Moser and colleagues, in a focused and important editorial critique Article, review their extensive experience with ALD and review the work of Takano et alArticle describing a mutational analysis of 29 unrelated Japanese patients with X-linked ALD. The frequency and types of mutations in the Japanese population are similar to other national groups. The detailed data provided by Takano et al and those in other ethnic groups provide the opportunity to determine whether the nature of the mutations determines the phenotype. The role of very long–chain fatty acids in the pathogenesis of X-linked ALD is discussed. The pathogenesis of the brain inflammatory response is also reviewed and placed into clinical context. Moser et al and Takano et al provide a synopsis of present molecular-clinical relationships.
This Month in the Archives of Neurology. Arch Neurol. 1999;56(3):269. doi:10.1001/archneur.56.3.269