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Cited by 76Metabolism and Genetic DisordersFetal and Pediatric Neurological DisordersNeurological diseases and metabolism

The MR spectrum of peroxisomal disorders

van der Knaap, M. S., Valk, J.·1991·Neuroradiology
DOI 10.1007/bf00593330
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Abstract

In the last decade an increasing number of peroxisomal disorders has been recognized. Almost all peroxisomal disorders affect the central nervous system. Many of them lead to demyelination, some of them lead to migrational disturbances. The MR pattern of X-linked adrenoleukodystrophy is well known, but the pattern of the other peroxisomal disorders is less well known. We evaluated the gray and white matter abnormalities of 20 patients on 32 occasions. We compared the results with histological data and in this way came to the description of a number of characteristic MR patterns occurring in peroxisomal disorders: (1) Neuronal migrational disturbances in combination with hypomyelination, dysmyelination or demyelination. (2) Symmetrical demyelination of posterior limb of the internal capsule, cerebellar white matter and brain stem tracts with a variable affection of cerebral hemispheres. (3) Symmetrical demyelination, exhibiting two zones, starting in the occipital area and spreading outwards and forwards; affection of brain stem tracts. (4) Less characteristic patterns of demyelination. The patterns are illustrated and differentiation from other disorders is discussed.

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Crossref · 10.1007/bf00593330

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  • In patients suffering from acatalasemia, no neurological involvement or brain abnormalities have been described.

    “Surprisingly, in patients suffering from acatalasemia, an inherited peroxisomal disorder caused by the loss of functional catalase, no neurological involvement or brain abnormalities have been described, although this enzyme plays such a prominent role in oxygen metabolism [37,38].”

    From Peroxisomes in brain development and function (2016) · cites this paper as [37]

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Authors
van der Knaap, M. S., & Valk, J.
Venue
Neuroradiology
Publisher
Springer Science and Business Media LLC
Published
1991-01
Volume
33
Issue
1
Pages
30-37
ISSN
  • 0028-3940 (ISSN-L)
  • 1432-1920
DOI
10.1007/bf00593330
OpenAlex
W2056156479
Cited by
76
References
46
Citations by year
2021: 1 · 2020: 2 · 2019: 1 · 2018: 3 · 2017: 2 · 2016: 4 · 2015: 4 · 2014: 3 · 2012: 4
FWCI
1.98
Type
journal article
Language
en
Open access
closed
Licence
www.springer.com/tdm (tdm)
Locations
  • Neuroradiology (publishedVersion)
  • PubMed (publishedVersion)
  • Data Archiving and Networked Services (DANS) (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
  • Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
Keywords
peroxisomal disorders, demyelination, hypomyelination, dysmyelination, neuronal migration disorders, X-linked adrenoleukodystrophy, internal capsule, cerebellar white matter, differential diagnosis
MeSH terms
Adolescent, Adrenoleukodystrophy, Adult, Brain, Central Nervous System Diseases, Child, Child, Preschool, Chondrodysplasia Punctata, Female, Humans, Infant, Magnetic Resonance Imaging, Male, Microbodies, Middle Aged, Zellweger Syndrome

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