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Neuroimaging findings in scleroderma en coup de sabre

From the Departments of Internal Medicine, Rheumatology Unit (Drs. Appenzeller, Sampaio-Barros, Marques-Neto, and Samara) and Neurology (Drs. Montenegro and Cendes), University of Campinas, Brazil; Department of Radiology (Dr. San Juan Dertkigil), State University of Campinas, Brazil; and Department of Neurology and Neurosurgery (Dr. Andermann), Montreal Neurological Institute, McGill University, Montreal, Canada.

Address correspondence and reprint requests to Dr. Fernando Cendes, Departamento de Neurologia, Faculdade de Ciências Médicas/UNICAMP, CEP 13081-970 Campinas SP, Brazil; e-mail: fcendes{at}unicamp.br

Objectives: To describe the neuroimaging and clinical findings in patients with localized scleroderma en coup de sabre (LScs).

Methods: Patients with LScs were evaluated by high-resolution MRI and CT. The authors performed three-dimensional reconstructions of MRI and CT scans to evaluate brain and bone structures.

Results: Nine patients with LScs were evaluated (five women), with ages ranging from 6 to 53 years (mean, 30.7 years). Brain CT showed bone deformities with thinning of the skull under the skin lesions in six patients. MRI scans showed focal atrophy and blurring of the gray-white matter interface localized under the skin lesion in all patients. In three patients it was associated with hyperintense signal on fluid-attenuated inversion recovery (FLAIR) and T2-weighted images. Follow-up MRI showed extension of the brain lesion in one patient; in the remaining patients, the lesion did not progress. Four of the nine patients had partial epilepsy. One had surgery for management of refractory seizures, and pathologic findings indicated a focal inflammatory process.

Conclusion: Localized scleroderma en coup de sabre is associated with focal, and in some progressive, brain lesions underlying the skin atrophy. Epilepsy, when present, is related to these brain lesions. Imaging findings and histopathology indicated that the process, most likely focal inflammatory, may be progressive.

Received July 12, 2003. Accepted in final form January 8, 2004.

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