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  • 1
    Electronic Resource
    Electronic Resource
    Computed tomography in Krabbe's disease: Comparison with neuropathology (1983)
    Springer
    Neuroradiology 25 (1983), S. 323-327 
    Details
    ISSN: 1432-1920
    Keywords: Krabbe's disease ; CT scanning ; globoid cell leukodystrophy ; gliosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report computed tomography (CT) appearance of two patients with Krabbe's disease. The most common findings included severe brain atrophy: an enlarged frontal extracerebral space, dilatation of ventricles, enlarged cisterns and enlarged cortical sulci. There was low attenuation in the corpus medullaris of the cerebellum, and symmetrical focal hypodensity in the central periventricular white matter. CT at the terminal stage (16 months) showed marked cerebral atrophy including flattening of the heads of caudate nuclei and widening of the third ventricles confirmed by a neuropathological study. There was relatively less low density on the white matter of Krabbe's disease compared with that of other leukodystrophies. These CT findings may be useful in the diagnosis. The relative lack of low density in the white matter of Krabbe's disease might be related to severe gliosis and reduced total lipid contents.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00439212
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    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Neuroradiological findings in the carbohydrate-deficient glycoprotein syndrome (1995)
    Springer
    Neuroradiology 37 (1995), S. 491-495 
    Details
    ISSN: 1432-1920
    Keywords: Carbohydrate-deficient glycoprotein syndrome ; Olivopontocerebellar atrophy ; Transferrin ; Stroke-like episodes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The carbohydrate-deficient glycoprotein syndrome is a newly recognised genetic disorder characterised by mental retardation, liver disfunction during infancy, cerebellar ataxia and atrophy, polyneuropathy, growth retardation, stroke-like episodes, and the appearance of carbohydrate-deficient fractions of multiple glycoproteins in the serum. The neuroradiological findings have been known as features of olivopontocerebellar atrophy. However, whether the abnormalities in the cerebellum and brain stem progress after birth is not known. We have carried out serial CT and MRI on three Japanese patients with this syndrome at different ages. A small cerebellum, with peculiar enlargement of the cisterna magna, and a small brain stem are present in infancy and atrophy of the anterior vermis and from before backwards in the cerebellar hemispheres seem to progress throughout early childhood.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00600103
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Neuroradiological findings in the carbohydrate-deficient glycoprotein syndrome (1995)
    Springer
    Neuroradiology 37 (1995), S. 491-495 
    Details
    ISSN: 1432-1920
    Keywords: Key words Carbohydrate-deficient glycoprotein syndrome ; Olivopontocerebellar atrophy ; Transferrin ; Stroke-like episodes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The carbohydrate-deficient glycoprotein syndrome is a newly recognised genetic disorder characterised by mental retardation, liver disfunction during infancy, cerebellar ataxia and atrophy, polyneuropathy, growth retardation, stroke-like episodes, and the appearance of carbohydrate-deficient fractions of multiple glycoproteins in the serum. The neuroradiological findings have been known as features of olivopontocerebellar atrophy. However, whether the abnormalities in the cerebellum and brain stem progress after birth is not known. We have carried out serial CT and MRI on three Japanese patients with this syndrome at different ages. A small cerebellum, with peculiar enlargement of the cisterna magna, and a small brain stem are present in infancy and atrophy of the anterior vermis and from before backwards in the cerebellar hemispheres seem to progress throughout early childhood.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00600103
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
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