Alexander Disease [Alexanderova nemoc]

topical
6
Terms

Alexanderova choroba

 

Alexander's Disease
Demyelinogenic Leukodystrophy
Dysmyelinogenic Leukodystrophy
Fibrinoid Degeneration of Astrocytes
Leukodystrophy with Rosenthal Fibers

Persistent link   https://www.medvik.cz/link/D038261
Definition

Rare leukoencephalopathy with infantile-onset accumulation of Rosenthal fibers in the subpial, periventricular, and subependymal zones of the brain. Rosenthal fibers are GLIAL FIBRILLARY ACIDIC PROTEIN aggregates found in ASTROCYTES. Juvenile- and adult-onset types show progressive atrophy of the lower brainstem instead. De novo mutations in the GFAP gene are associated with the disease with propensity for paternal inheritance.

DUI
D038261 MeSH Browser
CUI
M0415670
History note
2003
Public note
2003

C Diseases
C10.228.140 Brain Diseases 1 177
C10.228.140.163 Brain Diseases, Metabolic 84
C10.228.140.163.100.362.250 Adrenoleukodystrophy 41
C10.228.140.163.100.362.312 Alexander Disease 6
C10.228.140.163.100.362.375 Canavan Disease 9
C10.228.140.163.100.362.500 Leukodystrophy, Globoid Cell 16
C10.228.140.163.100.362.550 Leukodystrophy, Metachromatic 29
C10.228.140.163.100.362.775 Pelizaeus-Merzbacher Disease 11
C10.228.140.695 Leukoencephalopathies 11
C10.228.140.695.625.250 Adrenoleukodystrophy 41
C10.228.140.695.625.312 Alexander Disease 6
C10.228.140.695.625.375 Canavan Disease 9
C10.228.140.695.625.500 Leukodystrophy, Globoid Cell 16
C10.228.140.695.625.550 Leukodystrophy, Metachromatic 29
C10.228.140.695.625.775 Pelizaeus-Merzbacher Disease 11
C10.314.400.250 Adrenoleukodystrophy 41
C10.314.400.312 Alexander Disease 6
C10.314.400.375 Canavan Disease 9
C10.574.500.024 Alexander Disease 6
C10.574.500.300 Canavan Disease 9
C10.574.500.362 Cockayne Syndrome 4
C10.574.500.497 Huntington Disease 322
C10.574.500.529 Lafora Disease 1
C10.574.500.545 Myotonia Congenita 17
C10.574.500.547 Myotonic Dystrophy 94
C10.574.500.549 Neurofibromatoses 28
C10.574.500.850 Tourette Syndrome 86
C10.574.500.865 Tuberous Sclerosis 141
C16.320.400.024 Alexander Disease 6
C16.320.400.150 Canavan Disease 9
C16.320.400.200 Cockayne Syndrome 4
C16.320.400.430 Huntington Disease 322
C16.320.400.480 Lafora Disease 1
C16.320.400.540 Myotonia Congenita 17
C16.320.400.542 Myotonic Dystrophy 94
C16.320.400.550 Neuroacanthocytosis 6
C16.320.400.560 Neurofibromatoses 28
C16.320.400.820 Tourette Syndrome 86
C16.320.400.880 Tuberous Sclerosis 141
C16.320.565.189.362.250 Adrenoleukodystrophy 41
C16.320.565.189.362.312 Alexander Disease 6
C16.320.565.189.362.375 Canavan Disease 9
C16.320.565.189.362.500 Leukodystrophy, Globoid Cell 16
C16.320.565.189.362.550 Leukodystrophy, Metachromatic 29
C16.320.565.189.362.775 Pelizaeus-Merzbacher Disease 11
C18.452 Metabolic Diseases 1 196
C18.452.132.100.362.250 Adrenoleukodystrophy 41
C18.452.132.100.362.312 Alexander Disease 6
C18.452.132.100.362.375 Canavan Disease 9
C18.452.132.100.362.500 Leukodystrophy, Globoid Cell 16
C18.452.132.100.362.550 Leukodystrophy, Metachromatic 29
C18.452.132.100.362.775 Pelizaeus-Merzbacher Disease 11
C18.452.648.189.362.250 Adrenoleukodystrophy 41
C18.452.648.189.362.312 Alexander Disease 6
C18.452.648.189.362.375 Canavan Disease 9
C18.452.648.189.362.500 Leukodystrophy, Globoid Cell 16
C18.452.648.189.362.550 Leukodystrophy, Metachromatic 29
C18.452.648.189.362.775 Pelizaeus-Merzbacher Disease 11