Concomitant progressive supranuclear palsy and multiple system atrophy: A rare case report of tauopathy and synucleinopathy interface

IF 3 3区医学 Q2 CLINICAL NEUROLOGY

Journal of Neuroradiology Pub Date : 2025-04-28 DOI:10.1016/j.neurad.2025.101347

Nitesh Shekhrajka, Márcio Luís Duarte, Leonardo Furtado Freitas

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Abstract

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are neurodegenerative disorders characterized by parkinsonian symptoms. PSP typically presents with supranuclear gaze palsy, postural instability, and subcortical dementia, while dysautonomia and cerebellar signs are hallmark features of MSA. We report the case of a 77-year-old male patient with a four-year history of progressive parkinsonian symptoms that have shown poor response to treatment. His clinical manifestations included bradykinesia, rigidity, swallowing difficulties, urinary frequency, nocturia, downward gaze palsy, hypometric saccades, and finger-nose dysmetria. Examination revealed increased tone in both upper extremities, more pronounced on the right side, along with bradykinesia, slow hand movements, and bilateral decrement in finger tapping. Brain magnetic resonance imaging (MRI) revealed mixed MRI findings consistent with atypical parkinsonian syndrome, showing signs of MSA-C with pontocerebellar atrophy and the "hot cross bun" sign, as well as PSP with midbrain atrophy (hummingbird sign) and tegmental hypersignal.

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伴进行性核上性麻痹及多系统萎缩：牛头病及突触核蛋白病交界面一例罕见报告

进行性核上性麻痹（PSP）和多系统萎缩（MSA）是以帕金森症状为特征的神经退行性疾病。PSP通常表现为核上凝视性麻痹、体位不稳定和皮质下痴呆，而自主神经异常和小脑体征是MSA的标志性特征。我们报告的情况下，77岁的男性患者与四年的进行性帕金森症状的历史，已显示不良反应的治疗。临床表现为运动迟缓、强直、吞咽困难、尿频、夜尿、向下凝视性麻痹、低血压性扫视、手指-鼻子发育障碍。检查显示双上肢张力增加，右侧更明显，同时运动迟缓，手部运动缓慢，双侧手指敲击减少。脑磁共振成像（MRI）显示与非典型帕金森综合征一致的混合MRI表现，表现为MSA-C伴桥小脑萎缩和“热十字面包”征，以及PSP伴中脑萎缩（蜂鸟征）和被盖高信号。

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来源期刊

Journal of Neuroradiology 医学-核医学

CiteScore

6.10

自引率

5.70%

发文量

142

审稿时长

6-12 weeks

期刊介绍： The Journal of Neuroradiology is a peer-reviewed journal, publishing worldwide clinical and basic research in the field of diagnostic and Interventional neuroradiology, translational and molecular neuroimaging, and artificial intelligence in neuroradiology. The Journal of Neuroradiology considers for publication articles, reviews, technical notes and letters to the editors (correspondence section), provided that the methodology and scientific content are of high quality, and that the results will have substantial clinical impact and/or physiological importance.