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

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.

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.