Progressive Supranuclear Palsy
Conditions
Brief summary
The purpose of this cohort study is to develop a reliable biomarker in progressive nuclear palsy (PSP).
Detailed description
Progressive supranuclear palsy is a rapidly progressive neurodegenerative disease without a cure. Thus, the development of a biomarker that reflects and monitors disease severity in PSP is critical for early diagnosis and performing a successful clinical trial. Thus, we will prospectively recruit patients with PSP and collect comprehensive clinical, imaging and blood biomarkers at baseline with longitudinal follow-up for 1 year.
Interventions
None listed
Sponsors
SMG-SNU Boramae Medical Center
Seoul National University Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
50 Years to 80 Years
Healthy volunteers
Yes
Inclusion criteria
for the patient group: * Age 50 to 80 years, male or female * Progressive supranuclear palsy (PSP) patients who are diagnosed as Probable, Possible or suggestive PSP with Movement Disorder society diagnostic criteria for PSP (Hoglinger et al., 2017)
Exclusion criteria
for the patient group: * Subjects with clinically significant psychiatric illness * Subjects with cancer or severe medical illness * Lactating, pregnant, or possibly pregnant * Subjects with small vessel disease (\> grade II) in brain MRI or other structural lesions by causes other than PSP * Subjects with severe dementia patients (MMSE \< 19 or MoCA \<13 or General deterioration scale \>= 5) Inclusion Criteria for the healthy control group: * Age 50 to 80 years, male or female * Those who agreed to participate in this study
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change in the Progressive Supranuclear Palsy Rating Scale (PSP-RS) | From the baseline to 6 months and 12 months follow-up | A scale for assessment of motor and non-motor symptom severity in PSP patients. The PSPRS comprises 28-items with total score ranges from 0 (normal) to 100. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Change in the Schwab & England Activity of Daily Living scale (SEADL) | From the baseline to 6 months and 12 months follow-up | A scale for activity of daily living assessment that ranges from 0% (complete dependence) to 100% (total independence). The decline in the ADL percentage over time indicates worsening. |
| Change in cognitive battery for seoul neuropsychological screening battery (SNSB-2) scale | From the baseline to 6 months and 12 months follow-up | SNSB-2 measures 5 cognitive domain including memory, frontal executive function, attention, visuospatial, language. SNSB scores are provided as age, sex normalized score (z-score) which have a mean of 0 with standard deviation of 1. Higher score indicates better performance. |
| Change in MoCA (Montreal cognitive assessment) | From the baseline to 6 months and 12 months follow-up | MoCA scale measures global cognitive status including attention and concentration, executive function, memory, language, visuospatial skills, conceptual thinking, calculations, and orientation. MoCA score ranges from 0 to 30. Higher score indicates better performance |
| Change in MMSE(Mini-mental state examination) | From the baseline to 6 months and 12 months follow-up | MMSE scale measures global cognitive status that ranges from 0 to 30. Higher score indicates better performance |
Other
| Measure | Time frame | Description |
|---|---|---|
| Amyloid beta 42 (Aβ-42) | From the baseline to 12 months follow-up | Change in serum and/or plasma Amyloid beta 42 (Aβ-42) in PSP patients |
| Proteomic markers | From the baseline to 12 months follow-up | Change in blood-based proteomic markers |
| FDG PET (18Fluorodeoxyglucose) | From the baseline to 12 months follow-up | Evaluation of metabolic activity and patterns of the brain |
| Retina biomarkers by OCT imaging | From the baseline to 12 months follow-up | Exploratory analysis of OCT and OCTA imaging |
| Genomic markers | Baseline | Identification of genetic markers in PSP that differentiates individuals with different clinical presentation and progression. |
| Tau PET (18F-taucipir) | From the baseline to 12 months follow-up | Evaluation of the tau aggregation in the brain |
| Structural analysis by 3T MRI | From the baseline to 12 months follow-up | Evaluation of the degenerative change in the brain |
| Change in p-tau 181 | From the baseline to 12 months follow-up | Change in serum and/or plasma phosphorylated tau (p-tau181) in PSP patients |
| total tau (T-tau) | From the baseline to 12 months follow-up | Change in serum and/or plasma total tau (T-tau) in PSP patients |
Countries
South Korea
Contacts
Primary ContactJee-Young Lee, M.D.
Outcome results
None listed