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Linear scleroderma en coup de sabre presenting with seizures.

Kevin Nguyen¹, Corrine Atty¹, Alexander Ree¹

¹John H. Stroger Cook County Hospital, 1969 Ogden Ave, Chicago, IL 60612, USA.

Radiology Case Reports

|September 21, 2020

Summary

Linear scleroderma en coup de sabre, a rare scalp condition, can present with neurological issues like seizures. This case highlights unique MRI findings, including perfusion and spectroscopic imaging, in diagnosing this rare disorder.

Keywords:

En coup de sabre MR perfusion MRI Morphea Scleroderma Spectroscopy

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Area of Science:

Neurology
Dermatology
Radiology

Background:

Scleroderma is a rare connective tissue disease with localized and systemic forms.
Linear scleroderma en coup de sabre, a subtype, can involve the scalp and rarely presents with neurological symptoms.

Observation:

A 28-year-old female with linear scleroderma en coup de sabre presented with seizures and distinct skin lesions.
Seven years of imaging revealed fluctuating signal changes in the brain.
Advanced MRI techniques, including perfusion and spectroscopy, were used during clinical progression.

Findings:

Perfusion imaging showed reduced cerebral blood volume and increased mean transit time.
Spectroscopic imaging indicated altered metabolite levels.
These findings, alongside characteristic T2W hyperintensities and contrast enhancement on CT/MRI, suggest linear scleroderma en coup de sabre.

Implications:

This case introduces novel spectroscopic and perfusion MRI findings for linear scleroderma en coup de sabre.
These advanced imaging techniques may aid in diagnosing and understanding this rare condition.
The findings contribute to the limited knowledge base of neurological associations with linear scleroderma en coup de sabre.