title: The epidemiology of progressive encephalopathies in childhood. I. Live birth prevalence in west Sweden.
abstract: Progressive encephalopathies in the west Swedish region were investigated in a population-based study. Cases were allocated to one of five main groups: 1) disorders covered by impairments in subcellular organelles, 2) disorders covered by deficiencies in the intermediate metabolism, 3) biochemically undefined neurometabolic disorders, 4) neuroimmunological disorders and 5) a symptom-orientated miscellaneous group. Progressive encephalopathies were identified in 76 of 132,138 children born alive in the study area during the 16-year-period 1970-85. This gives a live birth prevalence of 0.58 per 1000. In Sweden the size of the group corresponds to that of neural tube defects and that of congenital hydrocephalus. However, a vast number of different disorders are represented.
authors.affiliation: Department of Pediatrics, Ostra Hospital, Gothenburg University, Sweden.
authors.forename: P
authors.initials: P
authors.lastname: Uvebrant
authors.forename: K
authors.initials: K
authors.lastname: Lanneskog
authors.forename: B
authors.initials: B
authors.lastname: Hagberg
mesh_terms: D000293:Adolescent; D001925:Brain Damage, Chronic / Q000453:epidemiology* / Q000235:genetics; D001928:Brain Diseases, Metabolic / Q000453:epidemiology* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D006233:Persons with Disabilities / Q000706:statistics & numerical data*; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007968:Leukoencephalopathy, Progressive Multifocal / Q000453:epidemiology*; D008297:Male; D013548:Sweden / Q000453:epidemiology