Real-world evaluation of Armstrong's criteria in corticobasal degeneration: Phenotypic overlap and diagnostic challenges

IF 3.4 3区医学 Q2 CLINICAL NEUROLOGY

Parkinsonism & related disorders Pub Date : 2026-04-01 Epub Date: 2026-02-09 DOI:10.1016/j.parkreldis.2026.108229

Ryuta Morihara , Emi Nomura , Yosuke Osakada, Taijun Yunoki, Mami Takemoto, Toru Yamashita, Hiroyuki Ishiura

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引用次数: 0

Abstract

Background

Corticobasal degeneration (CBD) is a four-repeat tauopathy with heterogeneous clinical manifestations. Armstrong's criteria involve a two-step diagnostic approach: first, classifying patients into five clinical phenotypes—probable/possible corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), non-fluent/agrammatic variant primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS); second, determining whether they meet the clinical research criteria for probable CBD (cr-CBD) or the clinical criteria for possible CBD (p-CBD), which are distinct from the initial CBS classifications.

Objective

To investigate how real-world patients with suspected CBD fulfill Armstrong's clinical phenotypes and diagnostic criteria, and to compare clinical and imaging features between the Alzheimer's disease (AD) group and the non-AD group defined by CSF amyloid biomarkers.

Methods

We retrospectively reviewed 137 patients undergoing differential diagnosis for CBS, frontotemporal dementia, primary progressive aphasia, or PSPS. Of these, 78 met the criteria for cr-CBD (n = 36) or p-CBD (n = 42). CSF was examined in 32 patients, and based on the CSF Aβ42/40 ratio, patients were classified into an AD-group (AD-CBS; n = 6) and a non-AD group (n = 26).

Results

Among patients classified as cr-CBD or p-CBD, 79% fulfilled two or more clinical phenotypes, with FBS and PSPS most commonly. Compared with the AD group, the non-AD group showed more parkinsonian features and frontal hypoperfusion on [¹²³I]-IMP SPECT.

Conclusion

Armstrong's criteria captured a spectrum of overlapping clinical features. While helpful in clinical phenotyping, further validation with biomarkers is essential to distinguish CBD from AD and related disorders. Prospective studies with pathological confirmation are warranted.

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现实世界中对Armstrong标准在皮质基底变性中的评价：表型重叠和诊断挑战

背景皮质基底变性（CBD）是一种具有异质性临床表现的四重复牛头病。Armstrong的标准包括两步诊断方法：首先，将患者分为五种临床表型——可能/可能的皮质基底综合征（CBS）、额部行为空间综合征（FBS）、非流利/语法变异型原发性进行性失语（naPPA）和进行性核上性麻痹综合征（PSPS）；其次，确定它们是否符合可能CBD的临床研究标准（cr-CBD）或可能CBD的临床标准（p-CBD），这与最初的CBS分类不同。目的探讨现实世界中疑似CBD患者如何满足Armstrong的临床表型和诊断标准，并比较脑脊液淀粉样蛋白生物标志物定义的阿尔茨海默病（AD）组和非AD组的临床和影像学特征。方法回顾性分析137例接受CBS、额颞叶痴呆、原发性进行性失语或PSPS鉴别诊断的患者。其中78例符合cr-CBD （n = 36）或p-CBD （n = 42）的标准。对32例患者进行脑脊液检查，根据脑脊液a β42/40比值将患者分为ad组（AD-CBS, n = 6）和非ad组（n = 26）。结果在分类为cr-CBD或p-CBD的患者中，79%的患者满足两种或两种以上的临床表型，其中以FBS和PSPS最为常见。与AD组相比，非AD组在[123I]-IMP SPECT上表现出更多的帕金森特征和额叶灌注不足。结论armstrong的标准涵盖了一系列重叠的临床特征。虽然有助于临床表型，但生物标志物的进一步验证对于区分CBD与AD和相关疾病至关重要。有病理证实的前瞻性研究是必要的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Parkinsonism & related disorders 医学-临床神经学

CiteScore

6.20

自引率

4.90%

发文量

292

审稿时长

39 days

期刊介绍： Parkinsonism & Related Disorders publishes the results of basic and clinical research contributing to the understanding, diagnosis and treatment of all neurodegenerative syndromes in which Parkinsonism, Essential Tremor or related movement disorders may be a feature. Regular features will include: Review Articles, Point of View articles, Full-length Articles, Short Communications, Case Reports and Letter to the Editor.