A SPORADIC CASE OF VERY SLOW PROGRESSIVE LEUKODYSTROPHY INVOLVING THECEREBELLAR PEDUNCLES

We describe a patient with infantile onset leukodystrophy involving th e cerebellar peduncles, She had mild mental retardation, spastic diple gia and mild cerebellar ataxia, The peripheral nerves seemed to be nor mal, The characteristic MRI findings in this case were extensive lesio ns of the white matter involving the cerebellar peduncles. In addition there was ventricular enlargement with a markedly decreased volume of the white matter and a hypoplastic corpus callosum, The clinical and laboratory findings imply that the white matter lesions in this patien t were the result of delayed myelination rather than demyelination. Th e patient was evaluated for known metabolic and degenerative diseases, but no abnormalities were observed. Her symptoms and neuroimaging fin dings did not fit the criteria for any defined leukodystrophy.