MR in Congenital and Hereditary Metabolic Disorders Associated with Epilepsy
Metabolic disorders result in various types of brain lesion. The commonest neurological symptoms are developmental delay, movement disorders and epileptic seizures. In some metabolic disorders, epilepsy is the most prominent symptom; in others it is one of several associated symptoms, and in other forms seizures are rare. This depends on the degree of disruption of normal brain structure. In general, the severity of epilepsy reflects the degree of cortical grey matter involvement and the level of maturity of the brain. The latter is relevant because metabolic disorders often become manifest during the early phases of development. MR imaging is invaluable for defining the underlying neuropathology. In this chapter we examine correlations between the brain lesion, type of epilepsy and MR appearances.
KeywordsMetabolic Disorder Maple Syrup Urine Disease Glutaric Aciduria Type Propionic Acidaemia Neuronal Migration Disorder
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- Chevrie, J. J., Aicardi, J., and Goutires, F., 1987, Epilepsy in childhood mitochondrial encephalomyopathies, in: “Advances in Epileptology,” P. Wolf, M. Dam, D. Janz, F. E. Dreifuss, eds, Raven Press, New York.Google Scholar
- Dalla Bernardina, B., Lombardi, A., and Tassinari, C. A., 1982, Aspetti EEG e neurofisio-logici delle malattie dismetaboliche con epilessia dell’ et a pediatrica, Bol Lega It Epil. 39: 143–148.Google Scholar
- Kendall, B. E., 1992, Disorders of lysosomes, peroxisomes, and mitochondria, AJNR 363: 621–653.Google Scholar