一名患有中枢神经系统厄尔海姆-切斯特病的共济失调患者的“蟹征”：一项新的神经影像学发现并文献复习
"Crab sign" in an ataxia patient with central nervous system Erdheim Chester disease: a novel neuroimaging finding with review of literature.
作者信息
Mustafa Farsana, Rajanna Chaithra, Makharia Archita, Garg Divyani, Agarwal Ayush, Garg Ajay, Kakkar Aanchal, Ojha Shreya, Damle Nishikant A, Dharmashaktu Yamini, Radhakrishnan Divya M, Srivastava Achal Kumar
机构信息
Department of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India.
Department of Neuroimaging and Interventional Radiology, All India Institute of Medical Sciences, New Delhi, India.
出版信息
Neurol Sci. 2025 Jul 29. doi: 10.1007/s10072-025-08381-7.
BACKGROUND
Erdheim-Chester disease (ECD) of the central nervous system is a neoplastic, non-Langerhans cell histiocytosis which can present with a multitude of symptoms. Intra-axial involvement commonly leads to a spastic ataxic syndrome, ophthalmoplegia, and diabetes insipidus while extra-axial involvement manifests as pachymeningitis.
OBJECTIVES
To describe a novel imaging sign in Erdheim Chester Disease.
METHODS
We evaluated in detail a 55-year old lady with xanthelesmas and a progressive spastic ataxic syndrome over a 6 month period by history, examination, serological, radiological and histopathological investigations.
RESULTS
Our patient was diagnosed with Erdheim Chester disease based on histopathological evaluation. She had a novel neuroimaging finding of "crab sign" in the axial T2-FLAIR sections of her MRI brain.
CONCLUSION
The "crab sign" has the potential to aid early diagnosis and enhance diagnostic yield of Erdheim Chester disease, facilitating earlier therapeutic intervention and better outcomes.
背景
中枢神经系统的厄尔海姆-切斯特病（ECD）是一种肿瘤性非朗格汉斯细胞组织细胞增多症，可出现多种症状。轴内受累通常导致痉挛性共济失调综合征、眼肌麻痹和尿崩症，而轴外受累表现为硬脑膜炎。
目的
描述厄尔海姆-切斯特病一种新的影像学表现。
方法
我们通过病史、检查、血清学、影像学和组织病理学检查，对一名55岁患有睑黄瘤且在6个月内出现进行性痉挛性共济失调综合征的女性进行了详细评估。
结果
根据组织病理学评估，我们的患者被诊断为厄尔海姆-切斯特病。她在脑部MRI的轴位T2-FLAIR序列上有一个新的神经影像学表现“螃蟹征”。
结论
“螃蟹征”有可能有助于厄尔海姆-切斯特病的早期诊断并提高诊断率，促进早期治疗干预并取得更好的治疗效果。
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