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Autoimmune Dis. 2012; 2012: 719685.
Published online Jan 27, 2012. doi:  10.1155/2012/719685
PMCID: PMC3272788
Neurologic Involvement in Scleroderma en Coup de Sabre
Tiago Nardi Amaral, 1 João Francisco Marques Neto, 1 Aline Tamires Lapa, 2 Fernando Augusto Peres, 3 Caio Rodrigues Guirau, 3 and Simone Appenzeller 1, 3 *
1Rheumatology Division, Faculty of Medical Science, State University of Campinas, 13083-970 Campinas, SP, Brazil
2Postgraduate Program in Child and Adolescent Health, Faculty of Medical Science, State University of Campinas, 13083-970 Campinas (UNICAMP), Brazil
3Rheumatology Lab, Faculty of Medical Science, State University of Campinas, 13083-970 Campinas, SP, Brazil
*Simone Appenzeller: appenzellersimone/at/yahoo.com
Academic Editor: Jozélio Freire de Carvalho
Received October 31, 2011; Accepted December 4, 2011.
Abstract
Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs.
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