Treating inflammation in childhood neurodegenerative disorders

Abstract

Inflammation of the central nervous system is a prominent feature in many childhood neurodegenerative conditions, with various studies demonstrating the upregulation of the innate and adaptive immune system. Recent evidence also suggests that this inflammatory process can contribute to further neurodegeneration. Furthermore, immunosuppression in mouse models of a few lysosomal storage disorders has demonstrated that attenuation of this immune response can influence the clinical and neuropathological progression. However, there are significant challenges before this finding translates to patient care. Treating inflammation in neurodegenerative conditions requires the identification of the time point when inflammation becomes pathogenic, after which the safest therapeutic strategies are required to target the various components and confounders of inflammation. Nevertheless, as the progress made towards effective gene-, cellular-, and enzyme-based therapy in most of these disorders has been disappointing, treating pathogenic inflammation may offer the clinician another therapeutic strategy in managing these devastating disorders.