Diagnosing Frontotemporal Lobar Degeneration

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

Detailed Description
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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.

Official Title
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Multimodal Assessment For Predicting Specific Pathological Substrate in Frontotemporal Lobar Degeneration

Conditions
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Corticobasal Syndrome, Progressive Supranuclear Palsy, Behavioral Variant Frontotemporal Dementia, Semantic Dementia, Progressive Nonfluent Aphasia, Amyotrophic Lateral Sclerosis and/or Frontotemporal Dementia

Intervention / Treatment
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* Other: Observational Study


Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Progressive supranuclear palsy<br>Observational Study | Other: Observational Study<br> <br> |
| Corticobasal syndrome<br>Observational Study | Other: Observational Study<br> <br> |
| Behavoral variant FTD<br>Observational Study | Other: Observational Study<br> <br> |
| Semantic variant PPA<br>Observational Study | Other: Observational Study<br> <br> |
| Non-fluent variant PPA<br>Observational Study | Other: Observational Study<br> <br> |
| FTD-motor neuron disease<br>Observational Study | Other: Observational Study<br> <br> |
| Healthy controls<br>Observational Study | Other: Observational Study<br> <br> |

What is the study measuring?
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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 |