Diagnosing Frontotemporal Lobar Degeneration

Recruiting

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

• Registration Number: NCT02964637
• Lead Sponsor: University Health Network, Toronto

Brief Summary

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

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 Timeline

• Study Start: 2015-08
• Study First Submitted: 2016-09-23
• Study First Submitted QC: 2016-11-10
• Study First Posted: 2016-11-16
• Status Verified: 2019-04
• Last Update Submitted: 2025-03-25
• Last Update Posted: 2025-03-30
• Primary Completion: 2026-12
• Study Completion: 2026-12

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 100

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 & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Progressive supranuclear palsy	Observational Study	Observational Study
Behavoral variant FTD	Observational Study	Observational Study
Semantic variant PPA	Observational Study	Observational Study
Non-fluent variant PPA	Observational Study	Observational Study
FTD-motor neuron disease	Observational Study	Observational Study
Healthy controls	Observational Study	Observational Study
Corticobasal syndrome	Observational Study	Observational Study

Primary Outcome Measures

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

Trial Locations

Locations (1)

Toronto Western Hospital, University Health Network; 🇨🇦 Toronto, Ontario, Canada