Severe sub-cortical white matter abnormalities are unusual features in Wilson's disease and are reported to be poorly or not responsive to copper chelating therapy or to be worsened by it. We report on a 12-year-old boy with Wilson's disease and extensive sub-cortical white matter involvement. After five years of copper chelating therapy, an appreciable improvement of these lesions was obtained. The physiopathology of these unusual cerebral white matter abnormalities is discussed.

Background: Central giant cell granuloma (CGCG) and peripheral giant cell granuloma(PGCG) are pathological conditions of the jaws that share the same microscopic features, but differ clinically in terms of their behavior. While the giant cell tumor (GCT) of long bones is a rare benign neoplasm, tend to affect femur and tubular bone, characterized by local aggressiveness, high recurrence rates and metastasis to the lung. Objectives: To evaluate, compare and correlate the expression of TNF-α, IL-6 and VEGF in peripheral and central giant cell granuloma of the jaw and giant cell tumor of long bones. Methods: A total of 60 retrospective formalin-fixed, paraffine-embeded specimens of giant cell lesions of the jaws and long bones, where included in this study. An immunohistochemical staining with TNF-α , IL-6 and VEGF monoclonal antibodies were performed. Results: TNF-α, IL-6 and VEGF were expressedin all lesions. The PGCG comparedto the CGCG and GCT showed significantly increased expression of TNF-α and decreased expression of VEGF by the stromal cells..GCT showed increased expression of VEGF by M NGCs and stromal cells .There is a non significant difference between CGCG and GCT regarding the expression of all three cytokines. Conclusions:The present study confirmed the usefulness TNF-α,IL-6 and VEGF in evaluating osteoclastogenesis. The results of this study proved that the biological activity of TNF-α, IL-6 and VEGF was comparable between the central giant cell granuloma and giant cell tumors, supporting the observations that these two lesions are the same entity and have the same biological behavior.