Evidence map›Paper›PMID 40728558›Full record

ReviewNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2025

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

Farsana Mustafa, Chaithra Rajanna, Archita Makharia, Divyani Garg, Ayush Agarwal, Ajay Garg, Aanchal Kakkar, Shreya Ojha, Nishikant A Damle, Yamini Dharmashaktu and 2 more

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In one paragraph

Review in Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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0cells of the map it votes in
1citing papers in PubMed
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1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

12 authors.

Farsana MustafaDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India.
Chaithra RajannaDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India.
Archita MakhariaDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India.
Divyani GargDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India. divyanig@gmail.com.
Ayush AgarwalDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India. ayushthetaurian@gmail.com.ORCID http://orcid.org/0000-0003-2303-1961
Ajay GargDepartment of Neuroimaging and Interventional Radiology, All India Institute of Medical Sciences, New Delhi, India.
Aanchal KakkarDepartment of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Shreya OjhaDepartment of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Nishikant A DamleDepartment of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.
Yamini DharmashaktuDepartment of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.
Divya M RadhakrishnanDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India.
Achal Kumar SrivastavaDepartment of Neurology, All India Institute of Medical Sciences, Room 705, Cardio Neuro Centre, New Delhi, India.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundErdheim-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.

objectivesTo describe a novel imaging sign in Erdheim Chester Disease.

methodsWe 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.

resultsOur 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.

conclusionThe "crab sign" has the potential to aid early diagnosis and enhance diagnostic yield of Erdheim Chester disease, facilitating earlier therapeutic intervention and better outcomes.

Indexed as

AtaxiaBrainErdheim-Chester DiseaseNeuroimagingFemaleHumansMagnetic Resonance ImagingMiddle AgedCrabECDErdheim chesterHistiocytosis

Identifiers

PMID40728558

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