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Disorders of the cerebral white matter in children. The spectrum of lesions.

Artikel i vetenskaplig tidskrift
Författare Ragnhildur Kristjánsdóttir
Paul Uvebrant
Bengt Hagberg
Mårten Kyllerman
Lars-Martin Wiklund
G Blennow
O Flodmark
L Gustavsson
Sven Ekholm
Jan-Eric Månsson
Publicerad i Neuropediatrics
Volym 27
Nummer/häfte 6
Sidor 295-8
ISSN 0174-304X
Publiceringsår 1996
Publicerad vid Institutionen för klinisk neurovetenskap
Institutionen för kvinnors och barns hälsa, Avdelningen för pediatrik
Institutionen för särskilda specialiteter, Avdelningen för radiologi
Sidor 295-8
Språk en
Länkar www.ncbi.nlm.nih.gov/entrez/query.f...
Ämnesord Adolescent, Adult, Child, Child, Preschool, Diffuse Cerebral Sclerosis of Schilder, pathology, Female, Humans, Infant, Leukodystrophy, Metachromatic, pathology, Leukoencephalopathy, Progressive Multifocal, pathology, Magnetic Resonance Imaging, Male
Ämneskategorier Medicin och Hälsovetenskap

Sammanfattning

The use of magnetic resonance imaging (MRI) has resulted in the detection of an increasing number of children with an apparently leukodystrophic white matter. Laboratory tests and the clinical presentation, however, often do not correspond to any known entity and the course is sometimes not progressively deteriorating. Such children with white-matter changes and no known diagnosis were the subject of this Swedish multicentre study, in which MRI findings and clinical data from 100 children considered to have white-matter abnormalities were assessed during the period 1992-1995. At re-evaluation of MR images by an established "white-matter group" of neuroradiologists, paediatric neurologists, neurologists and neurochemists, the MRI signal of the white matter was considered normal in eleven children and eleven had mainly a grey matter affection. Of the remaining 78 children with white matter abnormalities, a diagnosis was found in 32, but in 46 children no diagnosis could be established. A progressive downhill course characterised 17, probably representing hitherto undefined types of leukodystrophies. Five children had a relapsing-remitting course, and in 11 it was difficult to establish whether the course was progressive or stationary. The disease was non-progressive in 13. This group of non-leukodystrophic white-matter changes obviously represents maldevelopments of myelin formation, thus dys- or hypomyelination rather than demyelination.

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