FTLD-causing mutation.
  • There is a known presence of an Alzheimer’s disease causing mutation in PSEN1, PSEN2 or APP; or neuropathological evidence for Alzheimer’s disease as a cause of syndrome (from brain biopsy).
  • There is a previous history of Korsakoff encephalopathy, severe alcohol dependence (within 5 years of onset of dementia), frequent alcohol or other substance intoxication, or other neurological disorder (such as multiple sclerosis).
  • There is evidence of B12 deficiency, hypothyroidism, HIV positive, renal failure, liver failure, respiratory failure (requiring oxygen), extra-axial brain tumor, large cerebral infarct that could account for clinical syndrome, large confluent white matter lesions (grades 3 or 4), or significant systemic medical illnesses such as deteriorating cardiovascular disease.

How to participate:

In order to participate in a study, you must personally contact the study coordinator of any of the participating institutions by phone or by e-mail. Please use the information below to inquire about participation.

United States


University of Alabama, Birmingham
Study Contact: Emily McKinley, MS, MSPH
Phone: 205-996-3659
E-mail: emckinley@uabmc.edu


University of California, Los Angeles
Study Contact: Elvira Jimenez
Phone: 310-478-3711x40584
E-mail: elvira@ucla.edu

University of California, San Diego
Study Contact: Patricia Torres
Phone: 858-246-2537
E-mail: patorres@ucsd.edu

University of California, San Francisco
Study Contact: Reilly Dever / Ping Wang
Phone: 415-476-0670 / 415-502-7518
E-mail: Reilly.Dever@ucsf.edu / pwang@ucsf.edu


Mayo Clinic College of Medicine, Jacksonville
Study Contact: Dana Haley
Phone: 904-953-9680
E-mail: Haley.Dana@mayo.edu


Northwestern University, Chicago
Study Contact: Michaela Riley
Phone: 312-503-5103
E-mail: Michaela.riley@northwestern.edu


Johns Hopkins University School of Medicine, Baltimore
Study Contact: Ann Fishman
Phone: 410-502-5816
E-mail: ann.fishman@jhu.edu


Massachusetts General Hospital, Boston
Study Contact: Samantha Krivensky
Phone: 617-726-6205
E-mail: skrivensky@mgh.harvard.edu


Mayo Clinic, Rochester
Study Contact: Ruth Kraft
Phone: 507-538-9487
E-mail: Kraft.Ruth@mayo.edu


Washington University School of Medicine, St. Louis
Study Contact: Tina Nolte
Phone: 314-362-3839
E-mail: nolte.tina@wustl.edu

New York

Columbia University, New York
Study Contact: Masood Manoochehri
Phone: 212-305-5710
E-mail: mm2626@cumc.columbia.edu

North Carolina

University of North Carolina School of Medicine, Chapel Hill
Study Contact: Jessica Ferrall
Phone: 919-962-8852
E-mail: jferrall@neurology.unc.edu


Case Western Reserve University, Cleveland
Study Contact: Fran Lissemore, PhD
Phone: 216-464-6203
E-mail: Frances.Lissemore@UHhospitals.org


University of Pennsylvania School of Medicine, Philadelphia
Study Contact: Christine Ray
Phone: 215-349-5873
E-mail: rayc@mail.med.upenn.edu


University of Texas Southwestern Medical Center, Dallas
Study Contact: Jana Windsor
Phone: 214-648-8543
E-mail: Jana.windsor@utsouthwestern.edu


British Columbia

University of British Columbia, Vancouver
Study Contact: Pheth Sengdy
Phone: 604-822-7989
E-mail: Pheth.Sengdy@vch.ca


University of Toronto Memory Clinic – Toronto Western Hospital, Toronto
Study Contact: Behnaz Ghazanfari
Phone: 416-603-5800x5910
E-mail: Behnaz.Ghazanfari@uhnresearch.ca