Frontotemporal Degeneration, Multiple System Atrophy, Progressive Supranuclear Palsy
Conditions
Brief summary
This is an observational study that aims to better understand the genetic causes of frontotemporal degeneration (FTD), Multiple Systems Atrophy (MSA), and Progressive Supranuclear Palsy (PSP). It is hoped the information gathered in this study will help lead to better diagnostics and future treatments.
Detailed description
Comparative and longitudinal studies reveal clinical differences between subgroups of patients with frontotemporal dementia (FTD), including Progressive Non-fluent Aphasia (PNFA), Semantic Dementia (SD), patients with a disorder of social comportment and personality (SOC), and non-aphasic patients with executive dysfunction (EXEC). MRI studies of cortical atrophy and fMRI studies show correlated neural defects in FTD subgroups. The investigators will obtain converging evidence from multiple sources to test hypotheses about the neural basis for cognitive functions such as semantic memory, grammatical processing, and social functioning in these FTD subgroups, while improving clinical care for these patients. Recent studies have linked progressive supra nuclear palsy (PSP) and multiple systems atrophy (MSA) to FTD. The investigators will obtain comparable neuropsychological and biomarker data in order to compare these patient groups.
Interventions
OTHERNone-Observation Only
No intervention-this is an observational study
Sponsors
University of Pennsylvania
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Inclusion criteria
* Individuals who have been diagnosed with FTD, PSP, and MSA
Exclusion criteria
* Individuals under 18 years of age * People with pacemakers or certain metallic implants * pregnant women
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Progression | This is a natural history study - participants are followed from date of enrollment until death, withdrawl, or funding is no longer available, or until 984 months have passed | Changes over time in MRI and cognitive testing data. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Impaired semantic memory in Semantic Dementia (SD). | This is a natural history study - participants are followed from date of enrollment until death, withdrawl, or funding is no longer available, or until 984 months have passed | The impact of cortical atrophy on language processing. |
Other
| Measure | Time frame | Description |
|---|---|---|
| Effortful speech in Progressive Nonfluent Aphasia (PNFA) | This is a natural history study - participants are followed from date of enrollment until death, withdrawl, or funding is no longer available, or until 984 months have passed | Speech samples related to cortical atrophy seen on MRI and fMRI studies of verb past tense inflection. |
| Social disinhibition and rule violation in FTD | This is a natural history study - participants are followed from date of enrollment until death, withdrawl, or funding is no longer available, or until 984 months have passed | Relationship of cognitive testing and MRI data and changes over time. |
Countries
United States
Outcome results
None listed