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FIBRINOID DEGENERATION OF ASTROCYTES
Descriptors Found:
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DeCS
Descriptor
English
:
Alexander Disease
Descriptor
Spanish
:
Enfermedad de Alexander
Descriptor
Portuguese
:
Doença de Alexander
Synonyms
English
:
Alexander's Disease
Alexanders Disease
Demyelinogenic Leukodystrophy
Dysmyelinogenic Leukodystrophy
Fibrinoid Degeneration of Astrocytes
Leukodystrophy with Rosenthal Fibers
Tree Number:
C10.228.140.163.100.362.312
C10.228.140.695.625.312
C10.314.400.312
C10.574.500.024
C16.320.400.024
C16.320.565.189.362.312
C18.452.132.100.362.312
C18.452.648.189.362.312
Definition
English
:
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.
See Related
English
:
Glial Fibrillary Acidic Protein
History Note
English
:
2003
Allowable Qualifiers
English
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis
DG
diagnostic imaging
DH
diet therapy
DT
drug therapy
EC
economics
EM
embryology
EN
enzymology
EP
epidemiology
EH
ethnology
ET
etiology
GE
genetics
HI
history
IM
immunology
ME
metabolism
MI
microbiology
MO
mortality
NU
nursing
PS
parasitology
PA
pathology
PP
physiopathology
PC
prevention & control
PX
psychology
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
UR
urine
VE
veterinary
VI
virology
Record Number:
37281
Unique Identifier:
D038261
Occurrence in VHL
:
Similar:
DeCS