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. 2024 Sep 27;14(1):22266.
doi: 10.1038/s41598-024-73475-9.

Clinical and neuroimaging features in neurological Wilson's disease with claustrum lesions

Xin-Feng Ma  1 , Ling-Yun Fan  2   3 , Ping Jin  1 , Kang Lin  1 , Guang-An Tong  1 , Gong-Qiang Wang  4   5
Affiliations

Clinical and neuroimaging features in neurological Wilson's disease with claustrum lesions

Xin-Feng Ma et al. Sci Rep. .

Abstract

According to early research, the incidence of claustrum lesions in patients with neurological Wilson's disease (WD) was inconsistent, ranging from 1.8 to 75% on magnetic resonance imaging (MRI). Our study aims to explore the incidence, clinical presentation features, iconography features, and possible pathological mechanisms in WD patients with claustrum lesions on magnetic resonance imaging (MRI), to characterize the clinical, and brain imaging findings and possible pathological mechanisms in the patients with WD. Retrospective cases meeting the inclusion criteria were studied for analyzing MRI characteristics and associated physicochemical examination data in neurological WD patients with claustrum lesions. 443 (66.3%) with brain MRI abnormalities were screened from 668 WD patients. The three (0.7%) patients with the claustrum lesions characteristics on MRI images were: (a) "bright claustrum" in T2-weighted and FLAIR sequences, (b) bilateral symmetrical, (c) non-isolated lesions, (d) occurred only in severe neurological manifestations. The claustrum lesions are not common in neurological WD and mainly appear in cases with severe neurological symptoms. On MRI, the "bright claustrum" signs may be a radiographic marker of neuroinflammation, the features of the lesions showed bilateral symmetry, and hyperintensity signals on T2-weighted, FLAIR, and DWI.

Keywords: Brain MRI; Claustrum; Copper; Neuroinflammation; Wilson’s disease.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
The brain lesions on MRI axial scanning T1-weighted, T2-weighted, FLAIR, DWI sequence in three WD patients. In claustrum, symmetrical bilateral hypointensity on T1-weighted and hyperintensity on T2-weighted, FLAIR, and DWI images (red arrow). In putamen, incomplete symmetrical bilateral mix intensity on T1-weighted, T2-weighted, FLAIR, and DWI images (green arrow). In brainstem, incomplete symmetrical hyperintensity on T2-weighted images (yellow arrow).
Fig. 2
Fig. 2
Disease species distribution of the claustrum lesions on MRI from reported literature cases (%).
See this image and copyright information in PMC

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