Snapshot A 9-year-old boy presents with confusion and decreased school performance. Spastic gait, dysarthria, dysphagia, and visual loss soon develop. On MRI, there is massive demyelination of the white matter in the posterior areas of the hemispheres. Introduction An X-linked recessive disorder that leads to a peroxisomal enzyme deficiency login to view 2 more bullets Spectrum of phenotype login to view 3 more bullets Affects young males usually begining at age 7-8 Characterized by login to view 2 more bullets Presentation Symptoms login to view 5 more bullets Evaluation VLCFA panel login to view 3 more bullets Plasma ACTH login to view 2 more bullets Neuroimaging login to view 3 more bullets Differential Metachromatic leukodystrophy Krabbe disease Tay-Sachs disease Treatment Corticosteroid replacement therapy login to view 1 more bullet Hematopoietic cell transplantation login to view 1 more bullet Lorenzo's oil login to view 2 more bullets Prognosis, Prevention, and Complications Death by age 12