Cerebellar involvement in progressive supranuclear palsy: A clinicopathological study

Area of Science:

• Neurology
• Pathology
• Neuroscience

Background:

• Progressive supranuclear palsy (PSP) is known for clinical heterogeneity, with subtypes like Richardson's syndrome (RS) and PSP-Parkinsonism (PSP-P).
• Previous studies have discussed the clinical variations within PSP, but clinicopathological correlations require further investigation.

Purpose of the Study:

• To investigate the clinicopathological heterogeneity of progressive supranuclear palsy (PSP).
• To explore the spectrum of clinical manifestations and their correlation with pathological findings in Japanese PSP patients.

Main Methods:

• Retrospective analysis of 22 consecutive Japanese patients with pathologically confirmed PSP.
• Evaluation of clinical features (early and throughout disease course) and pathological markers (neuronal loss, gliosis, tau pathology).

Main Results:

• Patients were categorized into RS (10), PSP-Parkinsonism (8), and unclassifiable PSP (4) based on clinical features.
• Four patients presented with cerebellar ataxia or cerebral cortical signs, with three showing ataxia as the primary initial symptom.
• Significantly more frequent tau-positive inclusion bodies in Purkinje cells were observed in patients with cerebellar ataxia.
• Patients with cerebellar ataxia showed increased neuronal loss, gliosis, and coiled bodies in the cerebellar dentate nucleus.

Conclusions:

• This study confirms a wide spectrum of clinicopathological manifestations in PSP across different ethnic origins.
• Progressive supranuclear palsy (PSP) can manifest with cerebellar ataxia, highlighting the need to consider this presentation in diagnosis.