Progressive Supranuclear Palsy Diagnosis and Treatment

IF 2 4区医学 Q1 Medicine Current Treatment Options in Neurology Pub Date : 2024-01-18 DOI:10.1007/s11940-024-00784-9

Lauryn Currens, Alexander Pantelyat

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Abstract

Purpose of review

This review describes the current approaches to the diagnosis and management of progressive supranuclear palsy (PSP)

Recent findings

PSP is an atypical parkinsonian disorder associated with the accumulation of abnormal 4-repeat tau protein in the brain. Initially, the recognized clinical phenotype included a progressive disorder with vertical supranuclear gaze palsy and prominent postural instability leading to early falls. However, the current PSP diagnostic criteria recognize a broader range of clinical PSP presentations and define eight clinical PSP variants according to the levels of diagnostic certainty. While definite PSP remains a neuropathological diagnosis, imaging modalities including brain magnetic resonance imaging (MRI), dopamine transporter (DAT), and tau positron emission tomography (PET) scans may aid in the diagnosis. In the future, new tau PET ligands and CSF and genetic biomarkers may improve diagnostic accuracy. There is no disease-modifying therapy currently available for PSP. However, there are many pharmacological and non-pharmacological treatment options for symptomatic management. Because PSP is a multisystem disease, optimal management requires a coordinated multidisciplinary team approach.

Summary

PSP is a fatal multisystem disease that can be challenging to diagnose and manage. However, improved clinical diagnostic criteria, emerging biomarkers, and availability of useful therapeutic approaches provide cause for optimism.

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进行性核上性麻痹的诊断和治疗

综述目的本综述介绍了目前诊断和治疗进行性核上性麻痹（PSP）的方法。最初，公认的临床表型包括伴有垂直核上凝视麻痹和突出的姿势不稳导致早期跌倒的进行性障碍。然而，目前的 PSP 诊断标准认识到 PSP 的临床表现范围更广，并根据诊断的确定程度定义了八种临床 PSP 变体。虽然明确的 PSP 仍属于神经病理学诊断，但包括脑磁共振成像（MRI）、多巴胺转运体（DAT）和 tau 正电子发射断层扫描（PET）在内的成像模式可能有助于诊断。未来，新的 tau PET 配体以及 CSF 和基因生物标记物可能会提高诊断的准确性。目前还没有针对 PSP 的疾病改变疗法。不过，有许多药物和非药物治疗方法可用于对症治疗。由于 PSP 是一种多系统疾病，因此最佳治疗需要多学科团队的协调配合。然而，临床诊断标准的改进、新出现的生物标记物以及有用的治疗方法的出现，都让人有理由感到乐观。

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来源期刊

Current Treatment Options in Neurology CLINICAL NEUROLOGY-

CiteScore

4.00

自引率

0.00%

发文量

审稿时长

6-12 weeks

期刊介绍： This journal aims to review the most important, recently published treatment option advances in the field of neurology. By presenting clear, insightful, balanced contributions by international experts, the journal intends to facilitate worldwide approaches to the treatment of neurologic conditions. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas, such as epilepsy, headache, neurologic ophthalmology and otology, neuromuscular disorders, psychiatric manifestations of neurologic disease, and sleep disorders. Section Editors select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. We also provide commentaries from well-known neurologists, and an international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research.