Neurologic Involvement in Scleroderma en Coup de Sabre

Author(s)

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

• Document type: Journal article
• Source: AUTOIMMUNE DISEASES; v. 2012, p. 7-pg., 2012-01-01.

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. (AU)

• FAPESP's process: 08/02917-0 - Blood and cerebrospinal fluid biomarkers associated with structural and functional central nervous system abnormalities in systemic lupus erythematosus
• Grantee: Simone Appenzeller
• Support Opportunities: Research Grants - Young Investigators Grants
• FAPESP's process: 10/13639-1 - Analysis of structural changes in gray and white matter by magnetic resonance in juvenile systemic lupus erythematosus.
• Grantee: Aline Tamires Lapa
• Support Opportunities: Scholarships in Brazil - Master
• FAPESP's process: 09/06049-6 - Blood and cerebrospinal fluid biomarkers associated with structural and functional central nervous system abnormalities in Systemic lupus erythematosus
• Grantee: Simone Appenzeller
• Support Opportunities: Scholarships in Brazil - Young Researchers