Leukodystrophy
| Classification according to ICD-10 | |
|---|---|
| E75.2 | Other sphingolipidoses |
| E71.3 | Fatty acid metabolism disorders |
| ICD-10 online (WHO version 2019) | |
As a leukodystrophy ( Greek coinage: λευκοδυστροφία , "white failure to thrive" or "deficiency of white [substance]"; from λευκός , leukós , "white", δυσ- , dysplastic , "poor", "poor" and τροφή , trophy , "Thriving", "growth", "nutrition") is a group of genetically determined metabolic diseases that cause progressive degeneration of the white matter of the nervous system . This is due to an incomplete expression or degradation of the myelin surrounding the nervesthe function of the nerves is massively impaired. Those affected therefore suffer from motor and other neurological impairments.
Classification
ICD-10 keys are given, if available:
- Adrenoleukodystrophy , also Addison-Schilder syndrome (E71.3)
- Metachromatic leukodystrophy (E75.2)
- Krabbe's disease , also globoidal cell leukodystrophy (E75.2)
- Pelizaeus-Merzbacher disease (E75.2)
- Canavan disease (E75.2)
- CACH (childhood ataxia with central hypomyelination, English for "childhood ataxia with central reduced myelination")
- Alexander disease (E75.2)
- Van Bogaert-Scherer-Epstein syndrome , also cerebrotendinous xanthomatosis (E75.5)
See also
Web links
literature
- S1 guideline for leukodystrophies in adulthood of the German Society for Neurology. In: AWMF online (as of 2012)
Individual evidence
- ↑ a b Alphabetical directory for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 504.
- ↑ Alphabetical index for the ICD-10-WHO version 2019, volume 3. German Institute for Medical Documentation and Information (DIMDI), Cologne, 2019, p. 39
