Atypical presentation of progressive supranuclear palsy

Area of Science:

• Neurology
• Neuroscience
• Pathology

Background:

• Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease.
• Classic diagnostic criteria for PSP include ophthalmoplegia, which may not always be present.

Observation:

• This study reports four pathologically confirmed cases of PSP.
• Two patients presented with severe dementia, and two with parkinsonism.
• None of the patients exhibited the classic ophthalmoplegia.

Findings:

• Retrospective analysis revealed key diagnostic clues: early gait disturbance, eyelid opening apraxia, absence of tremor, poor response to levodopa-carbidopa, and severe rigidity with neck extension.
• These findings suggest PSP's clinical presentation is more diverse than previously assumed.

Implications:

• The diagnosis of PSP can be missed if clinicians rely solely on rigid adherence to classic criteria.
• Awareness of atypical presentations is essential for timely and accurate PSP diagnosis.
• Further research into PSP's varied clinical manifestations is warranted.