Presently, there is increasing interest in rare PSP (progressive supranuclear palsy) variants, including PSP-PGF (PSP-progressive gait freezing), PSP-PI (PSP-postural instability), PSP-OM (PSP-ocular motor dysfunction), PSP-C (PSP-predominant cerebellar ataxia), PSP-CBS (PSP-corticobasal syndrome), PSP-SL (PSP-speech/language disorders), and PSP-PLS (PSP-primary lateral sclerosis). Diagnosis of these subtypes is usually based on clinical symptoms, thus thorough examination with anamnesis remains a major challenge for clinicians. The individual phenotypes often show great similarity to various neurodegenerative diseases and other genetic, autoimmune, or infectious disorders, manifesting as PSP-mimicking syndromes. At the current stage of knowledge, it is not possible to isolate a specific marker to make a definite ante-mortem diagnosis. The purpose of this review is to discuss recent developments in rare PSP phenotypes and PSP-like syndromes.
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http://dx.doi.org/10.3389/fnagi.2022.804385 | DOI Listing |
Parkinsonism Relat Disord
January 2025
Department of Neurology, Gifu University Graduate School of Medicine, Gifu, Japan. Electronic address:
Serum anti-IgLON5 antibodies, which were tested in 223 patients meeting the diagnostic criteria for progressive supranuclear palsy/corticobasal syndrome (PSP/CBS), were negative in all patients. Our study suggests that the frequency of anti-IgLON5 disease is extremely rare in patients with typical presentation of PSP/CBS.
View Article and Find Full Text PDFAJNR Am J Neuroradiol
January 2025
From the Department of Neurology, the First Hospital of Jilin University, Changchun, Jilin Province, China.
Background And Purpose: Differentiating Parkinson's Disease (PD) from Atypical Parkinsonism Syndrome (APS), including Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP), is challenging, and there is no gold standard. Integrating quantitative susceptibility mapping (QSM) and morphometry can help differentiate PD from APS and improve the internal diagnosis of APS.
Materials And Methods: In this retrospective study, we enrolled 55 patients with PD, 17 with MSA-parkinsonian type (MSA-P), 15 with MSA-cerebellar type (MSA-C), and 14 with PSP.
Gac Med Mex
January 2025
Laboratorio de Reprogramación Celular y Enfermedades Crónico-Degenerativas, Department of Physiology, Universidad Nacional Autónoma de México, Mexico City, Mexico.
Progressive supranuclear palsy (PSP) is a rare, atypical parkinsonism, characterized by the presence of intracerebral tau protein aggregates and determined by a wide spectrum of clinical features. The definitive diagnosis is postmortem and is identified through the presence of neuronal death, gliosis, and aggregates of the tau protein presented in the form of neurofibrillary tangles (MNF) with a globose appearance in regions such as the subthalamic nucleus, the substantia nigra, and the globus pallidus The findings in ancillary imaging studies, as well as fluids biomarkers, are not sufficient to support diagnosis of PSP but are used to rule out similar pathologies because there are still no specific or validated biomarkers for this disease. The current treatment of PSP is focused on reducing symptoms, although emerging therapies seek to counteract its pathophysiological mechanisms.
View Article and Find Full Text PDFBrain Behav
January 2025
Department of Neurology, Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.
Background: While automated methods for differential diagnosis of parkinsonian syndromes based on MRI imaging have been introduced, their implementation in clinical practice still underlies considerable challenges.
Objective: To assess whether the performance of classifiers based on imaging derived biomarkers is improved with the addition of basic clinical information and to provide a practical solution to address the insecurity of classification results due to the uncertain clinical diagnosis they are based on.
Methods: Retro- and prospectively collected data from multimodal MRI and standardized clinical datasets of 229 patients with PD (n = 167), PSP (n = 44), or MSA (n = 18) underwent multinomial classification in a benchmark study comparing the performance of nine machine learning methods.
Parkinsonism Relat Disord
January 2025
Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan. Electronic address:
Introduction: Recent studies by us and others have unveiled a frequent coexistence of idiopathic normal pressure hydrocephalus (iNPH) with neurodegenerative movement disorders, including progressive supranuclear palsy (PSP). This study aims to explore the clinical and radiological characteristics of patients with iNPH who also had comorbid with PSP, referred to as iNPHc + PSP.
Methods: We retrospectively analyzed patients with iNPH admitted to our department between 2009 and 2024.
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