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MR in Congenital and Hereditary Metabolic Disorders Associated with Epilepsy

  • F. Vigevano
  • G. Fariello
  • A. Bartuli
  • E. Bertini
  • C. Dionisi-Vici
  • L. Fusco
Part of the NATO ASI Series book series (NSSA, volume 264)

Abstract

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.

Keywords

Metabolic Disorder Maple Syrup Urine Disease Glutaric Aciduria Type Propionic Acidaemia Neuronal Migration Disorder 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1994

Authors and Affiliations

  • F. Vigevano
    • 1
  • G. Fariello
    • 1
  • A. Bartuli
    • 1
  • E. Bertini
    • 1
  • C. Dionisi-Vici
    • 1
  • L. Fusco
    • 1
  1. 1.Section of NeurophysiologyChildren’s Hospital and Scientific Research Institute “Bambino Gesu’”RomeItaly

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