title: The degenerative diseases or syndromes in maturing central nervous system.
abstract: The diagnosis of degenerative diseases or syndromes in the nervous system in based on their morphological picture. The changes occur in selected CNS structures or systems being induced in the course of more or less known processes sometimes with known, more often unknown etiology. Degenerative syndromes may be classified according to the topography of changes. They appear often with aging, but also in even greater number in infants. We tried to analyze the problem and find out to what degree the structure and topography of CNS degenerative changes in infants depend on maturity of nervous tissue constituting the background of pathologic process. The cases with two syndromes representative for small infants: progressive poliodystrophy of Alpers type and a degenerative syndrome with cerebral calcifications and disseminated demyelination were examined from this point of view. Our observations revealed that the stage of CNS development stipulates the type and topography of degenerative changes.
authors.affiliation: Department of Developmental Neuropathology, Polish Academy of Sciences, Warszawa, Poland.
authors.forename: M
authors.initials: M
authors.lastname: Dambska
authors.forename: A P
authors.initials: AP
authors.lastname: Anzil
authors.forename: M
authors.initials: M
authors.lastname: Wrzołek
authors.forename: O F
authors.initials: OF
authors.lastname: Patxot
mesh_terms: D017668:Age of Onset; D001284:Atrophy; D001921:Brain / Q000254:growth & development / Q000473:pathology; D002114:Calcinosis / Q000473:pathology; D002493:Central Nervous System Diseases / Q000145:classification / Q000473:pathology*; D003711:Demyelinating Diseases / Q000453:epidemiology / Q000473:pathology*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000453:epidemiology / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009128:Muscle Spasticity; D009207:Myoclonus / Q000473:pathology; D009410:Nerve Degeneration; D009474:Neurons / Q000473:pathology; D009896:Optic Atrophy / Q000473:pathology; D011782:Quadriplegia / Q000473:pathology