Evidence map›Paper›PMID 41154122›Full record

ArticleBrain sciences2025

Type B Fibers: A Novel Ultrastructural Biomarker for Cognitive Impairment in Neuronal Intranuclear Inclusion Disease.

Binbin Zhou, Shaoping Zhong, Yangye Lian, Jingzhen Liang, Luyao Huang, Jing Ding, Xin Wang

Abstract read
In one paragraph

Article in Brain sciences, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Binbin ZhouDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.
Shaoping ZhongDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.
Yangye LianDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.
Jingzhen LiangDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.ORCID 0009-0005-2431-8739
Luyao HuangDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.
Jing DingDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.
Xin WangDepartment of Neurology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, China.

Funding

China Postdoctoral Science Foundation 2023M730676China Postdoctoral Science Foundation 2023M730689National Natural Science Foundation of China 32171216National Natural Science Foundation of China 82271499National Natural Science Foundation of China 82301603National Science and Technology Innovation 2030 Major Projects of China STI2030-Major Projects 2022ZD0212600
6 · The paper itself

Abstract

BACKGROUND/

objectiveNeuronal intranuclear inclusion disease (NIID) is characterized by widespread deposition of eosinophilic intranuclear inclusions in multiple systems throughout the body. The aim of this study was to investigate the clinical and phenotypic features of NIID, with a focus on the potential association between the morphological features of fibrils formed by polyG (polyglycine) proteins and cognitive dysfunction in patients with NIID.

methodsThis study involved a retrospective collection of clinical data from 15 patients with NIID harboring GGC repeat expansions in the

resultsThe majority of patients in this cohort with NIID were female (73.3%), with an average age of onset of 61.06 ± 7.67 years. The core clinical manifestations were cognitive decline (93.3%) and autonomic dysfunction (93.3%). Cranial MRI revealed characteristic DWI "ribbon sign" in 93.3% of patients, accompanied by lateral ventricle enlargement (93.3%), cerebellar atrophy (86.6%), and high T2-FLAIR signal in the corpus callosum (93.3%). All patients were found to have pathogenic GGC amplification in the

conclusionsThe presence of two different forms of fibrils, type A and type B, in the inclusion bodies of NIID patients, and the poorer cognitive level of NIID patients in the type B group than that of type A suggest that type B fibrils can be used as a novel pathological marker of severe cognitive impairment and poor prognosis in NIID.

Indexed as

cognitive impairmentGGC repeat expansionMMSE scoreneuronal intranuclear inclusion diseaseultrastructural pathology

Identifiers

PMID41154122
PMCPMC12563326

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