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Degenerative and Metabolic Brain Diseases

  • Casper JansenEmail author

Abstract

Neurodegenerative diseases are rare in childhood. Most of them result from disturbances in the metabolism of energy, lipids, amino acids, or metals. Symptoms may not appear before childhood or adolescence, but some are fatal in utero or in the neonatal period; they form the core of this overview. Before starting a postmortem examination, clinical and radiological findings should be obtained, because they may yield important diagnostic clues. Neuropathologically, a useful starting point is to establish whether the gray or the white matter is primarily affected. Microscopically, the neuropathological features can be diagnostic for a certain disease, but in most cases additional biochemical, genetic, and molecular studies are required to come to a final diagnosis.

Keywords

Neurodegeneration Neurometabolic disease Leukodystrophy Poliodystrophy Neuronal storage disease Mitochondrial disease Lysosomal disease Peroxisomal disease 

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Copyright information

© Springer International Publishing 2015

Authors and Affiliations

  1. 1.Laboratory for Pathology Eastern NetherlandsHengeloThe Netherlands

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