Diagnosing Frontotemporal Lobar Degeneration

Multimodal Assessment For Predicting Specific Pathological Substrate in Frontotemporal Lobar Degeneration

To establish diagnostic tools to make an accurate clinical and pathological diagnosis of patients with clinical FTLD syndromes

Study Overview

• Status: Unknown
• Conditions: Progressive Supranuclear Palsy; Semantic Dementia; Corticobasal Syndrome; Progressive Nonfluent Aphasia; Behavioral Variant Frontotemporal Dementia; Amyotrophic Lateral Sclerosis and/or Frontotemporal Dementia
• Intervention / Treatment: Other: Observational Study

Detailed Description

The goal of this study is to determine the best diagnostic test for diagnosing frontotemporal lobar degeneration. To accomplish this, the current study will evaluate different tests: brain imaging, skin biopsy, body fluid samples (blood and cerebrospinal fluid), thinking abilities, everyday functioning, and brain autopsy. The study team hopes that this information can be used to guide diagnosis and further understanding of mechanism of disease in Frontotemporal Lobar Degeneration and possibly treatment in the future.

• Study Type: Observational
• Enrollment (Anticipated): 100

Contacts and Locations

• Study Contact: Name: Cristina Salvo, BSc, MD; Phone Number: 416-507-6880; Email: cristina.salvo@uhn.ca

Study Locations

• Canada
  • Ontario
    • Toronto, Ontario, Canada, M5T 2S8
      • Recruiting
      • Toronto Western Hospital, University Health Network
      • Contact: Carmela Tartaglia, MD; Phone Number: 416-603-5483; Email: carmela.tartaglia@uhn.ca

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 90 years (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients with a diagnosis of progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), semantic variant primary progressive aphasia (sv-PPA), non-fluent variant PPA (nfv-PPA), behavioral variant frontotemporal dementia (bvFTD) or FTD-motor neuron disease (FTD-MND).

Description

Inclusion Criteria:

• Participant must have a reliable study partner who can provide an independent evaluation of functioning.
• Able to read, understand and speak English for neuropsychological testing.
• All subjects must meet one of these diagnostic criteria (A) probable behavioral variant FTD, (B) MRI-supported non-fluent variant PPA; (C) MRI-supported semantic variant PPA and [18F]T807 negative (D) probable CBS: using current criteria for CBS(27); (E) PSP: inclusion criteria for PSP are based upon the National Institute of Neurological Disorders and Stroke Society of Progressive Supranuclear Palsy (NINDS-SPSP) (F) FTD-MND
• Control subjects must have a normal neurological exam, a CDR sum of boxes = 0, and MMSE score equal to or greater than 28

Exclusion Criteria:

• Patients with clinical, imaging or CSF A beta/ tau profile consistent with AD
• History of traumatic brain injury, brain tumors, stroke or other neurological or psychiatric disorders that can explain symptoms will be excluded.
• Premenopausal women will be asked to consent to a pregnancy test prior to each scan as pregnant women will be excluded from study because of potential harm to fetus from PET study.
• Presence of pacemakers, aneurysm clips, artificial heart valves, ear implants, metal fragments or foreign objects in the eyes, skin or body.

Study Plan

How is the study designed?

Number of groups / cohorts

7

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Progressive supranuclear palsy; Observational Study	Other: Observational Study
Corticobasal syndrome; Observational Study	Other: Observational Study
Behavoral variant FTD; Observational Study	Other: Observational Study
Semantic variant PPA; Observational Study	Other: Observational Study
Non-fluent variant PPA; Observational Study	Other: Observational Study
FTD-motor neuron disease; Observational Study	Other: Observational Study
Healthy controls; Observational Study	Other: Observational Study

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Structural and Functional Diffferences between the FTLD groups via MRI of the brain	Differences in brain volumes and resting state functional connectivity	One time visit through study completion of 5 years
Differences between the FTLD groups via PET imaging of the brain	Differences in ligand uptake	One time visit through study completion of 5 years

Collaborators and Investigators

• Sponsor: University Health Network, Toronto
• Investigators: Principal Investigator: Maria C Tartaglia, M.D., Toronto Western Hospital, UHN; Tanz CRND

Study record dates

Study Major Dates

• Study Start: August 1, 2015
• Primary Completion (ANTICIPATED): December 1, 2019
• Study Completion (ANTICIPATED): December 1, 2019

Study Registration Dates

• First Submitted: September 23, 2016
• First Submitted That Met QC Criteria: November 10, 2016
• First Posted (ESTIMATE): November 16, 2016

Study Record Updates

• Last Update Posted (ACTUAL): April 16, 2019
• Last Update Submitted That Met QC Criteria: April 15, 2019
• Last Verified: April 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Mental Disorders; Metabolic Diseases; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Eye Diseases; Neurologic Manifestations; Neurobehavioral Manifestations; Neurocognitive Disorders; Neuromuscular Diseases; Basal Ganglia Diseases; Movement Disorders; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Tauopathies; Cranial Nerve Diseases; Ocular Motility Disorders; Language Disorders; Communication Disorders; Paralysis; Speech Disorders; Ophthalmoplegia; Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Dementia; Aphasia; Frontotemporal Dementia; Aphasia, Primary Progressive; Pick Disease of the Brain; Supranuclear Palsy, Progressive; Aphasia, Broca; Frontotemporal Lobar Degeneration; Primary Progressive Nonfluent Aphasia
• Other Study ID Numbers: 14-8398-A

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO