A Convenient Prognostic Tool and Staging System for Progressive Supranuclear Palsy

Area of Science:

• Neurology
• Neurodegenerative Diseases

Background:

• Progressive Supranuclear Palsy (PSP) causes significant disability and mortality.
• Current treatments for PSP are limited, necessitating better prognostic tools.

Purpose of the Study:

• To calculate the time to specific disease milestones and death in PSP patients.
• To develop a prognostic tool for PSP care planning.

Main Methods:

• A cohort of 417 PSP-Richardson syndrome patients was followed from 1995 to 2016.
• The Progressive Supranuclear Palsy Rating Scale (PSPRS) was used to assess disease progression.
• Median times to 13 milestones (including death and disability levels) were calculated using patient data.

Main Results:

• Median times to milestones were derived, with variations based on sex and initial disease progression velocity.
• Time to death varied significantly: 4.8 years for a male patient with a low PSPRS score and slow progression, versus 1.8 years for a female patient with a high PSPRS score and rapid progression.
• Sex and initial progression velocity were significant predictors of time to death.

Conclusions:

• A practical, non-invasive tool was created to estimate time to 13 life-altering milestones for PSP patients.
• This tool aids in counseling patients and families regarding PSP progression and care.