UPenn Observational Research Repository on Neurodegenerative Disease

Study Overview
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Brief Summary
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The aim of this study is to create a repository of both cross-sectional and longitudinal data, including cognitive, linguistic, imaging and biofluid biological specimens, for neurodegenerative disease research and treatment.

Detailed Description
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The Principal Investigator (PI) at the University of Pennsylvania seeks to better understand neurodegenerative diseases and is continually expanding research efforts and collaborations regarding the factors which may contribute to these illnesses. Investigators seek to better understand the basis of neurodegenerative conditions by creating a multimodal repository, including: clinical data such as demographic characteristics, vital signs and motor scales; cognitive and speech data; neuroimaging data; and biological specimens with associated biofluid biomarkers and genetic data. Investigators pursue acquiring these data from neurodegenerative disease patients, people at risk for neurodegenerative disease due to a family history, and unaffected adults. Targeted conditions include frontotemporal degeneration (FTD), primary progressive aphasia PPA), amyotrophic lateral sclerosis (ALS), progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), familial frontotemporal lobar degeneration (fFTLD), non-amnestic variants of Alzheimer's disease including logopenic progressive aphasia and posterior cortical atrophy, and Lewy body disease. This study aims to collect clinical and cognitive data, imaging data, and biospecimen samples from people whose background can inform research and treatment for neurodegenerative diseases, and make these samples and data available to qualified researchers at the University of Pennsylvania and collaborating academic centers and industry partners.

Official Title
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University of Pennsylvania Centralized Observational Research Repository on Neurodegenerative Disease (UNICORN)

Conditions
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Frontotemporal Degeneration(FTD), Primary Progressive Aphasia(PPA), Familial Frontotemporal Lobar Degeneration (fFTLD), Amyotrophic Lateral Sclerosis(ALS), Lewy Body Disease(LBD), Progressive Supranuclear Palsy(PSP), Corticobasal Syndrome(CBS), Posterior Cortical Atrophy(PCA), Alzheimer's Disease(AD)

Intervention / Treatment
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* Other: No intervention


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: This protocol will include 3 groups of people: People with a clinical diagnosis of a neurodegenerative disease. such as frontotemporal degeneration(FTD), primary progressive aphasia(PPA), Lewy body disease(LBD), amyotrophic lateral sclerosis(ALS), progressive supranuclear palsy(PSP), corticobasal syndrome(CBS), posterior cortical atrophy(PCA), Alzheimer's disease(AD), Parkinson's disease(PD) People with a family history of neurodegenerative disease who may or may not be symptomatic, and may or may not be mutation carriers such as familial frontotemporal lobar degeneration (fFTLD) or familial ALS, People with no known neurological disease who will provide control data. Exclusion Criteria: Anyone who is under the age of 18. Anyone with a condition or in a situation which, in the Investigator's opinion, could confound the study findings or may interfere significantly with a person's participation, including but not limited to neurological, psychological and other medical conditions (such as cardiac, neurosurgical, infectious conditions). Individual participants may be excluded from some, but not all, study procedures for safety reasons when they have a contraindication or at the discretion of the Investigator. For example, persons with metal implants which are not MRI-safe will not be able to take part in imaging, and those on blood thinning medications may not be able to take part in lumbar puncture. Pregnant women; if a woman becomes pregnant during the study, research activities that may increase risk to the patient and the unborn fetus will be stopped until the end of pregnancy, at which point participation can be resumed.

Ages Eligible for Study
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Minimum Age: 18 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 |
| --- | --- |
| Cross-sectional<br> | Other: No intervention<br>* No intervention<br>|
| Longitudinal<br> | Other: No intervention<br>* No intervention<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Status of progression - changes in neuropsychological tests. | The changes of neuropsychological tests (well known cognitive measures such as: Naming test, CVLT, MoCA, Oral Trails, etc.) in neurodegenerative diseases over time. | This is a natural history study-participants are followed from date of enrollment until death, withdraw, or funding is no longer available, or until 600 months have passed. | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Status of progression - changes in language processing. | The effect of changes in language processing (e.g. effortful speech, lexical-semantic representations) as categorized by impaired speech fluency, motor speech deficits and semantic memory deficits in neurodegenerative diseases over time. | This is a natural history study-participants are followed from date of enrollment until death, withdraw, or funding is no longer available, or until 600 months have passed. | 
| Status of progression - changes in social disinhibition. | The effect of changes in social behavioral testing, which categorized by rule violation (social disinhibition) progression in neurodegenerative diseases over time. | This is a natural history study-participants are followed from date of enrollment until death, withdraw, or funding is no longer available, or until 600 months have passed. | 
| Status of progression - changes in biofluids. | The change of disease status as categorized by biomarkers in biofluids in neurodegenerative diseases over time. | This is a natural history study-participants are followed from date of enrollment until death, withdraw, or funding is no longer available, or until 600 months have passed. | 
| Status of progression -changes in Neuroimaging | The progressive changes of images in multimodal neuroimaging techniques in neurodegenerative diseases over time. | This is a natural history study-participants are followed from date of enrollment until death, withdraw, or funding is no longer available, or until 600 months have passed. |