It is an unmet need to estimate survival duration for patients with progressive supranuclear palsy (PSP). The objective of this study was to identify factors associated with the survival duration in patients with PSP. We followed up 23 patients with probable PSP-RS (Richardson syndrome) or PSP-P (parkinsonism) in our PSP center until death from 2011 to 2019. We prospectively and quantitatively rated their downgaze palsy whenever first noticed in our clinic. This was utilized along with the disease duration, motor function, medication use for parkinsonism, sex, age at onset of PSP, comorbid pulmonary and cardiovascular diseases, and the total survival duration from the onset of PSP to death for prediction analysis. A well-fitted linear regression model and a multivariant Cox model were applied to identify predicting factors for total survival duration. All patients had the specific hummingbird sign on brain MRI for PSP when downgaze palsy was documented. We found that the severity of downgaze palsy and the disease duration at the assessment were consistently correlated with the total survival duration in both models. The total survival duration could be further estimated by a formed regression equation. We conclude that severity and time to develop downgaze palsy could help to estimate the total survival duration in patients with probable PSP-RS and PSP-P, the major forms of PSP, which has significant clinical applications in clinical counseling and trial enrollment.
A 56-year-old female with early-stage breast cancer, stage IA grade 1 endometrial cancer, and stage IC grade 1 ovarian cancer developed sudden-onset visual changes and right inferior visual field defect following anastrozole therapy. Examination revealed severe bilateral optic disc swelling and impaired visual acuity. Laboratory work-up was otherwise unremarkable. Anastrozole was discontinued and over the next month, patient had near-complete resolution of swelling in the right eye and improvement in the left eye. This is the only reported case of optic disc swelling following anastrozole therapy.
The categorization of neurodegenerative diseases has evolved based on advances in genetic, molecular and pathological research. In many neurodegenerative diseases, aggregation of a misfolded protein is responsible for the development of pathologic inclusions. When the misfolded protein is tau or synuclein, these diseases are called tauopathies or synucleinopathies, respectively. This article focuses on ophthalmic findings in some of the most common tauopathies and synucleinopathies: Alzheimer's disease, progressive supranuclear palsy, Parkinson's disease, dementia with Lewy bodies and multisystem atrophy.
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