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Neuroimaging findings in scleroderma en coup de sabre
S Appenzeller1, M A Montenegro, S San Juan Dertkigil
1Department of Internal Medicine, Rheumatology Unit, University of Campinas, SP, Brazil.
Neurology
|May 12, 2004
Summary
Localized scleroderma en coup de sabre (LScs) is linked to brain abnormalities. These focal brain lesions, potentially inflammatory and progressive, can cause epilepsy in affected individuals.
Area of Science:
- Neurology
- Dermatology
- Radiology
Background:
- Localized scleroderma en coup de sabre (LScs) is a rare condition characterized by facial morphea.
- Understanding its neurological manifestations is crucial for comprehensive patient care.
Observation:
- High-resolution MRI and CT scans were used to evaluate nine patients with LScs.
- Three-dimensional reconstructions assessed brain and bone structures in relation to skin lesions.
Findings:
- All patients exhibited focal brain lesions, including atrophy and gray-white matter blurring, beneath skin lesions.
- Six patients showed skull deformities on CT scans.
- Epilepsy was present in four patients, with one undergoing surgery for refractory seizures, revealing a focal inflammatory process.
Implications:
- LScs is associated with focal, and sometimes progressive, brain lesions.
- Epilepsy in LScs patients is directly related to these underlying brain abnormalities.
- Imaging and histopathology suggest a potentially progressive, focal inflammatory etiology.