"Crab sign" in an ataxia patient with central nervous system Erdheim Chester disease: a novel neuroimaging finding with review of literature.

IF 2.4 4区医学 Q2 CLINICAL NEUROLOGY

Neurological Sciences Pub Date : 2025-07-29 DOI:10.1007/s10072-025-08381-7

Farsana Mustafa, Chaithra Rajanna, Archita Makharia, Divyani Garg, Ayush Agarwal, Ajay Garg, Aanchal Kakkar, Shreya Ojha, Nishikant A Damle, Yamini Dharmashaktu, Divya M Radhakrishnan, Achal Kumar Srivastava

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引用次数: 0

Abstract

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.

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中枢神经系统Erdheim Chester病共济失调患者的“螃蟹征”：一种新的神经影像学发现并文献复习。

背景：中枢神经系统Erdheim-Chester病（ECD）是一种肿瘤性非朗格汉斯细胞组织细胞增多症，可表现为多种症状。轴内受累通常导致痉挛性共济失调综合征、眼麻痹和尿崩症，而轴外受累表现为厚性脑膜炎。目的：描述Chester病的一种新的影像学征象。方法：我们通过病史、检查、血清学、放射学和组织病理学调查，详细评估了一位55岁的黄松病和进行性痉挛性共济失调综合征的女性。结果：根据组织病理学评估，我们的患者被诊断为Chester病。她有一个新的神经影像学发现“螃蟹征”在她的MRI脑轴位T2-FLAIR切片。结论：“蟹征”有助于早期诊断，提高Chester病的诊断率，有利于早期干预治疗，获得较好的预后。

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

Neurological Sciences 医学-临床神经学

CiteScore

6.10

自引率

3.00%

发文量

743

审稿时长

4 months

期刊介绍： Neurological Sciences is intended to provide a medium for the communication of results and ideas in the field of neuroscience. The journal welcomes contributions in both the basic and clinical aspects of the neurosciences. The official language of the journal is English. Reports are published in the form of original articles, short communications, editorials, reviews and letters to the editor. Original articles present the results of experimental or clinical studies in the neurosciences, while short communications are succinct reports permitting the rapid publication of novel results. Original contributions may be submitted for the special sections History of Neurology, Health Care and Neurological Digressions - a forum for cultural topics related to the neurosciences. The journal also publishes correspondence book reviews, meeting reports and announcements.