Neurologic Involvement in Scleroderma en Coup de Sabre

Autor(es)

Amaral, Tiago Nardi ; Marques Neto, Joao Francisco ; Lapa, Aline Tamires ; Peres, Fernando Augusto ; Guirau, Caio Rodrigues ; Appenzeller, Simone

• Tipo de documento: Artigo Científico
• Fonte: AUTOIMMUNE DISEASES; v. 2012, p. 7-pg., 2012-01-01.

Resumo

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. (AU)

• Processo FAPESP: 08/02917-0 - Determinação de marcadores séricos e do líquor associados a alterações estruturais e funcionais do sistema nervoso central no lúpus eritematoso sistêmico
• Beneficiário: Simone Appenzeller
• Modalidade de apoio: Auxílio à Pesquisa - Jovens Pesquisadores
• Processo FAPESP: 10/13639-1 - Análise das alterações nas estruturas de substância branca e cinzenta através da ressonância magnética no Lúpus Eritematoso Sistêmico juvenil
• Beneficiário: Aline Tamires Lapa
• Modalidade de apoio: Bolsas no Brasil - Mestrado
• Processo FAPESP: 09/06049-6 - Determinação de marcadores séricos e do líquor associados a alterações estruturais e funcionais do sistema nervoso central no Lúpus eritematoso sistêmico
• Beneficiário: Simone Appenzeller
• Modalidade de apoio: Bolsas no Brasil - Jovens Pesquisadores