Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder with no effective treatment. Dopaminergic agents occasionally produce transient symptomatic improvement. The authors report the results of pramipexole treatment (4.5 mg daily) in six patients with PSP (average disease duration, 4.4 years). Patients were treated for 2 months. Patients were evaluated with the Unified Parkinson's Disease Rating Scale, Hoehn and Yahr stage, and Schwab and England Activities of Daily Living Scale at baseline and 2 months. Pramipexole was not efficacious for the symptoms of PSP.
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http://dx.doi.org/10.1212/wnl.52.4.873 | DOI Listing |
J Biol Chem
January 2025
UK Dementia Research Institute at the University of Cambridge, Department of Clinical Neurosciences, Hills Road, Cambridge, CB2 0AH, United Kingdom. Electronic address:
The assembly of tau into filaments defines tauopathies, a group of neurodegenerative diseases including Alzheimer's disease (AD), Pick's disease (PiD), corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). The seeded aggregation of tau has been modelled in cell culture using pro-aggregant modifications such as truncation of N- and C-termini and point-mutations within the microtubule-binding repeat domain. This limits the applicability of research findings to sporadic disease, where aggregates contain wild-type, full-length tau.
View Article and Find Full Text PDFAnn Neurol
January 2025
Neuroscience Research Center, Department of Medical and Surgical Sciences, University "Magna Graecia", Catanzaro, Italy.
Objective: Progressive Supranuclear Palsy (PSP) is a severe neurodegenerative disease characterized by tangles of hyperphosphorylated tau protein and tufted astrocytes. Developing treatments for PSP is challenging due to the lack of disease models reproducing its key pathological features. This study aimed to model sporadic PSP-Richardson's syndrome (PSP-RS) using multi-donor midbrain organoids (MOs).
View Article and Find Full Text PDFMov Disord Clin Pract
January 2025
Department of Neurology, Hannover Medical School, Hannover, Germany.
Background: Patients with Progressive Supranuclear Palsy (PSP) suffer from several neuropsychological impairments. These mainly affect the frontal lobe and subcortical brain structures. However, a scale for the assessment of cognitive and neuropsychiatric disability in PSP is still missing.
View Article and Find Full Text PDFCo-existing neuropathological comorbidities have been repeatedly reported to be extremely common in subjects dying with dementia due to Alzheimer disease. As these are likely to be additive to cognitive impairment, and may not be affected by molecularly-specific AD therapeutics, they may cause significant inter-individual response heterogeneity amongst subjects in AD clinical trials. Furthermore, while originally noted for the oldest old, recent reports have now documented high neuropathological comorbidity prevalences in younger old AD subjects, who are more likely to be included in clinical trials.
View Article and Find Full Text PDFLancet Neurol
February 2025
Department of Clinical Neurological Sciences, University of Western Ontario, London, ON, Canada; Department of Cognitive Neurology, St Joseph's Health Care London, London, ON, Canada. Electronic address:
Background: No treatments exist for apathy in people with frontotemporal dementia. Previously, in a randomised double-blind, placebo-controlled, dose-finding study, intranasal oxytocin administration in people with frontotemporal dementia improved apathy ratings on the Neuropsychiatric Inventory over 1 week and, in a randomised, double-blind, placebo-controlled, crossover study, a single dose of 72 IU oxytocin increased blood-oxygen-level-dependent signal in limbic brain regions. We aimed to determine whether longer treatment with oxytocin improves apathy in people with frontotemporal dementia.
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