Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025
"Crab sign" in an ataxia patient with central nervous system Erdheim Chester disease: a novel neuroimaging finding with review of literature.
СтатьяКлинический случайОбзорНазвание и аннотация: Перевод в очереди
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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
Аннотация
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.
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.