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

Allowable subheadings

BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis 3
DG
diagnostic imaging
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology
GE
genetics 3
HI
history
IM
immunology
ME
metabolism 2
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology 3
PP
physiopathology
PC
prevention & control
PX
psychology
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
UR
urine
VE
veterinary
VI
virology

C Diseases
C10 Nervous System Diseases 1 897
C10.228 Central Nervous System Diseases 811
C10.228.140 Brain Diseases 1 186
C10.228.140.163 Brain Diseases, Metabolic 85
C10.228.140.163.100 Brain Diseases, Metabolic, Inborn 28
C10.228.140.163.100.362 Hereditary Central Nervous System Demyelinating Diseases 12
C10.228.140.163.100.362.250 Adrenoleukodystrophy 43
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 17
C10.228.140.163.100.362.550 Leukodystrophy, Metachromatic 30
C10.228.140.163.100.362.775 Pelizaeus-Merzbacher Disease 11
C10.228.140.695 Leukoencephalopathies 12
C10.228.140.695.625 Hereditary Central Nervous System Demyelinating Diseases 12
C10.228.140.695.625.250 Adrenoleukodystrophy 43
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 17
C10.228.140.695.625.550 Leukodystrophy, Metachromatic 30
C10.228.140.695.625.775 Pelizaeus-Merzbacher Disease 11
C10.314 Demyelinating Diseases 308
C10.314.400 Hereditary Central Nervous System Demyelinating Diseases 12
C10.314.400.250 Adrenoleukodystrophy 43
C10.314.400.312 Alexander Disease 6
C10.314.400.375 Canavan Disease 9
C10.314.400.500 Leukodystrophy, Globoid Cell 17
C10.314.400.550 Leukodystrophy, Metachromatic 30
C10.314.400.775 Pelizaeus-Merzbacher Disease 11
C10.574 Neurodegenerative Diseases 679
C10.574.500 Heredodegenerative Disorders, Nervous System 47
C10.574.500.024 Alexander Disease 6
C10.574.500.050 Amyloid Neuropathies, Familial 22
C10.574.500.175 Bulbo-Spinal Atrophy, X-Linked 8
C10.574.500.300 Canavan Disease 9
C10.574.500.362 Cockayne Syndrome 4
C10.574.500.393 Dystonia Musculorum Deformans 3
C10.574.500.425 Gerstmann-Straussler-Scheinker Disease 23
C10.574.500.487 Hepatolenticular Degeneration 243
C10.574.500.490 Hereditary Central Nervous System Demyelinating Diseases 12
C10.574.500.493 Hereditary Sensory and Autonomic Neuropathies 26
C10.574.500.495 Hereditary Sensory and Motor Neuropathy 72
C10.574.500.497 Huntington Disease 327
C10.574.500.529 Lafora Disease 1
C10.574.500.545 Myotonia Congenita 19
C10.574.500.547 Myotonic Dystrophy 96
C10.574.500.549 Neurofibromatoses 28
C10.574.500.550 Neuronal Ceroid-Lipofuscinoses 29
C10.574.500.662 Optic Atrophies, Hereditary 5
C10.574.500.700 Pantothenate Kinase-Associated Neurodegeneration 17
C10.574.500.812 Spinal Muscular Atrophies of Childhood 59
C10.574.500.825 Spinocerebellar Degenerations 30
C10.574.500.850 Tourette Syndrome 86
C10.574.500.865 Tuberous Sclerosis 144
C10.574.500.875 Unverricht-Lundborg Syndrome 1
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities 439
C16.320 Genetic Diseases, Inborn 1 219
C16.320.400 Heredodegenerative Disorders, Nervous System 47
C16.320.400.024 Alexander Disease 6
C16.320.400.050 Amyloid Neuropathies, Familial 22
C16.320.400.100 Bulbo-Spinal Atrophy, X-Linked 8
C16.320.400.150 Canavan Disease 9
C16.320.400.200 Cockayne Syndrome 4
C16.320.400.330 Dystonia Musculorum Deformans 3
C16.320.400.350 Gerstmann-Straussler-Scheinker Disease 23
C16.320.400.361 Hepatolenticular Degeneration 243
C16.320.400.367 Hereditary Central Nervous System Demyelinating Diseases 12
C16.320.400.375 Hereditary Sensory and Motor Neuropathy 72
C16.320.400.415 Hereditary Sensory and Autonomic Neuropathies 26
C16.320.400.430 Huntington Disease 327
C16.320.400.480 Lafora Disease 1
C16.320.400.525 X-Linked Intellectual Disability 18
C16.320.400.540 Myotonia Congenita 19
C16.320.400.542 Myotonic Dystrophy 96
C16.320.400.550 Neuroacanthocytosis 6
C16.320.400.560 Neurofibromatoses 28
C16.320.400.600 Neuronal Ceroid-Lipofuscinoses 29
C16.320.400.630 Optic Atrophies, Hereditary 5
C16.320.400.650 Pantothenate Kinase-Associated Neurodegeneration 17
C16.320.400.765 Spinal Muscular Atrophies of Childhood 59
C16.320.400.780 Spinocerebellar Degenerations 30
C16.320.400.820 Tourette Syndrome 86
C16.320.400.880 Tuberous Sclerosis 144
C16.320.400.940 Unverricht-Lundborg Syndrome 1
C16.320.565 Metabolism, Inborn Errors 715
C16.320.565.189 Brain Diseases, Metabolic, Inborn 28
C16.320.565.189.362 Hereditary Central Nervous System Demyelinating Diseases 12
C16.320.565.189.362.250 Adrenoleukodystrophy 43
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 17
C16.320.565.189.362.550 Leukodystrophy, Metachromatic 30
C16.320.565.189.362.775 Pelizaeus-Merzbacher Disease 11
C18 Nutritional and Metabolic Diseases 172
C18.452 Metabolic Diseases 1 205
C18.452.132 Brain Diseases, Metabolic 85
C18.452.132.100 Brain Diseases, Metabolic, Inborn 28
C18.452.132.100.362 Hereditary Central Nervous System Demyelinating Diseases 12
C18.452.132.100.362.250 Adrenoleukodystrophy 43
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 17
C18.452.132.100.362.550 Leukodystrophy, Metachromatic 30
C18.452.132.100.362.775 Pelizaeus-Merzbacher Disease 11
C18.452.648 Metabolism, Inborn Errors 715
C18.452.648.189 Brain Diseases, Metabolic, Inborn 28
C18.452.648.189.362 Hereditary Central Nervous System Demyelinating Diseases 12
C18.452.648.189.362.250 Adrenoleukodystrophy 43
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 17
C18.452.648.189.362.550 Leukodystrophy, Metachromatic 30
C18.452.648.189.362.775 Pelizaeus-Merzbacher Disease 11

Alexanders leukodystrophy Disease MeSH Browser

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