Investigating differences in young- and late-onset progressive supranuclear palsy

Area of Science:

• Neurology
• Neurodegenerative Diseases
• Clinical Phenotyping

Background:

• The influence of age of onset on progressive supranuclear palsy (PSP) phenotypes remains underexplored.
• A hypothesis suggests distinct presentations between young-onset PSP (YOPSP) and late-onset PSP (LOPSP).

Purpose of the Study:

• To compare the clinical phenotypes and the rate of disability progression between YOPSP and LOPSP.
• To identify differences in presenting symptoms and diagnostic criteria fulfillment.

Main Methods:

• Retrospective analysis of 107 patients diagnosed with PSP according to MDS 2017 criteria.
• Patients categorized into YOPSP and LOPSP based on a 65-year age cutoff.
• Phenotypes, symptoms, MDS core criteria, and disease severity (PSP-RS) were compared.

Main Results:

• YOPSP patients showed a higher prevalence of the PSP speech/language (SL) phenotype (18% vs 0%) and aphasia (16% vs 1.4%).
• Speech and language dysfunction (C1) was more common in YOPSP (33.3% vs 12.2%).
• Longitudinal PSP-RS data indicated a faster worsening of the bulbar score in YOPSP at 6 months.

Conclusions:

• Young-onset PSP is frequently associated with a speech and language variant.
• Age of onset is a potential predictor of PSP phenotypes, with significant clinical and prognostic implications.