Abstract #0665

Hyperintense globus pallidus rim sign on 7T MRI is a novel biomarker of neurological Wilson’s disease

Dongning Su¹, Zhijin Zhang¹, Zhe Zhang², Sujun Zheng³, Tingyan Yao⁴, Yi Dong⁵, Wanlin Zhu², Ning Wei², Yue Suo², Xinyao Liu², Huiqing Zhao¹, Zhan Wang¹, Huizi Ma¹, Wei Li¹, Junhong Zhou⁶, Joyce S. T. Lam⁷, Tao Wu¹, Yuan Li⁸, Petr Dusek⁹, A. Jon Stoessl⁷, Xiaoping Wang¹⁰, Jing Jing², and Tao Feng¹

¹Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China, ²Tiantan Neuroimaging Center of Excellence, Beijing Tiantan Hospital, Capital Medical University, Beijing, China, ³Department of Hepatology, Beijing Youan Hospital, Capital Medical University, Beijing, China, ⁴Department of Neurology, Xuanwu Hospital, Capital Medical University, National Clinical Research Center for Geriatric Disorders, Beijing, China, ⁵Senior Department of Hepatology, the Fifth Medical Center of PLA General Hospital, Beijing, China, ⁶Hinda and Arthur Marcus Institute for Aging Research, Hebrew SeniorLife, Roslindale, MA, United States, ⁷Pacific Parkinson’s Research Centre, Djavad Mowafaghian Centre for Brain Health, University of British Columbia, Vancouver, BC, Canada, ⁸MR Research Collaboration Team, Siemens Healthineers, Beijing, China, ⁹Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic, ¹⁰Department of Neurology, Jiading Branch of Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

Synopsis

Keywords: Other Neurodegeneration, Neurodegeneration, Wilson's disease; 7T MRI; biomarker; metal deposition pattern

Motivation: Excessive subcortical metal deposition seen on susceptibility imaging has suggested a characteristic pattern in neurological Wilson’s disease (NWD).

Goal(s): To develop a novel imaging biomarker of NWD using 7T SWI.

Approach: WD patients, monoallelic ATP7B variant carriers, health controls, and patients with comparable clinical or imaging manifestations were recruited for development of a novel biomarker of NWD and exploratory comparative analysis. All underwent 7T SWI with quantitative susceptibility mapping and principal component analysis performed.

Results: The novel biomarker of NWD termed "hyperintense globus pallidus rim sign" showed high diagnostic accuracy. It revealed a special metal deposition pattern in the lenticular nucleus in NWD.

Impact: A novel imaging biomarker of neurological Wilson’s disease (NWD) termed "hyperintense globus pallidus rim sign" could aid the diagnosis and monitoring of NWD.

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