The pathology of peroxisomal disorders with pathogenetic considerations.
J Neuropathol Exp Neurol
; 54(5): 710-9, 1995 Sep.
Article
em En
| MEDLINE
| ID: mdl-7666061
ABSTRACT
Peroxisomal disorders are rare systemic maladies in which specific major organ systems are typically involved most importantly the central nervous system (CNS), but also the peripheral nervous system, eyes, liver, adrenal, kidney and skeleton. Zellweger syndrome (ZS) is the most severe of the generalized types and exhibits major neocortical migration defects, less severe and non-inflammatory white matter lesions, and dysmorphic features. In adrenoleukodystrophy (ALD) the major lesion again is in the CNS, but consists of extensive dysmyelination/inflammatory demyelination without neuronal migration defects or dysmorphism. In adrenomyeloneuropathy long tract degeneration of spinal cord, peripheral neuropathy, and variable CNS dysmyelinative to inflammatory demyelinative lesions are the dominant nervous system lesions. Saturated very long chain fatty acids, either free in the cytoplasm of affected endocrine cells or as components of membrane lipids (e.g. gangliosides, glycerophospholipids, and proteolipid protein) in axons or myelin, may be central to the pathogenesis of these neuronal migration defects, dysmyelination/inflammatory demyelination and spinal tract degeneration. Cytokines, particularly tumor necrosis factor-alpha, and delayed cellular hypersensitivity appear to be major secondary pathogenic factors in ALD.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Medula Espinal
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Encéfalo
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Encefalopatias
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Microcorpos
Limite:
Humans
Idioma:
En
Revista:
J Neuropathol Exp Neurol
Ano de publicação:
1995
Tipo de documento:
Article
País de afiliação:
Estados Unidos