Leukoencephalopathy with a mild clinical course: a case report

Infantile-onset leukoencephalopathy of van der Knaap type is manifested by initially normal or near normal neurological findings despite infantile-ons et megalencephaly and magnetic resonance imaging evidence of severe white m atter affection. Until this entity was recently described, these cases were usually presented under the heading of atypical variants of Alexander dise ase. To date 63 individuals have been reported in English literature. We re port a four-year-old boy presented in the first months of life with progres sive megalencephaly. delay in walking, clumsiness, convulsions and magnetic resonance imaging evidence of diffuse swelling of white matter, cystic cav itations in frontal, temporal and parietal lobes. (C) 1999 Elsevier Science Ltd. All rights reserved.