Keith Van Haren

Publication Details

  • Immune response in Leukodystrophies PEDIATRIC NEUROLOGY Eichler, F., Van Haren, K. 2007; 37 (4): 235-244


    Although the genetics and biochemistry of leukodystrophies have been extensively explored, the immune response in these disorders has received relatively little attention. Both the disease course and its response to treatment may be highly dependent on the immune system. In this review, we compare three common leukodystrophies, each with a different immune response: (1) X-linked adrenoleukodystrophy, which demonstrates a severe, lymphocytic inflammatory response; (2) metachromatic leukodystrophy, which yields a histiocytic response; and (3) vanishing white-matter disease, in which no inflammation is typically seen. We highlight the biochemical, pathologic, and clinical differences, while focusing on the immune response in each disease. We also review the response of leukodystrophies to immunomodulatory therapies and interventions such as hematopoietic stem-cell transplantation. Future studies may delineate specific inflammatory markers as possible candidates for therapeutic intervention.

    View details for DOI 10.1016/j.pediatrneurol.2007.06.011

    View details for Web of Science ID 000250295000001

    View details for PubMedID 17903666

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