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Background <p>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.</p> Objectives <p>To describe a novel imaging sign in Erdheim Chester Disease.</p> Methods <p>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.</p> Results <p>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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

  • 发表日期：

    2025年7月29日

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  • 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

摘要

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.