Researchers have discovered a breakthrough in identifying progressive supranuclear palsy (PSP), a deadly neurological disorder, while patients are still alive, moving away from the traditional post-mortem diagnosis.
An article published in Neurology on July 3 reveals a distinct pattern in the spinal fluid of PSP patients using advanced technology capable of analyzing thousands of proteins from a small sample of fluid.
Scientists are hopeful that these protein biomarkers could pave the way for a diagnostic test and targeted treatments to slow down the disease’s progression.
PSP grabbed public attention 25 years ago when Dudley Moore, known for movies like “10” and “Arthur,” disclosed his PSP diagnosis. Often mistaken for Parkinson’s disease, PSP progresses rapidly, unresponsive to Parkinson’s treatments, and typically leads to death within seven years after symptom onset.
Early Diagnosis is Crucial for Effective Treatment
PSP is thought to be triggered by an accumulation of tau proteins that lead to cell degeneration. It falls under frontotemporal dementia (FTD) category affecting cognition, movement, and behavior. Key symptoms include impaired balance resulting in frequent falls backward and difficulties in moving eyes up and down.
Dr. Julio Rojas from UCSF Department of Neurology stresses, “Unlike Alzheimer’s disease, there are no definitive tests like tau scans, blood tests, or MRIs for PSP diagnosis, leading to many cases being undetected.”
“Administering treatment in the early stages of PSP, when it is most likely to be effective, will be crucial once new drugs are available,” Dr. Rojas added.
The inability to accurately diagnose PSP has impeded the progress of new therapies, noted Dr. Adam Boxer, a professor at UCSF specializing in memory and aging.
The study, encompassing 136 participants with an average age of 70, examined PSP-related symptoms from both UCSF and other institutions, alongside confirmed PSP cases from autopsies. Researchers compared biomarkers among these cases, healthy individuals, and other FTD variants.
The analysis revealed lower levels of various proteins in suspected or confirmed PSP participants compared to healthy individuals. Unique protein profiles were observed in autopsy-confirmed PSP cases compared to other FTD variants and living patients.
Individuals with suspected or confirmed PSP exhibited elevated levels of proteins linked to neurodegeneration. The study identified inflammatory proteins associated with disease severity and decreased levels of proteins vital for brain functions, suggesting potential targets for future therapies.
The first author of the study, Amy Wise, highlighted the significance of these findings for future clinical trials. She aims to establish a diagnostic framework using these newly identified proteins that could offer definitive results through a blood test or lumbar puncture.
