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After 8 years of age, additional white\u2010matter abnormalities occurred in the corpus callosum, internal and external capsule, and subcortical areas. From 11 years of age, white\u2010matter abnormalities were also found in the brainstem. Although there seemed to be a characteristic pattern of involvement over time, there were considerable variations between patients, reflected by variations in neuropsychological development. Cognitive development ranged from stable and normal to declines that lead to intellectual disabilities.\n <\/jats:p>\n <\/jats:sec>\n \n Interpretation<\/jats:title>\n \n As treatment enables patients with classic infantile Pompe disease to reach adulthood, white\u2010matter abnormalities are becoming increasingly evident, affecting the neuropsychological development. Therefore, we advise follow\u2010up programs are expanded to capture\n CNS<\/jats:styled-content>\n involvement in larger, international patient cohorts, to incorporate our findings in the counselling of parents before the start of treatment, and to include the brain as an additional target in the development of next\u2010generation therapeutic strategies for classic infantile Pompe disease.\n <\/jats:p>\n <\/jats:sec>\n \n What this paper adds<\/jats:title>\n \n \n \n In our long\u2010term survivors treated intravenously with enzyme replacement therapy, we found slowly progressive symmetric white\u2010matter abnormalities.<\/jats:p>\n <\/jats:list-item>\n \n Cognitive development varied from stable and normal to declines towards intellectual disabilities.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:p>\n 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